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Sample records for lymphoma extranodal marginal

  1. Extranodal marginal zone (MALT) lymphoma in common variable immunodeficiency.

    NARCIS (Netherlands)

    Desar, I.M.; Keuter, M.; Raemaekers, J.M.M.; Jansen, J.B.M.J.; Krieken, J.H.J.M. van; Meer, J.W.M. van der

    2006-01-01

    We describe two patients with common variable immunodeficiency (CVID) who developed extranodal marginal zone lymphoma (formerly described as mucosa-associated lymphoid tissue lymphoma or MALT lymphoma). One patient, with documented pernicious anaemia and chronic atrophic gastritis with metaplasia, d

  2. Chronic inflammatory disease, lymphoid tissue neogenesis and extranodal marginal zone B-cell lymphomas

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    R.J. Bende; F. van Maldegem; C.J.M. van Noesel

    2009-01-01

    Chronic autoimmune or pathogen-induced immune reactions resulting in lymphoid neogenesis are associated with development of malignant lymphomas, mostly extranodal marginal zone B-cell lymphomas (MZBCLs). In this review we address (i) chemokines and adhesion molecules involved in lymphoid neogenesis;

  3. Extranodal Marginal Zone Lymphoma Presenting within the Meckel Diverticulum as Diverticulitis: A Case Report

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    A. Nael

    2014-01-01

    Full Text Available Meckel diverticulum is the most common congenital defect of the gastrointestinal tract. It can be asymptomatic or mimic appendicitis and may be complicated by bleeding, diverticulitis, obstruction, and, rarely, neoplasia. We report the first case of extranodal marginal zone lymphoma occupying a Meckel diverticulum. A 44-year-old man with history of colonic diverticulitis presented to the emergency department for evaluation of acute abdominal pain. Radiography showed enteric obstruction, prompting diagnostic laparoscopy. Above the level of mid-ileum an intact Meckel diverticulum was identified. Microscopy showed extensive infiltration of sheets of small lymphocytes with abundant cytoplasm (monocytoid B-cells prominently in submucosa and focally transmural involving serosal adipose tissue with multiple reactive germinal centers. The immunostains showed positivity for CD20, BCL-2, and CD43 (weak and negativity for CD3, CD5, BCL-1, CD10, and BCL-6 in monocytoid B-cells. Fluorescence in situ hybridization studies revealed API2-MALT1 fusion signals consistent with t(11;18(q21;q21, which confirmed the diagnosis of extranodal marginal zone lymphoma, also known as mucosa associated lymphoid tissue lymphoma.

  4. Molecular pathogenesis and histologic and clinical features of extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue type.

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    Kuper-Hommel, M.J.; Krieken, J.H.J.M. van

    2012-01-01

    Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) type (EMZL) is considered an antigen driven lymphoid malignancy associated with protracted antigenic stimulation by microbial pathogens, auto-antigens or other unknown stimuli, which trigger a sustained lymphoid proliferat

  5. Extranodal marginal zone non Hodgkin's lymphoma of the lung: A ten-year experience

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    Milošević Violeta

    2011-01-01

    Full Text Available Background/Aim. Bronchus-associated lymphoid tissue (BALT lymphoma is a rare subtype of low grade marginal zone B cell lymphoma representing 10% of all MALT lymphomas. The purpose of this study was to analyze the outcome of this group of patients comparing prognostic parameters and therapy modalities. Methods. A total of eight patients with BALT lymphoma had diagnosed between January 1998 - April 2008 at the Institute of Hematology, Clinical Center of Serbia, Belgrade, and they were included in this retrospective analysis. Results. Male/female ratio was 2/6, the median age was 64 years (range 37-67 years. Six patients had nonspecific respiratory symptoms and all of them had B symptoms. The patients were seronegative for HIV, HCV and HBsAg. Three patients had Sjogren's syndrome, rheumatoid arthritis and pulmonary tuberculosis, respectively. Seven patients were diagnosed by transbronchial biopsy and an open lung biopsy was done in one patient. Patohistological findings revealed lymphoma of marginal zone B cell lymphoma: CD20+/CD10-/CD5-/CyclinD1- /CD23-/IgM- with Ki-67+<20% of all cells. According to the Ferraro staging system, five patients had localized disease (CS I-IIE and three had stage IVE; bulky tumor mass had 3 patients. All patients had Eastern Cooperative Oncology Group (ECOG performance status (PS 0 or 1. Five patients received monochemotherapy with chlorambucil and 3 were treated with CHOP regimen (cyclophosphamide, doxorubicin, vincristine and prednisone. A complete response (CR was achieved in 5 patients and a partial response (PR in 3 of them, treated with chlorambucil monotherapy and CHOP regimen. All patients were alive during a median follow-up period of 49 months (range 6- 110 months. Three patients relapsed after monochemotherapy into the other extranodal localization. They were treated with CHOP regimen and remained in stable PR. Conclusion. BALT lymphoma tends to be localised disease at the time of diagnosis, responds well

  6. Primary Thymic Extranodal Marginal-Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue Type Exhibits Distinctive Clinicopathological and Molecular Features

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    Inagaki, Hiroshi; Chan, John K. C.; Ng, Josephine W. M.; Okabe, Mitsukuni; Yoshino, Tadashi; Okamoto, Masataka; Ogawa, Hiroshi; Matsushita, Hiroshi; Yokose, Tomoyuki; Matsuno, Yoshihiro; Nakamura, Naoya; Nagasaka, Tetsuro; Ueda, Ryuzo; Eimoto, Tadaaki; Nakamura, Shigeo

    2002-01-01

    Extranodal marginal-zone B-cell lymphoma (MZBL) of mucosa-associated lymphoid tissue (MALT) arising in the thymus is rare, with the largest series in the literature including only three cases. In the present study, we investigated 15 cases of thymic MALT lymphoma to systematically characterize its clinical, histopathological, and molecular features. There was a marked female predilection (male:female = 1:4), with a mean age of 55 years at diagnosis. There was a strong association with autoimmune disease, especially Sjögren’s syndrome. Histologically, the thymic lymphoma showed the characteristic morphological features of extranodal MZBL of MALT type. Cysts were common. Prominent lymphoepithelial lesions were formed by centrocyte-like cells infiltrating and expanding the Hassall’s corpuscles and epithelium lining the cysts. Plasmacytic differentiation was apparent in all cases. Notably, 13 of 15 cases expressed immunoglobulin (Ig) A phenotype; IgA expression in thymic MALT lymphoma was in striking contrast with the IgM phenotype observed in most of the Sjögren’s syndrome-associated MZBLs and MALT lymphomas at other sites. Epstein-Barr virus was absent, and API2-MALT1 gene fusion, a recently reported MALT lymphoma-specific gene abnormality, was not detected in any case. Although one patient died of disease 85 months after the diagnosis, other patients were alive with overall 3-year and 5-year survival rates being 89% and 83%, respectively. Among the 22 patients reported previously and in the present series, at least 17 patients (77%) were Asians. These data indicate that thymic MALT lymphoma may represent a distinct subgroup of MALT lymphoma characterized by apparent predilection for Asians, a strong association with autoimmune disease, frequent presence of cysts, consistent plasma cell differentiation, tumor cells expressing IgA phenotype, and consistent lack of API2-MALT1 gene fusion. PMID:11943727

  7. Abdominal manifestations of extranodal lymphoma: pictorial essay*

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    Fajardo, Laís; Ramin, Guilherme de Araujo; Penachim, Thiago José; Martins, Daniel Lahan; Cardia, Patrícia Prando; Prando, Adilson

    2016-01-01

    In the appropriate clinical setting, certain aspects of extranodal abdominal lymphoma, as revealed by current cross-sectional imaging techniques, should be considered potentially diagnostic and can hasten the diagnosis. In addition, diagnostic imaging in the context of biopsy-proven lymphoma can accurately stage the disease for its appropriate treatment. The purpose of this article was to illustrate the various imaging aspects of extranodal lymphoma in the abdomen. PMID:28057966

  8. Abdominal manifestations of extranodal lymphoma: pictorial essay

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    Fajardo, Lais; Cardia, Patricia Prando; Prando, Adilson, E-mail: laisfajardo@gmail.com [Centro Radiologico Campinas/Hospital Vera Cruz, Campinas, SP (Brazil); Ramin, Guilherme de Araujo; Penachim, Thiago Jose; Martins, Daniel Lahan [Pontificia Universidade Catolica de Campinas (PUC- Campinas), SP (Brazil)

    2016-11-15

    In the appropriate clinical setting, certain aspects of extranodal abdominal lymphoma, as revealed by current cross-sectional imaging techniques, should be considered potentially diagnostic and can hasten the diagnosis. In addition, diagnostic imaging in the context of biopsy-proven lymphoma can accurately stage the disease for its appropriate treatment. The purpose of this article was to illustrate the various imaging aspects of extranodal lymphoma in the abdomen. (author)

  9. Marginal zone B-cell lymphoma with multiple extranodal locations in a patient with Sjögren’s syndrome – a diagnostic problem

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    Marta Domżalska

    2014-09-01

    Full Text Available Sjögren’s syndrome is a chronic autoimmune disease characterized by the presence of lymphocytic infiltrates in exocrine glands, mainly salivary and lacrimal glands, which result in xerophthalmia and xerostomia. About half of the patients develop systemic complications, including lymphoproliferative disorders. We report a case of a 27-year-old woman with a diagnosis of Sjögren’s syndrome and a suspicion of respiratory system involvement in the course of granulomatosis with polyangiitis. Histopathological examination of a skin lesion suggested marginal zone B-cell lymphoma. After pathological and immunohistochemical evaluation of all available previous biopsy samples and the medical documentation the diagnosis of extranodal marginal zone B-cell lymphoma stage IV according to the Ann Arbor classification was rendered. The patient was referred to the Department of Haematology and was treated with R-CVP (cyclophosphamide, vincristine, prednisone, rituximab.

  10. Modern radiation therapy for extranodal lymphomas

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    Yahalom, Joachim; Illidge, Tim; Specht, Lena

    2015-01-01

    Extranodal lymphomas (ENLs) comprise about a third of all non-Hodgkin lymphomas (NHL). Radiation therapy (RT) is frequently used as either primary therapy (particularly for indolent ENL), consolidation after systemic therapy, salvage treatment, or palliation. The wide range of presentations of EN...

  11. Epstein-barr virus-associated extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT Lymphoma) arising in the parotid gland of a child with ataxia telangiectasia.

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    Bennett, Jennifer A; Bayerl, Michael G

    2015-03-01

    Hematologic malignancies, in particular T-cell lymphomas/leukemias, are prevalent in patients with ataxia telangiectasia (AT), with most reported cases being clinically aggressive and high grade. Epstein-Barr virus (EBV) is often associated with lymphoid proliferations/neoplasms arising in immunodeficient patients. Reports of low-grade B-cell neoplasms in the ataxia telangiectasia population are extremely rare. Here, we describe a case of EBV-associated extranodal marginal zone lymphoma (mucosa-associated lymphoid tissue lymphoma) of the parotid gland in a 16-year-old boy with AT. In addition, we review the literature of hematologic malignancies in the AT population as well as the occurrence of EBV in mucosa-associated lymphoid tissue lymphoma.

  12. A minute focus of extranodal marginal zone B-cell lymphoma arising in Hashimoto thyroiditis diagnosed with PCR after laser capture microdissection: a case report

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    D'Antonio Antonio

    2009-09-01

    Full Text Available Abstract Background Primary thyroid gland lymphomas are uncommon tumours that occur in the setting of lymphocytic thyroiditis or Hashimoto's disease in almost all cases. In this condition a distinction between an inflammatory lymphoid infiltrate and a low grade lymphoma may be extremely difficult and precise criteria are necessary for a correct diagnosis. Patient and methods We report a case of a minute focus of primary extranodal marginal zone B-cell lymphoma (EMZBCL, incidentally discovered in a 63-year-old man with Hashimoto thyroiditis (HT and diagnosed by means of polymerase chain reaction (PCR after laser capture microdissection. The histological examination of surgical specimen confirmed the diagnosis of HT and showed a minute focus of dense lymphoid infiltrate (less than 4 mm in diameter, composed by centrocyte-like cells forming MALT balls. Immunoistochemistry was not useful. A microscopic focus of EMZBCL was suspected on the basis of morphological features. PCR assays revealed the rearrangement of the heavy chain of immunoglobulins only in the microdissected suspicious area, confirming the diagnosis of EMZBCL. Conclusion Our finding suggests that in cases of autoimmune thyroiditis a careful examination of the thyroid specimen is warranted, in order to disclose areas or small foci of lymphomatous transformation. Furthermore, in difficult cases with doubtful immunohistological findings, ancillary techniques, such as molecular studies, are necessary for a conclusive diagnosis.

  13. Long-Term Outcomes and Patterns of Relapse of Early-Stage Extranodal Marginal Zone Lymphoma Treated With Radiation Therapy With Curative Intent

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    Teckie, Sewit; Qi, Shunan; Lovie, Shona [Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, New York (United States); Navarrett, Scott [Weill Cornell Medical College, New York, New York (United States); Hsu, Meier [Department of Epidemiology and Biostatistics, Memorial Sloan Kettering Cancer Center, New York, New York (United States); Noy, Ariela; Portlock, Carol [Lymphoma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York (United States); Yahalom, Joachim, E-mail: yahalomj@mskcc.org [Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, New York (United States)

    2015-05-01

    Purpose: To report the long-term outcome and patterns of relapse of a large cohort of marginal zone lymphoma (MZL) patients treated with curative-intent radiation therapy (RT) alone. Patients and Methods: We reviewed the charts of 490 consecutive patients with stage IE or IIE MZL referred between 1992 and 2012 to our institution. Of those, 244 patients (50%) were treated with RT alone. Pathology was confirmed by hematopathologists at our institution. Patient and disease factors were analyzed for association with relapse-free survival (RFS) and overall survival (OS). Results: Median age of the cohort was 59 years, and median follow-up was 5.2 years. Ann Arbor stage was IE in 92%. Most common disease sites were stomach (50%), orbit (18%), non-thyroid head-and-neck (8%), skin (8%), and breast (5%). Median RT dose was 30 Gy. Five-year OS and RFS were 92% and 74%, respectively. Cumulative incidence of disease-specific death was just 1.1% by 5 years. Sixty patients (24%) developed relapse of disease; 10 were in the RT field. Crude rate of transformation to pathologically confirmed large-cell lymphoma was 1.6%. On multivariable analysis, primary disease site (P=.007) was independently associated with RFS, along with age (P=.04), presence of B-symptoms (P=.02), and International Prognostic Index risk group (P=.03). All disease sites except for head-and-neck had worse RFS relative to stomach. Conclusion: Overall and cause-specific survival are high in early-stage extra-nodal MZL treated with curative RT alone. In this large cohort of 244 patients, most patients did not experience relapse of MZL after curative RT; when relapses did occur, the majority were in distant sites. Stomach cases were less likely to relapse than other anatomic sites. Transformation to large-cell lymphoma was rare.

  14. Extranodal lymphoplasmacytoid lymphoma: spectrum of disease

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    Guermazi, Ali [Department of Radiology, University of California at San Francisco, 350 Parnassus Avenue, Suite 150, San Francisco, CA 94117 (United States); Department of Radiology, Saint Louis University Hospital, AP-HP, Paris (France); Meignin, Veronique [Department of Pathology, Saint Louis University Hospital, AP-HP, Paris (France); Brice, Pauline [Department of Hematology, Saint Louis University Hospital, AP-HP, Paris (France)

    2003-04-01

    Lymphoplasmacytoid lymphomas (LPL) are non-Hodgkin's lymphomas characterized by a proliferation of lymphoplasmacytoid cells or plasma cells with intracytoplasmic monoclonal Ig. The LPL are low-grade B-cell neoplasms close to B chronic lymphocytic leukemia with plasmacytoid differentiation. They show an indolent course, typically affect older men, and present as a disseminated disease with predominantly nodal involvement. Nevertheless, localized forms, some of them extranodal, have been described. The cases that best represent the range of radiographic findings on X-ray, CT, and MR imaging are presented. (orig.)

  15. Primary extranodal marginal zone lymphoma of the uvea associated with massive diffuse epibulbar extension and focal infiltration of the optic nerve and meninges, clinically presented as uveitis masquerade syndrome: a case report.

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    Rasić, D M; Stanković, Z; Terzić, T; Kovacević, D; Koturović, Z; Marković, V

    2010-09-01

    To report a clinical, histopathological and immunohistochemical findings in a case of primary extranodal marginal zone lymphoma of the uvea associated with massive diffuse extraocular episcleral extension and focal infiltration of the optic nerve and meninges, clinically presented as longstanding uveitis masquerade syndrome. Interventional case reports with histopathological correlation. We describe a 80-year-old male patient with a 3-year history of chronic recurrent hypertensive (pan) uveitis associated with ocular pain, unresponsive to topical and systemic anti-inflammatory, immunosuppressive, antibiotic/antiviral and antiglaucomatous therapy. Because the eye was not salvageable with conservative treatment, enucleation of blind and painful eye was performed. Findings from histopathological and immunohistochemistry examination of the enucleated eye showed an extranodal marginal zone lymphoma of the uveal tract with massive epibulbar extension and optic nerve and meningeal penetration. During almost 3 years of clinical course and 6 months after the enucleation, there were no systemic manifestations of lymphoma, and patient has not required subsequent treatment. Primary lymphoproliferative lesions of the uvea, comprising the iris, ciliary body and choroid are very rare, associated with epibulbar extension extremely and with optic nerve and menigeal penetration exceptionally. Despite its rarity, primary lymphoma of the uvea should be included in the differential diagnosis particularly in older patients with longstanding recurrent uveitis.

  16. T(14;18)(q32;q21) involving MALT1 and IGH genes occurs in extranodal diffuse large B-cell lymphomas of the breast and testis

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    Kuper-Hommel, M.J.; Schreuder, M.I.; Gemmink, A.H.; Krieken, J.H. van

    2013-01-01

    Primary B-cell lymphoma of the testis, breast and thyroid are rare and data concerning cytogenetic aberrations at these extranodal sites are scarce. We examined the presence of extranodal marginal zone lymphoma-associated translocations, t(11;18)(q21;q21), t(1;14)(p22;q32), t(14;18)(q32;q21), t(3;14

  17. Radiation Therapy Administration and Survival in Stage I/II Extranodal Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue

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    Olszewski, Adam J., E-mail: adam_olszewski@brown.edu; Desai, Amrita

    2014-03-01

    Purpose: To determine the factors associated with the use of radiation therapy and associated survival outcomes in early-stage marginal zone lymphoma of the mucosa-associated lymphoid tissue (MALT). Methods and Materials: We extracted data on adult patients with stage I/II MALT lymphoma diagnoses between 1998 and 2010 recorded in the Surveillance, Epidemiology, and End Results (SEER) database. We studied factors associated with radiation therapy administration in a logistic regression model and described the cumulative incidence of lymphoma-related death (LRD) according to receipt of the treatment. The association of radiation therapy with survival was explored in multivariate models with adjustment for immortal time bias. Results: Of the 7774 identified patients, 36% received radiation therapy as part of the initial course of treatment. Older patients; black or Hispanic men; white, Hispanic, and black women; and socioeconomically disadvantaged and underinsured patients had a significantly lower chance of receiving radiation therapy. Radiation therapy administration was associated with a lower chance of LRD in most sites. In cutaneous, ocular, and salivary MALT lymphomas, the 5-year estimate of LRD after radiation therapy was 0%. The association of radiation therapy with overall survival in different lymphoma sites was heterogeneous, and statistically significant in cutaneous (hazard ratio 0.45, P=.009) and ocular (hazard ratio 0.47, P<.0001) locations after multivariate adjustment. Conclusions: Demographic factors are associated with the use of radiation therapy in MALT lymphoma. Clinicians should be sensitive to those disparities because the administration of radiation therapy may be associated with improved survival, particularly in cutaneous and ocular lymphomas.

  18. Multifocal primary cutaneous extranodal NK/T lymphoma nasal type*

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    de Vasconcelos, Pedro; Ferreira, Cristina; Soares-Almeida, Luís; Filipe, Paulo

    2016-01-01

    Nasal type extranodal NK/T-cell lymphoma is a distinct entity according to the World Health Organization classification. Although 60% to 90% of patients with this disease present with a destructive mass in the midline facial tissues, it may also primarily or secondarily involve extranasal sites, like the skin. We report the case of a 77-year-old patient that came to our department with erythematous plaques of the right leg and eczematous lesions of the trunk. These lesions were biopsied and the patient was diagnosed with extranodal NK/T-cell lymphoma, nasal type. He was treated with multi-agent systemic chemotherapy but died 5 months after diagnosis. This case highlights the rarity and variability of cutaneous features of this disease and its aggressive course and poor prognosis. PMID:27192524

  19. Radiotherapy studies and extra-nodal non-Hodgkin lymphomas, progress and challenges

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    Specht, L

    2012-01-01

    for the more common extra-nodal organs, e.g. stomach, Waldeyer's ring, skin and brain, are fairly well known and show significant variation. A few randomised trials have been carried out testing the role of radiotherapy in these lymphomas. However, for most extra-nodal lymphomas, randomised trials have...... coverage of extra-nodal lymphomatous involvement with better sparing of normal tissues. The necessary radiation doses and volumes need to be defined for the different extra-nodal lymphoma entities. The challenge is to optimise the use of radiotherapy in the modern multimodality treatment of extra...... not been carried out, and treatment decisions are made on small patient series and extrapolations from nodal lymphomas. Hopefully, wide international collaboration will make controlled clinical trials possible in the less common extra-nodal lymphomas. Modern highly conformal radiotherapy allows better...

  20. Extranodal non-Hodgkin's lymphoma presenting as gingival mass

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    Manjunatha, B. S.; Gowramma, R.; Nagarajappa, D.; Tanveer, Ahmed

    2011-01-01

    Non-Hodgkin's lymphoma (NHL) commonly presents as non-tender, enlarged lymph nodes, accompanied by diffuse symptoms of fatigue and low-grade intermittent fever and it is derived predominantly from the cells of the B lymphocyte series. NHL cases occur extra-nodally and in 3% of these cases the initial presentation may be in the oral cavity. Though extra-nodal NHL of the oral cavity is a rare finding, patients with oral lesions of NHL commonly present at the dental clinic in the first instance. A careful clinical evaluation supported by histopathological and other laboratory investigations will help in identifying the disease at an early stage, resulting in better prognosis. Any delay in diagnosis has important implications on the morbidity and mortality of the condition. Due to the rarity of intraoral NHL, we present one such a case with a complaint of tumor-like mass on the gingiva of lower molar region. The lesion was clinically thought as pyogenic granuloma and later diagnosed as extra nodal NHL of the oral cavity. PMID:22368372

  1. LARGE EXTRANODAL NON HODGKIN'S LYMPHOMA OF THE PARAPHARYNGEAL SPACE

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    Zorica Aleric

    2013-03-01

    Full Text Available Of all head and neck tumors, 0.5% to 0.8% of them are localized in the parapharyngeal space. Non-Hodgkin’s lymphomas in this site are extremely rare. There are described in literature as either isolated cases or small series of tumors of that space. We are showing a case of a younger man presented with the recent facial nerve paralysis, hearing loss, otalgia and on the examination seen bulged right side of the soft palate and medialization of the pharyngeal wall. The biopsy was performed transorally. The pathohistology finding described a large B cell non-Hodgkin ́s lymphoma. Usually, the parapharyngeal tumors are benign and the surgery is the treatment of choice. In this case patient underwent chemotherapy. We point out, although rare, extranodal non-Hodgkin’s lymphomas are possible pathological findings in the head and neck. Because of different treatment it is of great importance to know when we deal with this kind of pathology.

  2. Primary extranodal Non-Hodgkin lymphoma of the orbital and paranasal region—A retrospective study

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    Sandner, Annett, E-mail: annett.sandner@medizin.uni-halle.de [Department of Otorhinolaryngology, Martin-Luther-University Halle-Wittenberg, Ernst-Grube-Str. 40, 06097 Halle (Germany); Surov, Alexey, E-mail: alex.surow@medizin.uni-halle.de [Department of Radiology, Martin-Luther-University Halle-Wittenberg, Ernst-Grube-Str. 40, 06097 Halle (Germany); Bach, Andreas Gunter, E-mail: andreas.bach@medizin.uni-halle.de [Department of Radiology, Martin-Luther-University Halle-Wittenberg, Ernst-Grube-Str. 40, 06097 Halle (Germany); Kösling, Sabrina, E-mail: sabrina.koesling@medizin.uni-halle.de [Department of Radiology, Martin-Luther-University Halle-Wittenberg, Ernst-Grube-Str. 40, 06097 Halle (Germany)

    2013-02-15

    Purpose: Primary extranodal lymphomas of the orbit and sinonasal region are rare and occur almost only as Non-Hodgkin lymphoma (NHL). The purpose of this study was to determine the frequency of different subtypes of NHL in these regions and to describe their radiological features. Materials and methods: Between January 2005 and January 2010, 567 patients with malignant immunoproliferative diseases (MID) were treated at our institution. Primary sinonasal and orbital manifestation was diagnosed in 36 cases. There were 13 women and 23 men with a median age of 67 years. CT and MRI were performed in 14 and 24 patients, respectively. Imaging was re-interpretated and histological subtypes were listed. Results: Among all MID primary sinonasal and orbital NHL occurred with a frequency of 6%. Diffuse large cell lymphoma was identified in 11 cases (30%), marginal cell lymphoma in 6 (16%), and extranodal plasmacytoma in 5 (14%). Other subtypes were rare. On CT, lesions of soft tissue attenuation with homogeneous moderate contrast enhancement were seen in all cases. On T2-weighted fat saturated images 52% of the lesions were slightly hyperintense in comparison to unaffected musculature, 41% were isointense, and 7% slightly hypointense. On T1-weighted sequences most lesions (81%) were homogeneously isointense. After contrast administration marked enhancement was seen in 41%, moderate in 52%, and slight enhancement in 7%. Conclusion: The identified radiological features should be included in the differential analysis of lesions in the orbital and sinonasal regions, but they are not specific enough. For exact therapeutic planning histopathological diagnosis of the subtype is required.

  3. Agatolimod Sodium, Rituximab, and Yttrium Y 90 Ibritumomab Tiuxetan in Treating Patients With Recurrent or Refractory Non-Hodgkin Lymphoma

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    2016-01-04

    Adult Non-Hodgkin Lymphoma; Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Nodal Marginal Zone Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma; Waldenstrom Macroglobulinemia

  4. Extranodal Imaging Manifestations of Non-Hodgkin's Lymphoma

    Institute of Scientific and Technical Information of China (English)

    张景峰; 王仁法; 李勇刚; 张芳

    2003-01-01

    A series of imaging features of extranodal, multi-systemic involvements in Non-Hodgkin's lymphoma (NHL) were investigated. The clinical data and imaging findings of 16 patients withpathologically proved NHL were retrospectively analyzed. The related literatures were reviewed.Of the 16 cases of NHL, skeletal involvement was found in 4, nasal cavity and nasal sinuses wereinvolved in 4, too. Lesion in the thorax was seen in 3 patients, hepatic involvement occurred in onecase, cerebral ventricle was affected in 3 cases, mesentery was involved in one case. Even thoughextranodal involvement of NHL exhibited extremely variable patterns, there were some relativelytypical imaging findings. Emphasized in this report were the relatively specific imaging manifesta-tions of different systems, which may mimic infectious or other neoplasms of different sites. Theimportance of imaging studies lies in the availability for diagnosis, staging and follow-up of NHL.Combined with the clinical and other related information, the diagnostic accuracy can be further im-proved, thus, providing reliable evidence in guiding clinical management.

  5. 506U78 in Treating Patients With Recurrent or Refractory Non-Hodgkin's Lymphoma or T-cell Lymphoma

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    2013-01-22

    Angioimmunoblastic T-cell Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma; Waldenström Macroglobulinemia

  6. Primary extranodal lymphomas - spectrum of distribution and morphology with immunophenotyping: A 3-year institutional study

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    Chinnam Aparna

    2015-01-01

    Full Text Available Background: Malignant lymphomas arising in extranodal sites are intriguing. The histological types of lymphomas vary from one site to another. This study is undertaken to diagnose and categorize extranodal lymphomas using histochemistry and immunohistochemistry (IHC. Materials and Methods: Formalin processed paraffin blocks and hematoxylin and eosin stained sections were used for routine histology. IHC was done in all cases. Results: We have encountered 31 cases of extra nodal lymphomas over a period of 3 years. The tumors occurred at different sites, including brain, nasopharynx, nose, gastrointestinal tract, thyroid, bone, testis, breast, lung, vagina, and skin. Majority of the cases were B-cell lymphomas, while four cases were T-cell lymphomas. Among the B-cell lymphomas diffuse large B-cell lymphoma was the most common variant. Conclusion: This study reiterates the key role of IHC particularly when the differential diagnosis includes an undifferentiated epithelial malignancy and a high-grade lymphoma in the extranodal sites as the treatment modalities and prognosis are different.

  7. Extranodal Natural Killer/T-cell lymphoma, nasal type: ‘midline lethal granuloma.’ A case report

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    Tlholoe Martha M

    2013-01-01

    Full Text Available Abstract Extranodal natural killer/T cell lymphoma, nasal type, is a non-Hodgkin lymphoma, most commonly affecting the nasal cavity, paranasal sinuses and nasopharynx. Clinically it is characterised by destruction of facial tissues, commencing in the midline. In most cases it arises from malignant transformation of natural killer cells (NK; sometimes from malignant transformation of cytotoxic T cells. Extranodal NK/T cell lymphoma, nasal type, is rare, but even more rare in black persons. The purpose of this article is to report a severe case of extranodal NK/T cell lymphoma, nasal type, in an elderly black male.

  8. Prognostic CT and MR imaging features in patients with untreated extranodal non-Hodgkin lymphoma of the head and neck region

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    Zhou, Cuiping; Lan, Bowen; Liao, Junjie [Huizhou Central Municipal Hospital, Department of Radiology, Huizhou, Guangdong (China); Duan, Xiaohui; Shen, Jun [SunYat-Sen University, Department of Radiology, Sun Yat-Sen Memorial Hospital, Guangzhou, Guangdong (China)

    2015-10-15

    To determine the prognostic CT and MR imaging features of extranodal non-Hodgkin lymphoma in the head and neck region. The clinical data and CT and MR imaging features of 59 patients with histologically confirmed extranodal non-Hodgkin lymphoma in the head and neck region were retrospectively reviewed. Subjects included 27 male and 32 female patients between 13 and 81 years of age, with a mean age of 60.3 years. The clinical outcomes were categorized according to whether relapse or metastasis occurred within 2 years after therapy. The association between the clinical outcome and radiologic factors including tumour size, margin, shape, local tumour invasiveness, regional lymph node involvement, number of involvement sites, and contrast enhancement patterns was determined using univariate and multiple logistic regression analysis. Radiologic factors including tumour size, margin, shape, and local tumour invasiveness were associated with poor clinical outcomes, as determined by univariate analysis (P < 0.05). Only the lesion margin category (ill-defined) remained an independent risk factor for clinical outcome in multivariate logistic regression analysis, with an OR of 8.14 (P < 0.05). Ill-defined margin of the primary lesion was indicative of unfavourable survival outcome for patients with extranodal non-Hodgkin lymphoma of the head and neck region. (orig.)

  9. Genetically Engineered Lymphocytes, Cyclophosphamide, and Aldesleukin in Treating Patients With Relapsed or Refractory Mantle Cell Lymphoma or Indolent B-Cell Non-Hodgkin Lymphoma

    Science.gov (United States)

    2014-08-04

    B-cell Chronic Lymphocytic Leukemia; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Splenic Marginal Zone Lymphoma; Waldenström Macroglobulinemia

  10. Arsenic Trioxide in Treating Patients With Relapsed or Refractory Lymphoma or Leukemia

    Science.gov (United States)

    2013-01-31

    Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Prolymphocytic Leukemia; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Splenic Marginal Zone Lymphoma; Waldenström Macroglobulinemia

  11. Monoclonal Antibody Therapy in Treating Patients With Chronic Lymphocytic Leukemia, Lymphocytic Lymphoma, Acute Lymphoblastic Leukemia, or Acute Myeloid Leukemia

    Science.gov (United States)

    2013-06-03

    Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Noncontiguous Stage II Marginal Zone Lymphoma; Noncontiguous Stage II Small Lymphocytic Lymphoma; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Splenic Marginal Zone Lymphoma; Stage III Marginal Zone Lymphoma; Stage III Small Lymphocytic Lymphoma; Stage IV Marginal Zone Lymphoma; Stage IV Small Lymphocytic Lymphoma

  12. Pembrolizumab Alone or With Idelalisib or Ibrutinib in Treating Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia or Other Low-Grade B-Cell Non-Hodgkin Lymphomas

    Science.gov (United States)

    2016-06-02

    Recurrent Chronic Lymphocytic Leukemia; Recurrent Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Lymphoplasmacytic Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Nodal Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Recurrent Splenic Marginal Zone Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Refractory Follicular Lymphoma; Refractory Lymphoplasmacytic Lymphoma; Refractory Nodal Marginal Zone Lymphoma; Refractory Small Lymphocytic Lymphoma; Refractory Splenic Marginal Zone Lymphoma; Richter Syndrome; Waldenstrom Macroglobulinemia

  13. Emerging role of infectious etiologies in the pathogenesis of marginal zone B-cell lymphomas.

    Science.gov (United States)

    Zucca, Emanuele; Bertoni, Francesco; Vannata, Barbara; Cavalli, Franco

    2014-10-15

    Extranodal marginal zone B-cell lymphomas of the mucosa-associated lymphoid tissue (MALT) arise from lymphoid populations that are induced by chronic inflammation in extranodal sites. The most frequently affected organ is the stomach, where MALT lymphoma is incontrovertibly associated with a chronic gastritis induced by a microbial pathogen, Helicobacter pylori. Gastric MALT lymphoma therefore represents a paradigm for evaluating inflammation-associated lymphomagenesis, which may lead to a deeper understanding of a possible etiologic association between other microorganisms and nongastric marginal zone lymphomas. Besides infectious etiology, chronic inflammation caused by autoimmune diseases, such as Sjögren syndrome or Hashimoto thyroiditis, can also carry a significant risk factor for the development of marginal zone lymphoma. In addition to the continuous antigenic drive, additional oncogenic events play a relevant role in lymphoma growth and progression to the point at which the lymphoproliferative process may eventually become independent of antigenic stimulation. Recent studies on MALT lymphomas have in fact demonstrated genetic alterations affecting the NF-κB) pathway, a major signaling pathway involved in many cancers. This review aims to present marginal zone lymphoma as an example of the close pathogenetic link between chronic inflammation and tumor development, with particular attention to the role of infectious agents and the integration of these observations into everyday clinical practice. See all articles in this CCR Focus section, "Paradigm Shifts in Lymphoma."

  14. Emerging Insights on the Pathogenesis and Treatment of Extranodal NK/T Cell Lymphomas (ENKTL)

    Science.gov (United States)

    Haverkos, Bradley M.; Coleman, Carrie; Gru, Alejandro A.; Pan, Zenggang; Brammer, Jonathan; Rochford, Rosemary; Mishra, Anjali; Oakes, Christopher C.; Baiocchi, Robert A.; Freud, Aharon G.; Porcu, Pierluigi

    2017-01-01

    Extranodal NK/T-cell lymphoma (ENKTL) is a rare aggressive extranodal non-Hodgkin lymphoma (NHL) universally associated with Epstein-Barr virus (EBV). ENKTL most commonly occurs in non-elderly immune competent males in Asia and South America. A number of antecedent lymphoproliferative disorders (LPDs) have been described in Asian and South American patients, but the majority of Caucasian ENKTL patients have no known preceding LPD or underlying immunodeficiency. Other than EBV, no environmental or extrinsic factor has been implicated in oncogenesis. The precise mechanisms by which EBV infects NK or T cells and the virus’ role in the pathogenesis of ENKTL have not been fully deciphered. However, a number of recent discoveries including disturbances in cell signaling and mutations in tumor suppressor genes have been identified, which are providing insights into the pathogenesis of ENKTL. In this review, we highlight the molecular, viral, and genetic underpinnings of ENKTL and discuss potential therapeutic implications. PMID:28472613

  15. Everolimus and Lenalidomide in Treating Patients With Relapsed or Refractory Non-Hodgkin or Hodgkin Lymphoma

    Science.gov (United States)

    2016-04-18

    Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Peripheral T-cell Lymphoma; Post-transplant Lymphoproliferative Disorder; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Splenic Marginal Zone Lymphoma; Waldenstrom Macroglobulinemia

  16. Extranodal diffuse non hodgkin lymphoma in the thigh

    Directory of Open Access Journals (Sweden)

    Bölke E

    2010-08-01

    Full Text Available Abstract Diffuse large B-cell lymphoma usually starts as a rapidly growing mass in an internal lymph node and can grow in other areas such as the bone or intestines. About 1/3 of these lymphomas are confined to one part of the body when they are localized. In the case of a 78-year-old man, an extensive tumour was located on the right thigh. Biopsies of the tumour revealed diffuse proliferation of large lymphoid cells which have totally affected the normal architecture of striated muscle. The patient received multimodality treatment including chemotherapy of the CHOP regimen and adjuvant radiotherapy. Despite this being a fast growing lymphoma, about 3 out of 4 people will have no signs of disease after initial treatment, and about half of all people with this lymphoma are cured with therapy.

  17. Non-Hodgkin lymphoma: computed tomographic demonstration of unusual extranodal involvement.

    Science.gov (United States)

    Glazer, H S; Lee, J K; Balfe, D M; Mauro, M A; Griffith, R; Sagel, S S

    1983-10-01

    With the advent of computed tomography, lymphomatous involvement of sites other than lymph nodes is being seen with increasing frequency. Review of computed tomographic scans in 400 patients with newly diagnosed or recurrent non-Hodgkin lymphoma revealed 37 patients to have involvement of 56 unusual sites below the diaphragm: psoas/iliacus muscle (16 patients), kidney (13 patients), pancreas (5 patients), adrenal (4 patients), skin/subcutaneous tissue (4 patients), abdominal wall musculature (4 patients), peritoneum (4 patients), omentum (3 patients), and female reproductive tract (3 patients). These were mostly seen in patients with lymphomas of diffuse architecture, especially diffuse histiocytic lymphoma. Concomitant retroperitoneal and/or mesenteric adenopathy was very common; extranodal involvement was rarely the only site of initial or recurrent lymphoma.

  18. Diffuse large B-cell lymphoma: clinical implications of extranodal versus nodal presentation--a population-based study of 1575 cases

    DEFF Research Database (Denmark)

    Møller, Michael B; Pedersen, Niels T; Christensen, Bjarne E

    2004-01-01

    Differences in genetic origin between nodal and extranodal diffuse large B-cell lymphomas (DLBCL) exist. Using population-based data from the registry of the Danish Lymphoma Group, the present study is the first to analyse clinical implications of nodal versus extranodal presentation of DLBCL....... Of 4786 newly diagnosed non-Hodgkin's lymphoma patients in a 16-year period, 1575 (33%) had DLBCL. The annual incidence rate was 2.9 per 100 000; 40% were extranodal. The clinical profile of patients with extranodal DLBCL was different from the nodal DLBCL patients. Extranodal DLBCL was associated...... with older age and poorer performance score, but also lower tumour burden. In extranodal DLBCL, 51% of the cases were stage I and 36% were stage IV, whereas the patients were relatively equally distributed between the four stages in nodal DLBCL. For stage I patients, extranodal DLBCL was independently...

  19. Primary Extranodal Lymphomas of Lip – A Rare Manifestation in Sjogren’s Syndrome

    Science.gov (United States)

    V, Shwetha; B.K, Yashoda Devi; Mysorekar, Vijaya V; Kamath, Namrata P

    2014-01-01

    Sjogren’s syndrome (SS) is a chronic autoimmune disorder which is characterized by lymphocyte-mediated destruction of exocrine glands, which produces the classical symptoms of dry eyes and dry mouth, which is referred to as primary SS or the Sicca complex. When it is associated with another autoimmune disease such as rheumatoid arthritis or lupus erythematosus, the condition is termed as secondary SS. One of the known major complications in patients with Sjogren’s syndrome is the occurrence of Non-Hodgkin’s lymphoma of B cell type. It is not uncommon for malignant lymphomas to occur in head and neck region at nodal and sometimes, extranodal sites. However, only rarely may they involve the oral cavity primarily. This case report describes a rare occurrence of isolated extranodal lymphomas in the upper and lower lips of a patient, which clinically resembled a mucocele, and eventually was diagnosed as lymphoma which was associated with Sjogren’s syndrome, thereby stressing the importance which was played by an oral diagnostician. PMID:24783157

  20. Rituximab, Cyclophosphamide, Bortezomib, and Dexamethasone in Treating Patients With Relapsed or Refractory Low-Grade Follicular Lymphoma, Waldenstrom Macroglobulinemia, or Mantle Cell Lymphoma

    Science.gov (United States)

    2016-04-13

    Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Nodal Marginal Zone Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Splenic Marginal Zone Lymphoma; Waldenstrom Macroglobulinemia

  1. Rituximab and Interleukin-12 in Treating Patients With B-Cell Non-Hodgkin's Lymphoma

    Science.gov (United States)

    2013-08-23

    Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma

  2. Extranodal Natural Killer/T-Cell Lymphoma of the Nasal Type with Skin Metastases

    Directory of Open Access Journals (Sweden)

    Fadi Al Akhrass

    2016-01-01

    Full Text Available Extranodal natural killer/T-cell lymphoma (ENKL of the nasal type is a rare, clinically aggressive disease. ENKL of the nasal type is often localized in the upper aerodigestive tract, including the nasal cavity, nasopharynx, paranasal sinuses, tonsils, hypopharynx and larynx, and usually presents as stage I/II. Extranasal involvement can occur, and a common site of extranasal involvement or metastatic disease includes the skin. Identifying skin metastases is important for the appropriate staging and treatment. We report a case of ENKL of the nasal type that presented with localized disease and subsequent skin lesions that were consistent with skin metastases.

  3. Multifocal Extranodal Involvement of Diffuse Large B-Cell Lymphoma

    Directory of Open Access Journals (Sweden)

    Devrim Cabuk

    2013-01-01

    Full Text Available Endobronchial involvement of extrapulmonary malignant tumors is uncommon and mostly associated with breast, kidney, colon, and rectum carcinomas. A 68-year-old male with a prior diagnosis of colon non-Hodgkin lymphoma (NHL was admitted to the hospital with a complaint of cough, sputum, and dyspnea. The chest radiograph showed right hilar enlargement and opacity at the right middle zone suggestive of a mass lesion. Computed tomography of thorax revealed a right-sided mass lesion extending to thoracic wall with the destruction of the third and the fourth ribs and a right hilar mass lesion. Fiberoptic bronchoscopy was performed in order to evaluate endobronchial involvement and showed stenosis with mucosal tumor infiltration in right upper lobe bronchus. The pathological examination of bronchoscopic biopsy specimen reported diffuse large B-cell lymphoma and the patient was accepted as the endobronchial recurrence of sigmoid colon NHL. The patient is still under treatment of R-ICE (rituximab-ifosfamide-carboplatin-etoposide chemotherapy and partial regression of pulmonary lesions was noted after 3 courses of treatment.

  4. Bryostatin 1 Plus Vincristine in Treating Patients With Progressive or Relapsed Non-Hodgkin's Lymphoma After Bone Marrow or Stem Cell Transplantation

    Science.gov (United States)

    2013-01-09

    Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma

  5. Addition of rituximab to chlorambucil produces superior event-free survival in the treatment of patients with extranodal marginal-zone B-cell lymphoma: 5-year analysis of the IELSG-19 Randomized Study.

    Science.gov (United States)

    Zucca, Emanuele; Conconi, Annarita; Laszlo, Daniele; López-Guillermo, Armando; Bouabdallah, Reda; Coiffier, Bertrand; Sebban, Catherine; Jardin, Fabrice; Vitolo, Umberto; Morschhauser, Franck; Pileri, Stefano A; Copie-Bergman, Christiane; Campo, Elias; Jack, Andrew; Floriani, Irene; Johnson, Peter; Martelli, Maurizio; Cavalli, Franco; Martinelli, Giovanni; Thieblemont, Catherine

    2013-02-10

    Apart from localized gastric disease, there is no consensus on standard initial treatment of mucosa-associated lymphoid tissue lymphoma. The IELSG-19 study (Randomized Trial of Chlorambucil Versus Chlorambucil Plus Rituximab Versus Rituximab in MALT Lymphoma) was launched to compare chlorambucil alone versus chlorambucil plus rituximab in patients not previously given systemic anticancer therapy. Patients not responding to or not suitable for local therapy were eligible. In arm A, chlorambucil was given daily 6 mg/m(2) orally (PO) for 6 weeks. Responding patients and those with stable disease continued to be given daily chlorambucil 6 mg/m(2) PO for 14 consecutive days every 28 days for four cycles. In arm B, intravenous rituximab 375 mg/m(2) per day was added on days 1, 8, 15, 22, 56, 84, 112, and 140. After completion of the planned accrual, the protocol was amended to introduce a third arm with rituximab alone. We report the planned final analysis of the first two arms (113 patients in arm A and 114 in arm B). At a median follow-up of 62 months, the 5-year event-free survival (EFS) was significantly better for the patients treated in arm B (68% v 50%; P = .002) who, despite similar overall response rates (90% v 87%), achieved a higher complete remission rate (78% v 65%; P = .025). Progression-free survival was also improved but it did not reach statistical significance (P = .057). Five-year overall survival (OS) was 89% in both arms. Both treatments were well tolerated without unexpected toxicities. Both treatments were active; the better response rate and EFS obtained with the addition of rituximab did not translate into improved OS.

  6. Glomerulonephritis associated with marginal zone B-cell lymphoma: clinical, pathological characteristics of renal injury and treatment (clinical cases

    Directory of Open Access Journals (Sweden)

    B. T. Dzhumabaeva

    2015-01-01

    Full Text Available Glomerulonephritis associated with marginal zone B-cell lymphoma at the onset of disease is rarely diagnosed. In this article we reported two patient of the extranodal marginal zone B-cell lymphoma with kidney damage. The first patient with the extranodal marginal zone B-cell lymphoma involved the stomach, lymph nodes, bone marrow and associated with mesangioproliferative glomerulonephritis and renal failure. The second patient with the splenic form of marginal zone B-cell lymphoma associated with fibrillary glomerulonephritis and hepatitis C and involve the lymph nodes, liver, bone marrow, and synthesis monoclonal immunoglobulin (IgMκ, cryoglobulin type II. Glomerulonephritis of the both cases were established on the renal biopsies by the morphological investigation, immunofluorescence, and electron microscopy.Both patients received therapy with bendamustine and rituximab, which has resulted in complete remission for lymphatic tumors and improve of kidney function. Overall and event-free survival in the first case corresponds to 21 and 16 months, the second 29 and 20, respectively.These cases illustrates that the kidney may be initially involved by extranodal marginal zone B-cell lymphoma, and the need for expanded investigation of the possible dissemination. Combination of bendamustine and rituximab were effective and safety treatment in these cases.

  7. Extranodal non-Hodgkin's lymphoma of the oral tissues. An analysis of 20 cases.

    Science.gov (United States)

    Slootweg, P J; Wittkampf, A R; Kluin, P M; de Wilde, P C; van Unnik, J A

    1985-04-01

    A series of 20 patients with extra-nodal non-Hodgkin's lymphoma (ENHL) of the oral cavity was analysed with the emphasis on histopathological variability and prognostic factors. The current diagnostic schemes as devised for nodal NHL proved also to be useful in diagnosing ENHL in the oral cavity. With respect to histopathology, intra-oral ENHL differs from nodal NHL in a lower incidence of nodular growth pattern and a relative predominance of the lymphoma sub-type with large vesicular indented nuclei. These are features, however, that are shared with ENHL in other body sites and thus are not unique to the oral location. Another salient histological feature was the presence of proliferating bizarre spindle cells with formation of whorling bundles of reticulin, thus creating a pseudosarcomatous growth pattern in some cases. The clinical stage proved to be the main discriminating factor between those who survived and those who died of their lymphoma. Of the patients who were in stage IE on admission, 70% survived as opposed to only 20% of those who were in stage II or IV. A better prognosis for cases with soft tissue involvement as opposed to intraosseous lymphoma is probably due to a consistently lower clinical stage in the former group. The prognostic value of the clinical stage emphasizes the importance of adequate clinical staging procedures.

  8. Modern Radiation Therapy for Extranodal Lymphomas: Field and Dose Guidelines From the International Lymphoma Radiation Oncology Group

    Energy Technology Data Exchange (ETDEWEB)

    Yahalom, Joachim, E-mail: yahalomj@mskcc.org [Department of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, New York (United States); Illidge, Tim [Institute of Cancer Sciences, University of Manchester, Manchester Academic Health Sciences Centre, The Christie National Health Service Foundation Trust, Manchester (United Kingdom); Specht, Lena [Department of Oncology and Hematology, Rigshospitalet, University of Copenhagen, Copenhagen (Denmark); Hoppe, Richard T. [Department of Radiation Oncology, Stanford University, Palo Alto, California (United States); Li, Ye-Xiong [Department of Radiation Oncology, Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing (China); Tsang, Richard [Department of Radiation Oncology, Princess Margaret Hospital, University of Toronto, Toronto, Ontario (Canada); Wirth, Andrew [Division of Radiation Oncology, Peter MacCallum Cancer Institute, St. Andrews Place, East Melbourne (Australia)

    2015-05-01

    Extranodal lymphomas (ENLs) comprise about a third of all non-Hodgkin lymphomas (NHL). Radiation therapy (RT) is frequently used as either primary therapy (particularly for indolent ENL), consolidation after systemic therapy, salvage treatment, or palliation. The wide range of presentations of ENL, involving any organ in the body and the spectrum of histological sub-types, poses a challenge both for routine clinical care and for the conduct of prospective and retrospective studies. This has led to uncertainty and lack of consistency in RT approaches between centers and clinicians. Thus far there is a lack of guidelines for the use of RT in the management of ENL. This report presents an effort by the International Lymphoma Radiation Oncology Group (ILROG) to harmonize and standardize the principles of treatment of ENL, and to address the technical challenges of simulation, volume definition and treatment planning for the most frequently involved organs. Specifically, detailed recommendations for RT volumes are provided. We have applied the same modern principles of involved site radiation therapy as previously developed and published as guidelines for Hodgkin lymphoma and nodal NHL. We have adopted RT volume definitions based on the International Commission on Radiation Units and Measurements (ICRU), as has been widely adopted by the field of radiation oncology for solid tumors. Organ-specific recommendations take into account histological subtype, anatomy, the treatment intent, and other treatment modalities that may be have been used before RT.

  9. Malignant lymphoma of the conjunctiva

    DEFF Research Database (Denmark)

    Kirkegaard, Marina M; Coupland, Sarah E; Prause, Jan U;

    2015-01-01

    Conjunctival lymphomas constitute 25% of all ocular adnexal lymphomas. The majority are B-cell non-Hodgkin lymphomas (NHLs) (98%), whereas conjunctival T-cell NHLs are rare (2%). The most frequent subtype of conjunctival B-cell lymphoma is extranodal marginal zone lymphoma (EMZL; 81%), followed b...

  10. Extranodal NK/T-cell lymphoma presenting with primary cardiac involvement

    Directory of Open Access Journals (Sweden)

    Lisa M. Lepeak

    2011-08-01

    Full Text Available Primary cardiac lymphoma is extremely uncommon. We report a case of a 54 year old Caucasian male with a history of non-small cell lung cancer treated by surgical resection who presented with chest pain and dyspnea on exertion. Computerized tomography (CT imaging confirmed a 7.8¥3.8 cm right atrial soft tissue mass infiltrating the lateral wall of the right atrium, and a 5 cm pericardiophrenic mass. Echocardiography confirmed a moderate pericardial effusion without tamponade physiology. Percutaneous biopsy of the pericardiophrenic mass revealed pathologic features diagnostic of NK/T-cell lymphoma. He received CHOP chemotherapy with some improvement in symptoms, but experienced radiographic progression after 2 cycles. He received palliative involved field radiotherapy but developed new sites of progressive disease within the abdomen and died shortly after completing radiotherapy. NK/T-cell lymphomas are aggressive tumors that may present with unusual extranodal disease sites. Prompt diagnosis with consideration for referral to a specialty center with experience in treatment of these rare tumors may offer the greatest potential for improving treatment outcomes.

  11. Genetically Modified Peripheral Blood Stem Cell Transplant in Treating Patients With HIV-Associated Non-Hodgkin or Hodgkin Lymphoma

    Science.gov (United States)

    2015-05-06

    Adult Nasal Type Extranodal NK/T-cell Lymphoma; AIDS-related Diffuse Large Cell Lymphoma; AIDS-related Diffuse Mixed Cell Lymphoma; AIDS-related Diffuse Small Cleaved Cell Lymphoma; AIDS-related Immunoblastic Large Cell Lymphoma; AIDS-related Lymphoblastic Lymphoma; AIDS-related Peripheral/Systemic Lymphoma; AIDS-related Small Noncleaved Cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; HIV-associated Hodgkin Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Stage I AIDS-related Lymphoma; Stage II AIDS-related Lymphoma; Stage III AIDS-related Lymphoma; Stage IV AIDS-related Lymphoma; T-cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenström Macroglobulinemia

  12. Nasal Type Extranodal Natural Killer/T (NK/T) Cell Lymphoma Presenting as Periorbital Cellulitis: A Case Report

    Science.gov (United States)

    Shawabkeh, Ma’in Ali Al; Sulaiti, Mansour Al; Sa’ey, Hamad Al; Ganesan, Shanmugam

    2016-01-01

    Patient: Male, 25 Final Diagnosis: Nasal type • extra nodal NK/T-cell lymphoma Symptoms: Left periorbital swelling • redness • pain for 25 days • yellowish eye discharge associated • headache • fever Medication: — Clinical Procedure: — Specialty: Otolaryngology Objective: Unusual clinical course Background: Extranodal lymphoma of the paranasal sinuses is a rare clinical entity seen in only 5–8% of extranodal lymphomas of the head and neck. Nasal natural killer/T cell lymphoma (Nasal NKTCL), which is a subtype of peripheral T cell lymphoma, constitutes about 1.4% of all lymphomas. NKTCL is usually diagnosed at a late stage because it presents with nonspecific symptoms in the early stages. Case Report: We report the case of a 25-year-old male patient who presented with periorbital swelling treated as fungal sinusitis but proven to have NKTCL. We review the literature and discuss the clinical manifestations of the disease, its relation to EBV virus, the histological and radiological characteristics, the prognostic indicators, and treatment options. This case report shows physicians that NKTCL lymphoma can present as periorbital cellulitis, although few similar cases are found in the literature. Conclusions: NKTCL is a destructive midline tumor that should be kept in mind as a differential diagnosis of paranasal sinus lesions to help in early diagnosis, which can improve the prognosis. PMID:27932776

  13. A clinical study of chromosome translocations in extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue in Chinese patients%中国人黏膜相关淋巴组织结外边缘区淋巴瘤染色体易位的临床研究

    Institute of Scientific and Technical Information of China (English)

    董格红; 冯振博; 高子芬; 叶洪涛; 王桂秋; 宫丽平; 王晋芬; 莫祥兰; 刘红刚; 董丽娜; 周英琼; 张雪梅

    2009-01-01

    Objective To investigate the genetic aberrations in extranodal marginal zone lymphoma of mueosa-associated lymphoid tissue (MALT) lymphomas from different sites of the body in Chinese patients. Methods Two hundred and seventeen paraffin-embedded MALT lymphoma specimens from 11 major sites were studied with interphase fluorescence in situ hybridization (FISH) to detect t(11; 18) (q21;q21)/API2-MALT1, t(1; 14) (p22; q32)/IGH-BCL10, (14; 18) (q32; q21)/IGH-MALT1 and BCL6 gene involved chromosome translocations. Results These translocations were mutually exclusive and detected in 21% (46/217) of the cases, including t(11;18) (q21;q21) API2-MALT1 13% (29/217), t (1;14)(p22 ;q32) IGH-BCLIO in 1% (3/217), t(14;18) (q32;q21) IGH-MALT1 1% (2/217), BCL6 involved translocation in 2% (4/217) and IGH-unknown translocation partner in 4% (8/217). t(11; 18) (q21;q21)API2-MALT1 was found with the highest frequency in MALT lymphoma from lungs (47% , 8/17) and small intestine (29%, 4/14), followed by salivary gland (17%, 1/6), stomach (14%, 12/84) and ocular adnexae (6% , 4/68). t(1 ;14) (p22;q32) was only detected in lungs (12%, 2/17) and stomach (1%, 1/84). t(14;18) (q32;q21) was mainly detected in lungs (6%, 1/17) and ocular adnexae (2%, 1/68). BCL6 gene involved translocation was detected in salivary gland (17% , 1/6) and stomach (4%, 3/84). Conclusions It is demonstrated that the four translocatidns occur with markedly variable frequencies in MALT lymphoma of different sites in Chinese patients. The distributions of these chromosome translocations in Chinese patients are slightly different from those reported in western patients.%目的 探讨中国人不同部位黏膜相关淋巴组织结外边缘区淋巴瘤(MALTL)中分子遗传学异常的发生情况.方法 应用间期荧光原位杂交(FISH)方法,检测217例不同部位MALTL的t(11;18)(q21;q21)/API2-MALT1、t(1;14)(p22;q32)/IGH-BCL10、t(14;18)(q32;q21)/IGH-MALT1和涉及BCL6基因的染色体易位.结果 染色

  14. 胸腺原发黏膜相关淋巴组织结外边缘区B细胞淋巴瘤的临床病理特征%Clinicopathologic features of primary thymic extranodal marginal zone B-cell lymphoma of mucosaassociated lymphoid tissue type

    Institute of Scientific and Technical Information of China (English)

    孙璐; 石怀银; 韦立新

    2012-01-01

    Objective To study the clinicopathologic features of primary thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT). Methods The clinical and pathologic findings were evaluated in 3 cases of biopsy confirmed thymic MALT lymphoma. The clincopathologic features,treatment and prognosis were discussed and literatures reviewed.Results One male and two female patients presented with asymptomatic mediastinal masses with a history of Sj(o)gren syndrome.They were aged 36,35 and 41 years respectively,and only one patient had B symptoms.Grossly,all three tumors were encapsulated and had multiple variable-sized cysts on cut-surface.Histopathologically,the normal thymic lobular architecture was effaced by abnormal dense lymphoid infiltration. Prominent lymphoepithelial lesions were formed by centrocyte-like cells infiltrating and expanding Hassall's corpuscles and epithelial cyst lining. All cases showed apparent plasmacytic differentiation.Immunhistochemically,the tumor cells were positive for CD20,CD79a,bcl-2 and negative for CD3,CD5,cyclin D1,CD43,CD10,bcl-6,and CD23.The plasma cells showed kappa light chain restriction.Immunoglobulin heavy chain rearrangement in three cases was confirmed by PCR.All patients were at early stage and received routine chemotherapy with or without radiothcrapy aftcr surgical rcmoval.All patients achieved complete remission with 24,18 and 3 months follow-up,respectively. Conclusions Primary thymic MALT lymphoma may be a rare distinctive lymphoma. It can be diagnosed by HE and immunohistochemical study and should be differentiated from reactive lymphoid proliferation,other types of lymphoma and mediastinal thymoma.%目的 探讨胸腺原发黏膜相关淋巴组织(MALT)结外边缘区B细胞淋巴瘤的临床病理特征.方法 对3例经于术切除、病理证实的胸腺原发MALT淋巴瘤病例进行临床病理分析,并复习文献,讨论其临床、病理特征、治疗和预后.结果 3

  15. Rare case of Primary Pulmonary Extranodal Non-Hodgkin’s Lymphoma in a Patient with Sjogrens Syndrome: Role of FDG-PET/CT in the Initial Staging and Evaluating Response to Treatment

    Directory of Open Access Journals (Sweden)

    Gonca G. Bural

    2012-12-01

    Full Text Available A 64-year old woman with a long standing Sjogren’s syndrome was undergoing evaluation for renal transplant surgery when two pulmonary opacities were detected on chest CT. Subsequent biopsy revealed extranodal marginal B-cell non-Hodgkin’s lymphoma (NHL. An FDG-PET/CT scan was then performed which demonstrated isolated FDG avid pulmonary involvement. After therapy, FDG-PET/CT scans showed good response to treatment with near complete resolution of FDG avidity. This rare case illustrates the rare pulmonary manifestation of extranodal lymphoma in a patient with Sjogren’s syndrome and emphasizes the value of FDG PET/CT in the initial staging and evaluation of response to treatment, which has not previously been published. (MIRT 2012;21:117-120

  16. Extranodal NK/T-cell lymphoma of the nasal type with predominant T-cell markers: A rare subtype of rare disease entity

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    Ankur Nandan Varshney

    2015-01-01

    Full Text Available Extranodal NK/T-cell Lymphoma of nasal type is a rare and comparatively a new entry among group of Non-Hodgkin lymphomas. The disease is characterized by a clinically aggressive course with involvement of upper aero-digestive tract and classical immune-phenotyping with CD2, CD3 and CD56 positivity. Being a rare entity, treatment entities are yet not formulated in guidelines. We hereby report a case of extranodal NK/T-cell lymphoma with predominant T cell markers who was initially treated with CHOP regime of non-Hodgkin lymphoma and later successfully treated with SMILE regime.

  17. Primary bone marrow lymphoma: an uncommon extranodal presentation of aggressive non-hodgkin lymphomas.

    NARCIS (Netherlands)

    Martinez, A.; Ponzoni, M.; Agostinelli, C.; Hebeda, K.M.; Matutes, E.; Peccatori, J.; Campidelli, C.; Espinet, B.; Perea, G.; Acevedo, A.; Mehrjardi, A.Z.; Martinez-Bernal, M.; Gelemur, M.; Zucca, E.; Pileri, S.; Campo, E.; Lopez-Guillermo, A.; Rozman, M.

    2012-01-01

    Bone marrow involvement by lymphoma is considered a systemic dissemination of the disease arising elsewhere, although some tumors may arise primarily in the bone marrow microenvironment. Primary bone marrow lymphoma (PBML) is a rare entity whose real boundaries and clinicobiological significance are

  18. Interleukin-12 in Treating Patients With Previously Treated Non-Hodgkin's Lymphoma or Hodgkin's Disease

    Science.gov (United States)

    2015-04-14

    Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma; Waldenström Macroglobulinemia

  19. Simultaneous occurrence of follicular lymphoma and mixed-cellularity Hodgkin's lymphoma: lymph node and extranodal involvement

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    Grangeiro Maria do Patrocínio F.

    2004-01-01

    Full Text Available An unusual and well-characterised case of composite lymphoma in the spleen and lymph node is presented. The simultaneous occurrence of mixed-cellularity Hodgkin's lymphoma (HL and follicular non-Hodgkin's lymphoma (NHL was demonstrated in a 66-year-old man admitted in our Service with anaemia, hepatosplenomegaly and multiple abdominal lymph nodes. The morphological study of the spleen and lymph node of the splenic hilum showed an infiltrate composed of two distinct neoplasias. The liver was involved by NHL infiltrate and the peripancreatic lymph node exhibited HL. The Reed-Sternberg (RS cells expressed CD 15 and CD 30, whereas the NHL cells presented standard immunohistochemical features of follicular lymphoma. To our knowledge, this is the fifth case report of concurrent spleen involvement by composite lymphoma. The incidence, clinicopathological and immunohistochemical features of this rare association are discussed.

  20. Enteropathy Associated T Cell Lymphoma – A Case Report of An Uncommon Extranodal T Cell Lymphoma

    Science.gov (United States)

    V, Geetha; Kudva, Ranjini

    2014-01-01

    Enteropathy associated T cell lymphoma is a rare primary intestinal lymphoma. It is often, but not always associated with celiac disease. Intraepithelial T cells are postulated as the cell of origin. It is a rare disease accounting for fewer than 5% of all gastrointestinal tract lymphomas. Recent studies indicate that EATL consists of two diseases that are morphologically and genetically distinct and differ with respect to their frequency of association with celiac disease. Current WHO classification recognises two subtypes of EATL – type 1 (classic) and type 2, based on morphology and immunophenotype. EATL type 1 is a large cell lymphoma which is more common and is more commonly associated with celiac disease compared to type 2. Most common site of involvement is the small intestine. We report a case of EATL type 1, in a 62-year-old female patient who presented with features of intestinal obstruction. However, she did not have spruce like featutes. PMID:25478355

  1. Myeloid cell nuclear differentiation antigen is expressed in a subset of marginal zone lymphomas and is useful in the differential diagnosis with follicular lymphoma.

    Science.gov (United States)

    Metcalf, Ryan A; Monabati, Ahmad; Vyas, Monika; Roncador, Giovanna; Gualco, Gabriela; Bacchi, Carlos E; Younes, Sheren F; Natkunam, Yasodha; Freud, Aharon G

    2014-08-01

    The diagnosis of marginal zone lymphomas (MZL) is challenged by the lack of specific markers that distinguish them from other low-grade non-Hodgkin B-cell lymphomas. Myeloid cell nuclear differentiation antigen (MNDA) is a nuclear protein that labels myelomonocytic cells as well as B lymphocytes that localize to the marginal zone areas of splenic white pulp. We evaluated MNDA expression in a large series of B-cell lymphomas to assess the sensitivity and specificity of this antigen for the characterization of MZL. A total of 440 tissue sections containing extramedullary B-cell lymphomas and 216 bone marrow biopsies containing atypical or neoplastic lymphoid infiltrates were stained for MNDA by immunohistochemistry. Among the extramedullary lymphoma cases, approximately 67% of nodal MZL, 61% of extranodal MZL, and 24% of splenic MZL expressed MNDA. MNDA was also infrequently expressed in other B-cell neoplasms including mantle cell lymphoma (6%), chronic lymphocytic leukemia/small lymphocytic lymphoma (13%), follicular lymphoma (FL) (4%), lymphoplasmacytic lymphoma (25%), and diffuse large B-cell lymphoma (3%). In contrast, MNDA was only expressed in 2.3% of all bone marrow biopsies involved by lymphoid infiltrates, including 2 cases of FL and one case of MZL. Collectively, these data support the inclusion of MNDA in the diagnostic evaluation of extramedullary B-cell lymphomas, particularly those in which the differential diagnosis is between low-grade FL and MZL.

  2. Lymphoma of the eyelid

    DEFF Research Database (Denmark)

    Svendsen, Frederik H; Heegaard, Steffen

    2017-01-01

    Lymphoma of the eyelid constitutes 5% of ocular adnexal lymphoma. In previously published cases, 56% of lymphomas of the eyelid are of B-cell origin and 44% are of T-cell origin. The most frequent B-cell lymphomas are extranodal marginal zone lymphoma (27 cases-14%) and diffuse large B-cell lymph......Lymphoma of the eyelid constitutes 5% of ocular adnexal lymphoma. In previously published cases, 56% of lymphomas of the eyelid are of B-cell origin and 44% are of T-cell origin. The most frequent B-cell lymphomas are extranodal marginal zone lymphoma (27 cases-14%) and diffuse large B...... chemotherapy with or without adjuvant treatment is the treatment of choice for high-grade or disseminated lymphomas. The majority of subtypes, especially low-grade subtypes, have a good prognosis with few recurrences or progression. Some subtypes, including mycosis fungoides, have a poorer prognosis...

  3. New prognostic model for extranodal natural killer/T cell lymphoma, nasal type.

    Science.gov (United States)

    Cai, Qingqing; Luo, Xiaolin; Zhang, Guanrong; Huang, Huiqiang; Huang, Hui; Lin, Tongyu; Jiang, Wenqi; Xia, Zhongjun; Young, Ken H

    2014-09-01

    Extranodal natural killer/T cell lymphoma, nasal type (ENKTL) is an aggressive disease with a poor prognosis, requiring risk stratification in affected patients. We designed a new prognostic model specifically for ENKTL to identify high-risk patients who need more aggressive therapy. We retrospectively reviewed 158 patients who were newly diagnosed with ENKTL. The estimated 5-year overall survival rate was 39.4 %. Independent prognostic factors included total protein (TP) 100 mg/dL, and Korean Prognostic Index (KPI) score ≥2. We constructed a new prognostic model by combining these prognostic factors: group 1 (64 cases (41.0 %)), no adverse factors; group 2 (58 cases (37.2 %)), one adverse factor; and group 3 (34 cases (21.8 %)), two or three adverse factors. The 5-year overall survival (OS) rates of these groups were 66.7, 23.0, and 5.9 %, respectively (p KPI model alone (p KPI model alone.

  4. Prognostic significance of peripheral monocyte count in patients with extranodal natural killer/T-cell lymphoma.

    Science.gov (United States)

    Huang, Jia-Jia; Li, Ya-Jun; Xia, Yi; Wang, Yu; Wei, Wen-Xiao; Zhu, Ying-Jie; Lin, Tong-Yu; Huang, Hui-Qiang; Jiang, Wen-Qi; Li, Zhi-Ming

    2013-05-03

    Extranodal natural killer/T-cell lymphoma (ENKL) has heterogeneous clinical manifestations and prognosis. This study aims to evaluate the prognostic impact of absolute monocyte count (AMC) in ENKL, and provide some immunologically relevant information for better risk stratification in patients with ENKL. Retrospective data from 163 patients newly diagnosed with ENKL were analyzed. The absolute monocyte count (AMC) at diagnosis was analyzed as continuous and dichotomized variables. Independent prognostic factors of survival were determined by Cox regression analysis. The AMC at diagnosis were related to overall survival (OS) and progression-free survival (PFS) in patients with ENKL. Multivariate analysis identified AMC as independent prognostic factors of survival, independent of International Prognostic Index (IPI) and Korean prognostic index (KPI). The prognostic index incorporating AMC and absolute lymphocyte count (ALC), another surrogate factor of immune status, could be used to stratify all 163 patients with ENKL into different prognostic groups. For patients who received chemotherapy followed by radiotherapy (102 cases), the three AMC/ALC index categories identified patients with significantly different survivals. When superimposed on IPI or KPI categories, the AMC/ALC index was better able to identify high-risk patients in the low-risk IPI or KPI category. The baseline peripheral monocyte count is shown to be an effective prognostic indicator of survival in ENKL patients. The prognostic index related to tumor microenvironment might be helpful to identify high-risk patients with ENKL.

  5. Primary Hepatic Marginal Zone Lymphoma in a Patient with Chronic Hepatitis C.

    Science.gov (United States)

    Gherlan, George S; Stoia, Razvan; Enyedi, Mihaly; Dobrea, Camelia; Calistru, Petre I

    2016-09-01

    Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) is a low-grade malignant lymphoma that appears frequently in the stomach, but other sites can also be involved: the intestinal tract, lungs, head, neck, skin, thyroid, breasts and liver. Recently, epidemiological evidences support the idea that there is an association between hepatitis C and B-cell non-Hodgkin lymphomas (that include MALT as a subtype). Primary non-Hodgkin lymphomas confi ned only to the liver are very rare (only 0.016% of all cases of all non-Hodgkin's lymphomas) and MALT is not the most frequent type. We present the case of a male patient, age 62, known with chronic hepatitis C, previously relapser a" er a 72 week treatment with peg-interferon alfa and ribavirin that was diagnosed at three years a" er the relapse with multiple focal liver lesions. One of the tumors was surgically removed and the histological exam performed demonstrated an extranodal marginal zone lymphoma with small B-cell with plasmacytoid diff erentiation confi ned only to the liver. Direct acting antiviral (DAA) therapy was started, but the virologic clearance was not obtained by week 10, leading to a change of DAA regimen at week 12. The antiviral therapy was continued until week 24. Imaging showed an increase in number and size of the focal lesions until week 12. At week 12 chemo- and immune-therapy was started with bendamustine and rituximab. A" erwards the evolution was favorable, the patient being now in complete remission and with undetectable viral load.

  6. Extranodal natural killer cell/t-cell lymphoma, nasal type, presenting as cutaneous nodules and a small-bowel perforation.

    Science.gov (United States)

    Agarwal, Priyanka; Ruzinova, Marianna B; Harris, Marian H; Qureshi, Abrar A; Stebbins, William G

    2010-02-01

    Extranodal natural killer (NK) cell/T-cell lymphoma, nasal type, is a rare aggressive neoplasm, most commonly presenting as a destructive lesion in the nasal cavity and nasopharynx in middle-aged to older adults. About one third of cases present in an extranasal location, commonly involving skin and gastrointestinal tract, and usually occur in the absence of superficial lymphadenopathy. Diagnosis of this malignancy can be missed given its rarity and heterogeneous presentation. We describe a patient with an extranodal NK cell/T-cell lymphoma, nasal type, who was initially diagnosed and treated for a presumed Mycobacterium marinum infection, after biopsies were unrevealing. However, after more serious complications developed, repeat biopsy was performed. An atypical lymphocytic infiltrate was noted, with cells being positive for NK cell/T-cell markers CD2, CD7, and CD3 (subset), as well as for cytotoxic lymphocyte markers perforin, T-cell intracellular antigen, and CD56. In situ hybridization for Epstein-Barr virus-encoded RNA was also positive. This case demonstrates an important diagnostic pitfall of confusing cutaneous involvement by an aggressive NK cell/T-cell lymphoma with an antibiotic-resistant infection. Repeat biopsies and close clinicopathologic correlation are essential for establishment of correct diagnosis.

  7. Primary extranodal soft-tissue B-cell lymphoma with abundant immunoglobulin inclusions mimicking adult rhabdomyoma: a case report

    Directory of Open Access Journals (Sweden)

    Wang Zhe

    2011-02-01

    Full Text Available Abstract Introduction Immunoglobulin inclusions are found in B-cell neoplasms as well as in crystal-storing histiocytosis associated with B-cell lymphoproliferative disorders. At times, the deposits may be so profound as to obscure the diagnosis and may even lead to misdiagnosis. We report one case of low-grade extranodal lymphoplasmacytic lymphoma with abundant immunoglobulin inclusions and emphasize the need for immunophenotyping and molecular assay to make the right decision in diagnosis. To the best of our knowledge, this is the first report of extranodal B-cell lymphoma with abundant intracellular immunoglobulin accumulation. Case presentation A 62-year-old Asian man from China presented with a 13-year history of a right shoulder mass with recent ongoing pain. A desmoplastic fibroma located in the posterior muscles of the neck was suggested by magnetic resonance imaging, and extended local excision was performed. A biopsy, however, revealed large, isolated rhabdoid cells in a diffuse pattern with mild atypia and eosinophilic cytoplasm. Clustered lymphoid cells were interspersed among these cells. The diagnosis was initially suggested to be adult rhabdomyoma. The final diagnosis of lymphoma was made after immunohistochemical, ultrastructural and molecular studies. Conclusion We emphasize this histopathologic and immunohistochemical finding because of the potential for confusion with other tumors or disorders, such as adult rhabdomyoma or crystal-storing histiocytosis.

  8. FISH analysis of MALT lymphoma-specific translocations and aneuploidy in primary cutaneous marginal zone lymphoma.

    NARCIS (Netherlands)

    Schreuder, M.I.; Hoefnagel, J.J.; Jansen, P.A.M.; Krieken, J.H.J.M. van; Willemze, R.; Hebeda, K.M.

    2005-01-01

    Primary cutaneous marginal zone lymphomas (PCMZL) share histological and clinical characteristics with mucosa-associated lymphoid tissue (MALT) lymphomas suggesting a common pathogenesis. A number of recurrent structural and numerical chromosomal aberrations have been described in MALT lymphoma, but

  9. FISH analysis of MALT lymphoma-specific translocations and aneuploidy in primary cutaneous marginal zone lymphoma.

    NARCIS (Netherlands)

    Schreuder, M.I.; Hoefnagel, J.J.; Jansen, P.A.M.; Krieken, J.H.J.M. van; Willemze, R.; Hebeda, K.M.

    2005-01-01

    Primary cutaneous marginal zone lymphomas (PCMZL) share histological and clinical characteristics with mucosa-associated lymphoid tissue (MALT) lymphomas suggesting a common pathogenesis. A number of recurrent structural and numerical chromosomal aberrations have been described in MALT lymphoma, but

  10. Pyoderma gangrenosum preceding the onset of extranodal natural killer/T-cell lymphoma

    Science.gov (United States)

    Yang, Ting-Hua; Hu, Chung-Hong; Tsai, Hsiou-Hsin

    2016-01-01

    Abstract Introduction: Pyoderma gangrenosum (PG) is a neutrophilic dermatosis that may be associated with systemic diseases. The association of PG with lymphoid malignancies has rarely been reported. Extranodal natural killer/T-cell lymphoma (ENKTL) is a rare but aggressive entity with a poor prognosis. Here, we report the case of a patient who had idiopathic PG refractory to systemic steroids and subsequently developed ENKTL. Case report: A 70-year-old man presented with a 2-month history of intermittent fever and multifocal painful papules, plaques, and ulcerations on his extremities. The histological and culture results of the lesions were consistent with those of PG. A thorough work-up was performed and did not demonstrate any underlying systemic diseases including malignancy. The PG lesions were refractory to systemic steroid therapy. An enlarging nodule was observed over his right infraorbital area 4 months after the onset of the skin eruptions. The nodule was later biopsied and diagnosed as ENKTL by using histopathological and immunohistochemical studies. Fludeoxyglucose positron emission tomography/computed tomography revealed multiple intense fludeoxyglucose-avid masses in the bones and lungs, suggesting multiorgan metastases. The patient rejected chemotherapy and died 4 weeks after the diagnosis. Conclusion: The present case indicates that in any patient with idiopathic PG refractory to conventional therapy, the presence of any underlying disease or malignancy must be thoroughly evaluated. The present case serves as a reminder that when assessing patients with PG, clinicians should increase their awareness regarding the delayed association with malignancy, even in the absence of a concomitant systemic disease at presentation. Furthermore, the prompt evaluation of any suspicious lesions in the context of PG for the possibility of a malignant nature can improve the prognosis, particularly in cases of aggressive malignancy. Understanding the cutaneous

  11. Radiographic Enlargement of Mandibular Canal as an Extranodal Primary Non-Hodgkin’s Lymphoma Early Sign in an Asymptomatic Patient

    Directory of Open Access Journals (Sweden)

    Luciana Munhoz

    2017-01-01

    Full Text Available Non-Hodgkin’s lymphoma (NHL is a lymphoproliferative disorder, from a subgroup of heterogeneous hematologic malignancies; the term “extranodal” refers to malignant involvement of tissues other than lymph nodes, tonsils, spleen, pharyngeal lymphatic ring, or thymus. Only 0.6% of all NHL are at mandible alone, and it may involve the inferior alveolar canal. We describe a case of bilateral enlargement of the mandibular canal without symptomatology, which was shown in a panoramic radiograph and cone beam computed tomography in a rehabilitation routine exam, as an early sign of primary extranodal NHL.

  12. Genetic alterations in systemic nodal and extranodal non-cutaneous lymphomas derived from mature T cells and natural killer cells.

    Science.gov (United States)

    Boi, Michela; Stathis, Anastasios; Zucca, Emanuele; Inghirami, Giorgio; Bertoni, Francesco

    2012-08-01

    Mature (peripheral) T-cell and natural killer (NK)-cell lymphomas comprise a series of rather different neoplasms. Based on morphologic, immunophenotypic, genetic, and clinical data, the World Health Organization classification recognizes more than 20 entities or provisional entities. The variable clinical presentations, the objective recognition and pathological stratification, the difficulties regarding treatment, and the hardly predictable response to therapy indicate that the management of these entities requires novel tools. In contrast to B-cell lymphomas or precursor T-cell neoplasms, few recurrent translocations have been identified so far in T-cell non-Hodgkin's and NK-cell lymphomas. Additionally, some of the entities recognized by the World Health Organization classification are very rare and very scarce molecular data are available for T-cell lymphomas. Here, we have reviewed published reports focusing on the genetic lesions and gene expression profiling underlying systemic nodal and extranodal non-cutaneous mature T-cell and NK-cell lymphomas. We also provide a summary of new agents in clinical development and outline some future directions.

  13. Minimal change disease: an unusual presentation of marginal zone MALT lymphoma.

    Science.gov (United States)

    Inayat, Faisal; Law, Jason K; Myers-Gurevitch, Patricia M; Seshan, Surya V; Perlman, Alan S

    2016-03-01

    Minimal change disease (MCD) in association with low-grade extra-nodal marginal zone B-cell lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT) (MALT lymphoma) is a rare clinicopathologic entity. We report a 68-year-old male who presented with nephrotic range proteinuria as the first manifestation of underlying MZL, confirmed with standard set of investigations. Being a steroid non-responder, he was treated with rituximab demonstrating a marked response with resolution of proteinuria. However, he relapsed after 3 months. Upon relapse, a combination of rituximab and bendamustine (R-Benda) was initiated achieving sustained resolution of proteinuria. No additional treatment was administered and the proteinuria has remained in remission for over a year.

  14. Iodine I 131 Tositumomab and Fludarabine Phosphate in Treating Older Patients Who Are Undergoing an Autologous or Syngeneic Stem Cell Transplant for Relapsed or Refractory Non-Hodgkin's Lymphoma

    Science.gov (United States)

    2014-08-04

    Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Splenic Marginal Zone Lymphoma; Waldenström Macroglobulinemia

  15. Pathogenesis of splenic marginal zone lymphoma

    Directory of Open Access Journals (Sweden)

    Ming-Qing Du

    2015-11-01

    Full Text Available Splenic marginal zone lymphoma (SMZL is a distinct low grade B-cell lymphoma with an immunophenotype similar to that of splenic marginal zone B-cells. Like the normal splenic marginal zone B-cells, SMZLs also show variable features in somatic mutations of their rearranged immunoglobulin genes, with ∼90% of cases harbouring somatic mutations but at remarkably variable degrees, suggesting that SMZL may have multiple cell of origins, deriving from the heterogeneous B-cells of the splenic marginal zone. Notably, ∼30% of SMZLs show biased usage of IGHV1-2*04, with the expressed BCR being potentially polyreactive to autoantigens. Recent exome and targeted sequencing studies have identified a wide spectrum of somatic mutations in SMZL with the recurrent mutations targeting multiple signalling pathways that govern the development of splenic marginal zone B-cells. These recurrent mutations occur in KLF2 (20–42%, NOTCH2 (6.5–25%, NF-κB (CARD11 ∼7%, IKBKB ∼7%, TNFAIP3 7–13%, TRAF3 5%, BIRC3 6.3% and TLR (MYD88 5–13% signalling pathways. Interestingly, the majority of SMZL with KLF2 mutation have both 7q32 deletion and IGHV1-2 rearrangement, and these cases also have additional mutations in NOTCH2, or TNFAIP3, or TRAF3. There is a potential oncogenic cooperation among concurrent genetic changes, for example between the IGHV1-2 expressing BCR and KLF2 mutation in activation of the canonical NF-κB pathway, and between KLF2 and TRAF3 mutations in activation of the non-canonical NF-κB pathway. These novel genetic findings have provided considerable insights into the pathogenesis of SMZL and will stimulate the research in both normal and malignant marginal zone B-cells.

  16. Primary marginal zone B-cell lymphoma of appendix

    Directory of Open Access Journals (Sweden)

    Radha S

    2008-07-01

    Full Text Available Primary lymphomas of appendix are extremely rare tumors. The first case of primary lymphoma of appendix was reported by Warren in the year 1898. Incidence of primary lymphoma of appendix is 0.015% of all gastrointestinal lymphomas. This is a report of primary marginal zone B-cell lymphoma of appendix which presented as appendicular mass. As some cases are incidentally discovered, this case emphasizes that histological examination of all appendicectomy specimens is mandatory.

  17. Flavopiridol in Treating Patients With Relapsed or Refractory Lymphoma or Multiple Myeloma

    Science.gov (United States)

    2016-06-27

    Adult Lymphocyte Depletion Hodgkin Lymphoma; Adult Lymphocyte Predominant Hodgkin Lymphoma; Adult Mixed Cellularity Hodgkin Lymphoma; Adult Nodular Sclerosis Hodgkin Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Multiple Myeloma; Splenic Marginal Zone Lymphoma; Stage I Multiple Myeloma; Stage II Multiple Myeloma; Stage III Multiple Myeloma; Waldenström Macroglobulinemia

  18. Serum levels of interleukin-9 correlate with negative prognostic factors in extranodal NK/T-cell lymphoma.

    Directory of Open Access Journals (Sweden)

    Jing Zhang

    Full Text Available Interleukin-9 (IL-9 is more functionally diverse than previously expected, especially with regards to lymphomagenesis. However, the relationship between IL-9 and the clinicopathological features of extranodal NK/T-cell lymphoma is less well established. Patients with this lymphoma in Sun Yat-Sen University Cancer Center between January 2003 and March 2013 were systematically reviewed in an intention-to-treat analysis. Baseline serum IL-9 levels were determined using sandwich enzyme-linked immunosorbent assays. A total of seventy-four patients were enrolled in this study. The mean concentration of serum IL-9 for all patients was 6.48 pg/mL (range: 1.38-51.87 pg/mL. Age, B symptoms and local lymph node involvement were found to be related to high serum IL-9 levels. Patients with low IL-9 levels tended to have higher rates of complete remission. Notably, the median progression-free survival (PFS and overall survival (OS were longer in the low IL-9 level group than in the high IL-9 level group (PFS: 68.7 months vs. 28.3 months, P<0.001; OS: 86 months vs. 42.8 months, P = 0.001. Multivariate analysis revealed independent prognostic factors for PFS. Similarly, high IL-9 levels (P = 0.003 and old age (P = 0.007 were independently predictive of shorter OS. Serum IL-9 is closely related to several clinical features, such as age, B symptoms and local lymph node involvement. It can also be a significant independent prognostic factor for extranodal NK/T-cell lymphoma, which suggests a role for IL-9 in the pathogenesis of this disease and offers new insight into potential therapeutic strategies.

  19. The genetics of nodal marginal zone lymphoma.

    Science.gov (United States)

    Spina, Valeria; Khiabanian, Hossein; Messina, Monica; Monti, Sara; Cascione, Luciano; Bruscaggin, Alessio; Spaccarotella, Elisa; Holmes, Antony B; Arcaini, Luca; Lucioni, Marco; Tabbò, Fabrizio; Zairis, Sakellarios; Diop, Fary; Cerri, Michaela; Chiaretti, Sabina; Marasca, Roberto; Ponzoni, Maurilio; Deaglio, Silvia; Ramponi, Antonio; Tiacci, Enrico; Pasqualucci, Laura; Paulli, Marco; Falini, Brunangelo; Inghirami, Giorgio; Bertoni, Francesco; Foà, Robin; Rabadan, Raul; Gaidano, Gianluca; Rossi, Davide

    2016-09-08

    Nodal marginal zone lymphoma (NMZL) is a rare, indolent B-cell tumor that is distinguished from splenic marginal zone lymphoma (SMZL) by the different pattern of dissemination. NMZL still lacks distinct markers and remains orphan of specific cancer gene lesions. By combining whole-exome sequencing, targeted sequencing of tumor-related genes, whole-transcriptome sequencing, and high-resolution single nucleotide polymorphism array analysis, we aimed at disclosing the pathways that are molecularly deregulated in NMZL and we compare the molecular profile of NMZL with that of SMZL. These analyses identified a distinctive pattern of nonsilent somatic lesions in NMZL. In 35 NMZL patients, 41 genes were found recurrently affected in ≥3 (9%) cases, including highly prevalent molecular lesions of MLL2 (also known as KMT2D; 34%), PTPRD (20%), NOTCH2 (20%), and KLF2 (17%). Mutations of PTPRD, a receptor-type protein tyrosine phosphatase regulating cell growth, were enriched in NMZL across mature B-cell tumors, functionally caused the loss of the phosphatase activity of PTPRD, and were associated with cell-cycle transcriptional program deregulation and increased proliferation index in NMZL. Although NMZL shared with SMZL a common mutation profile, NMZL harbored PTPRD lesions that were otherwise absent in SMZL. Collectively, these findings provide new insights into the genetics of NMZL, identify PTPRD lesions as a novel marker for this lymphoma across mature B-cell tumors, and support the distinction of NMZL as an independent clinicopathologic entity within the current lymphoma classification. © 2016 by The American Society of Hematology.

  20. Salvia Hispanica Seed in Reducing Risk of Disease Recurrence in Patients With Non-Hodgkin Lymphoma

    Science.gov (United States)

    2017-01-26

    Adult Nasal Type Extranodal NK/T-Cell Lymphoma; Adult T-Cell Leukemia/Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-Cell Lymphoma; B Lymphoblastic Leukemia/Lymphoma; Blastic Plasmacytoid Dendritic Cell Neoplasm; Burkitt Leukemia; Central Nervous System Lymphoma; Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma; Diffuse Large B-Cell Lymphoma; Enteropathy-Associated T-Cell Lymphoma; Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Grade 1 Follicular Lymphoma; Grade 2 Follicular Lymphoma; Grade 3 Follicular Lymphoma; Hepatosplenic T-Cell Lymphoma; Lymphoplasmacytic Lymphoma; Mantle Cell Lymphoma; Mediastinal (Thymic) Large B-Cell Lymphoma; Mycosis Fungoides; Nasal Type Extranodal NK/T-Cell Lymphoma; Nodal Marginal Zone Lymphoma; Peripheral T-Cell Lymphoma, Not Otherwise Specified; Post-Transplant Lymphoproliferative Disorder; Primary Cutaneous Anaplastic Large Cell Lymphoma; Primary Effusion Lymphoma; Sezary Syndrome; Splenic Marginal Zone Lymphoma; Subcutaneous Panniculitis-Like T-Cell Lymphoma; Systemic Anaplastic Large Cell Lymphoma; T Lymphoblastic Leukemia/Lymphoma; Transformed Recurrent Non-Hodgkin Lymphoma

  1. Lenalidomide With or Without Rituximab in Treating Patients With Progressive or Relapsed Chronic Lymphocytic Leukemia, Small Lymphocytic Lymphoma, Prolymphocytic Leukemia, or Non-Hodgkin Lymphoma Previously Treated With Donor Stem Cell Transplant

    Science.gov (United States)

    2017-07-24

    Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Prolymphocytic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; T-cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenström Macroglobulinemia

  2. Mutational Analysis of Extranodal NK/T-Cell Lymphoma Using Targeted Sequencing with a Comprehensive Cancer Panel

    Science.gov (United States)

    Choi, Seungkyu; Go, Jai Hyang; Kim, Eun Kyung; Lee, Hojung; Lee, Won Mi; Cho, Chun-Sung

    2016-01-01

    Extranodal natural killer (NK)/T-cell lymphoma, nasal type (NKTCL), is a malignant disorder of cytotoxic lymphocytes of NK or T cells. It is an aggressive neoplasm with a very poor prognosis. Although extranodal NKTCL reportedly has a strong association with Epstein-Barr virus, the molecular pathogenesis of NKTCL has been unexplored. The recent technological advancements in next-generation sequencing (NGS) have made DNA sequencing cost- and time-effective, with more reliable results. Using the Ion Proton Comprehensive Cancer Panel, we sequenced 409 cancer-related genes to identify somatic mutations in five NKTCL tissue samples. The sequencing analysis detected 25 mutations in 21 genes. Among them, KMT2D, a histone modification-related gene, was the most frequently mutated gene (four of the five cases). This result was consistent with recent NGS studies that have suggested KMT2D as a novel driver gene in NKTCL. Mutations were also found in ARID1A, a chromatin remodeling gene, and TP53, which also recurred in recent NGS studies. We also found mutations in 18 novel candidate genes, with molecular functions that were potentially implicated in cancer development. We suggest that these genes may result in multiple oncogenic events and may be used as potential bio-markers of NKTCL in the future. PMID:27729836

  3. Alisertib in Combination With Vorinostat in Treating Patients With Relapsed or Recurrent Hodgkin Lymphoma, B-Cell Non-Hodgkin Lymphoma, or Peripheral T-Cell Lymphoma

    Science.gov (United States)

    2016-07-12

    Adult B Acute Lymphoblastic Leukemia; Adult T Acute Lymphoblastic Leukemia; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-Cell Lymphoma; Chronic Lymphocytic Leukemia; Cutaneous B-Cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Hepatosplenic T-Cell Lymphoma; Intraocular Lymphoma; Lymphomatous Involvement of Non-Cutaneous Extranodal Site; Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma; Nodal Marginal Zone Lymphoma; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-Cell Leukemia/Lymphoma; Recurrent Cutaneous T-Cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides and Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Small Intestinal Lymphoma; Splenic Marginal Zone Lymphoma; T-Cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenstrom Macroglobulinemia

  4. Fasting blood glucose is a novel prognostic indicator for extranodal natural killer/T-cell lymphoma, nasal type

    Science.gov (United States)

    Cai, Q; Luo, X; Liang, Y; Rao, H; Fang, X; Jiang, W; Lin, T; Lin, T; Huang, H

    2013-01-01

    Background: Extranodal natural killer (NK)/T-cell lymphoma, nasal type (ENKTL) is an aggressive disease with poor prognosis, requiring risk stratification. However, the prognosis of ENKTL is not fully defined and needs supplementation. We hypothesised that fasting blood glucose (FBG) may be a new prognostic factor for ENKTL. Methods: We retrospectively analysed 130 patients newly diagnosed with ENKTL. Results: Both univariate analysis and multivariate analysis revealed that FBG >100 mg dl−1 was associated with a poor outcome. Patients with FBG >100 mg dl−1 at diagnosis had more adverse clinical features, achieved lower complete remission rates (P=0.003) and had worse overall survival (PKPI) scores with different survival outcomes (PKPI. PMID:23299534

  5. Primary extranodal non-Hodgkin's lymphoma of the lung presenting with bilateral, patchy infiltrates dramatically improving after corticosteroid therapy.

    Science.gov (United States)

    Boon, E S; Graal, M B; van Noord, J A

    1993-10-01

    A 63-year-old man was admitted to the hospital with fever and bilateral, peripheral infiltrates. Infectious disease and malignancy seemed to be excluded by fiberoptic diagnostic procedures. Subsequently, respiratory insufficiency developed, making open lung biopsy impossible. The diagnosis of bronchiolitis obliterans organizing pneumonia (BOOP) was strongly considered and treatment with corticosteroids was started; this led to dramatic clinical and radiologic improvement for a short time. Eventually, an open lung biopsy specimen disclosed primary extranodal non-Hodgkin's lymphoma of T-cell origin, immunoblastic, of high-grade malignancy according to the Kiel classification. After the first course of chemotherapy, total respiratory insufficiency developed and the patient died. This case is unique in a patient without AIDS.

  6. Etoposide, Filgrastim, and Plerixafor in Improving Stem Cell Mobilization in Treating Patients With Non-Hodgkin Lymphoma

    Science.gov (United States)

    2016-09-15

    Adult Acute Lymphoblastic Leukemia in Remission; Adult Grade III Lymphomatoid Granulomatosis; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; T-cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenström Macroglobulinemia

  7. An Unusual Case of Marginal Zone B-Cell Lymphoma Arising in the Breast - Its Diagnosis and the Role of Radiotherapy in its Management.

    LENUS (Irish Health Repository)

    Rock, Kathy

    2011-10-01

    BACKGROUND: Primary lymphoma of the breast accounts for 0.04-0.5% of all breast malignancies and approximately 1% of all extranodal lymphomas. For stage IE node-negative disease, involved field radiotherapy is recommended except for very young women in whom the risk of breast cancer is a concern. The rate of complete response for limited stage extranodal marginal B-cell lymphoma is in excess of 90%. CASE REPORT: We report the case of a 62-year-old lady who presented with a unilateral painless palpable right breast lump. She subsequently underwent a trucut biopsy of the lesion. The histology revealed a low-grade B-cell non-Hodgkin\\'s lymphoma (NHL). Immunohistochemistry showed that more than 95% of the cells were B cells which were CD 20+\\/CD 45+ and BC L6+. This confirmed the diagnosis of marginal zone lymphoma. Staging work-up was negative for distant metastases. Serum alkaline phosphatase and lactate dehydrogenase were normal. The patient had no \\'B\\' symptoms. Her final diagnosis was clinical stage IAE NHL, and she was referred for curative radiotherapy. CONCLUSION: Radiation treatment is a safe and extremely effective modality of treatment for early stage I marginal zone B-cell lymphomas of the breast.

  8. An Unusual Case of Marginal Zone B-Cell Lymphoma Arising in the Breast - Its Diagnosis and the Role of Radiotherapy in its Management.

    Science.gov (United States)

    Rock, Kathy; Rangaswamy, Guhan; O'Sullivan, Siobhra; Coffey, Jerome

    2011-10-01

    BACKGROUND: Primary lymphoma of the breast accounts for 0.04-0.5% of all breast malignancies and approximately 1% of all extranodal lymphomas. For stage IE node-negative disease, involved field radiotherapy is recommended except for very young women in whom the risk of breast cancer is a concern. The rate of complete response for limited stage extranodal marginal B-cell lymphoma is in excess of 90%. CASE REPORT: We report the case of a 62-year-old lady who presented with a unilateral painless palpable right breast lump. She subsequently underwent a trucut biopsy of the lesion. The histology revealed a low-grade B-cell non-Hodgkin's lymphoma (NHL). Immunohistochemistry showed that more than 95% of the cells were B cells which were CD 20+/CD 45+ and BC L6+. This confirmed the diagnosis of marginal zone lymphoma. Staging work-up was negative for distant metastases. Serum alkaline phosphatase and lactate dehydrogenase were normal. The patient had no 'B' symptoms. Her final diagnosis was clinical stage IAE NHL, and she was referred for curative radiotherapy. CONCLUSION: Radiation treatment is a safe and extremely effective modality of treatment for early stage I marginal zone B-cell lymphomas of the breast.

  9. 浅表器官结外淋巴瘤的超声表现%Ultrasonic feature of superficial organ extranodal lymphoma

    Institute of Scientific and Technical Information of China (English)

    宋琳琳; 詹维伟; 周建桥; 周伟

    2012-01-01

    Objective To analyze the ultrasound image and evaluate its diagnostic value for superficial organ extranodal lymphoma. Methods The ultrasonograms of 24 patients with superficial organ extranodal lymphoma confirmed by surgical or core needle biopsy were analyzed retrospectively. Results There were 24 patients with extranodal lymphoma,including 3 cases of salivary glands,7 cases of thyroid,7 cases of breast,6 cases of testis and 1 case of penis, including lymphoma on both side for one case each of testis and breast lymphoma. Altogether there were 28 superficial organ extranodal lymphoma,including 3 of salivary glands ,7 of thyroid,8 of breast,7 of testis and 3 of penis.The ultrasonographic findings were classified as focal type in 19 lymphoma and diffuse type in 9 lymphoma. The gray scale imaging showed hypoechogenicity, marked hypoechogenicity and cystoid echogenecity. Posterior enhancement was observed in 22 lymphoma (78.6%). Color Doppler ultrasonography showed markedly increased vascularity in 23 lymphoma (82.1% ). Peripheral lymphadenopathy was noted in 9 lymphoma (32.1% ),tended to be round with marked hypoechogenecity. Conclusions The ultrasonogram of superficial organ extranodal lymphoma has characteristic appearance. Ultrasonography is a useful tool for the screening,diagnosis and following up of superficial organ extranodal lymphoma.%目的:分析浅表器官结外淋巴瘤超声声像图特点,评价超声检查在诊断浅表器官结外淋巴瘤中的价值.方法:回顾性分析我院经手术或粗针穿刺活检病理证实的24例浅表器官结外淋巴瘤患者的超声声像图.结果:24例浅表器官结外淋巴瘤患者中,腮腺受累3例,甲状腺受累7例,乳房受累7例(其中1例双侧受累),睾丸受累6例(其中1例双侧受累),阴茎受累1例.24例浅表器官结外淋巴瘤患者共28个病灶,分别为腮腺3个,甲状腺7个,乳腺8个,睾丸7个,阴茎3个.超声声像图表现为局灶型19个,弥漫型9个.灰阶超声表

  10. Increased body mass index is associated with improved overall survival in extranodal natural killer/T-cell lymphoma, nasal type.

    Science.gov (United States)

    Li, Ya-Jun; Yi, Ping-Yong; Li, Ji-Wei; Liu, Xian-Ling; Liu, Xi-Yu; Zhou, Fang; OuYang, Zhou; Sun, Zhong-Yi; Huang, Li-Jun; He, Jun-Qiao; Yao, Yuan; Fan, Zhou; Tang, Tian; Jiang, Wen-Qi

    2017-01-17

    The role of body mass index (BMI) in lymphoma survival outcomes is controversial. The prognostic significance of BMI in extranodal natural killer (NK)/T-cell lymphoma (ENKTL) is unclear. We evaluated the prognostic role of BMI in patients with ENKTL. We retrospectively analyzed 742 patients with newly diagnosed ENKTL. The prognostic value of BMI was compared between patients with low BMIs (KPI) was also evaluated and compared with that of the BMI classification. Patients with low BMIs tended to exhibit higher Eastern Cooperative Oncology Group performance status (ECOG PS) scores (≥ 2) (P = 0.001), more frequent B symptoms (P KPI scores (P = 0.03), and lower rates of complete remission (P 60 years, mass > 5 cm, stage III/IV, elevated LDH levels, albumin levels KPI with respect to distinguishing between intermediate-low- and high-intermediate-risk patients. Higher BMI at the time of diagnosis is associated with improved overall survival in ENKTL. Using the BMI classification may improve the IPI and KPI prognostic models.

  11. Genetic alterations of JAK/STAT cascade and histone modification in extranodal NK/T-cell lymphoma nasal type.

    Science.gov (United States)

    Lee, Seungbok; Park, Ha Young; Kang, So Young; Kim, Seok Jin; Hwang, Jinha; Lee, Seungho; Kwak, Soo Heon; Park, Kyong Soo; Yoo, Hae Yong; Kim, Won Seog; Kim, Jong-Il; Ko, Young Hyeh

    2015-07-10

    Extranodal NK/T-cell lymphoma nasal type (ENKL) is a rare type of non-Hodgkin lymphoma that more frequently occurs in East Asia and Latin America. Even though its molecular background has been discussed in the last few years, the current knowledge does not explain the disease pathogenesis in most cases of ENKL. Here, we performed multiple types of next-generation sequencing on 34 ENKL samples, including whole-exome sequencing (9 cancer tissues and 4 cancer cell lines), targeted sequencing (21 cancer tissues), and RNA sequencing (3 cancer tissues and 4 cancer cell lines). Mutations were found most frequently in 3 genes, STAT3, BCOR, and MLL2 (which were present in 9, 7, and 6 cancer samples, respectively), whereas there were only 2 cases of JAK3 mutation. In total, JAK/STAT pathway- and histone modification-related genes accounted for 55.9% and 38.2% of cancer samples, respectively, and their involvement in ENKL pathogenesis was also supported by gene expression analysis. In addition, we provided 177 genes upregulated only in cancer tissues, which appear to be linked with angiocentric and angiodestructive growth of ENKL. In this study, we propose several novel driver genes of ENKL, and show that these genes and their functional groups may be future therapeutic targets of this disease.

  12. MicroRNA expression in nodal and extranodal Diffuse Large B-cell Lymphoma

    DEFF Research Database (Denmark)

    Mandrup, Charlotte; Petersen, Anders; Højfeldt, Anne Dirks

    (GOstat) to annotate target gene ontology revealed several prominent ontologies like proliferation (e.g. WNT5a, MAPK), and cell adhesion (several members of the PCDHA family) to be differentially affected in the two manifestations. Interestingly many miRNA had the wnt pathway as predicted target......-frozen samples. Results: It was possible to distinguish between the nodal and extranodal manifestations with the global miRNA screen (e.g. mir143, mir432, mir127, and mir195). Differentially expressed miRNA target genes were predicted by target prediction software (Targetscan and Miranda). Statistically software...

  13. Low-Dose Total Body Irradiation and Donor Peripheral Blood Stem Cell Transplant Followed by Donor Lymphocyte Infusion in Treating Patients With Non-Hodgkin Lymphoma, Chronic Lymphocytic Leukemia, or Multiple Myeloma

    Science.gov (United States)

    2015-10-30

    Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Refractory Multiple Myeloma; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Stage II Multiple Myeloma; Stage III Multiple Myeloma; Testicular Lymphoma; Waldenström Macroglobulinemia

  14. Non-Hodgkin lymphoma: computed tomographic demonstration of unusual extranodal involvement

    Energy Technology Data Exchange (ETDEWEB)

    Glazer, H.S.; Lee, J.K.T.; Balfe, D.M.; Mauro, M.A.; Griffith, R.; Sagel, S.S.

    1983-10-01

    With the advent of computed tomography, lymphomatous involvement of sites other than lymph nodes is being seen with increasing frequency. Review of computed tomographic scans in 400 patients with newly diagnosed or recurrent non-Hodgkin lymphoma revealed 37 patients to have involvement of 56 unusual sites below the diaphragm: psoas/iliacus muscle (16 patients), kidney (13 patients), pancreas (5 patients), adrenal (4 patients), skin/subcutaneous tissue (4 patients), abdominal wall musculature (4 patients), peritoneum (4 patients), omentum (3 patients), and female reproductive tract (3 patients). These were mostly seen in patients with lymphomas of diffuse architecture, especially diffuse histiocytic lymphoma. Concomitant retroperitoneal and/or mesenteric adenopathy was very common; extraodal involvement was rarely the only site of initial or recurrent lymphoma.

  15. LMP1 and LMP2A are potential prognostic markers of extranodal NK/T-cell lymphoma, nasal type (ENKTL

    Directory of Open Access Journals (Sweden)

    Mao Yuan

    2012-12-01

    Full Text Available Abstract Background Latent membrane protein (LMP 1 and LMP2A encoded by Epstein-Barr virus (EBV are associated with the development of malignancies, but their expression in extranodal NK/T-cell lymphoma, nasal type (ENKTL and the relationship with clinical characteristics of this disease remain poorly understood. In the present study, we examined the expression of LMP1 and LMP2A in ENKTL, and investigated the correlations between LMP1 and LMP2A expression with clinicopathological characteristics of ENKTL patients. Methods Paraffin sections of surgically removed samples from 16 ENKTL patients were analyzed by immunohistochemistry and the related clinicopathological data were collected and analyzed. Results Elevated expression (immunohistochemistry score ≥ 4 of LMP1 and LMP2A was detected in the tumor cells of ENKTL. High LMP1 expression was associated with positive B symptoms (p = 0.012, while high LMP2A expression was related to gender (p = 0.029. The expression of both LMP1 and LMP2A showed significant correlations with patients’ overall survival (p = 0.049, p = 0.036. Conclusion LMP1 and LMP2A may be prognostic indicators of survival in patients with ENKTL. Virtual slides http://www.diagnosticpathology.diagnomx.eu/vs/2443352538545899

  16. FAU in Treating Patients With Advanced Solid Tumors or Lymphoma

    Science.gov (United States)

    2014-01-06

    Adult Grade III Lymphomatoid Granulomatosis; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Stage III Adult Burkitt Lymphoma; Stage III Adult Diffuse Large Cell Lymphoma; Stage III Adult Diffuse Mixed Cell Lymphoma; Stage III Adult Diffuse Small Cleaved Cell Lymphoma; Stage III Adult Hodgkin Lymphoma; Stage III Adult Immunoblastic Large Cell Lymphoma; Stage III Adult Lymphoblastic Lymphoma; Stage III Adult T-cell Leukemia/Lymphoma; Stage III Cutaneous T-cell Non-Hodgkin Lymphoma; Stage III Grade 1 Follicular Lymphoma; Stage III Grade 2 Follicular Lymphoma; Stage III Grade 3 Follicular Lymphoma; Stage III Mantle Cell Lymphoma; Stage III Marginal Zone Lymphoma; Stage III Mycosis Fungoides/Sezary Syndrome; Stage III Small Lymphocytic Lymphoma; Stage IV Adult Burkitt Lymphoma; Stage IV Adult Diffuse Large Cell Lymphoma; Stage IV Adult Diffuse Mixed Cell Lymphoma; Stage IV Adult Diffuse Small Cleaved Cell Lymphoma; Stage IV Adult Hodgkin Lymphoma; Stage IV Adult Immunoblastic Large Cell

  17. Genetically Modified T-cell Infusion Following Peripheral Blood Stem Cell Transplant in Treating Patients With Recurrent or High-Risk Non-Hodgkin Lymphoma

    Science.gov (United States)

    2017-01-27

    Adult Grade III Lymphomatoid Granulomatosis; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Post-transplant Lymphoproliferative Disorder; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenström Macroglobulinemia

  18. Dose Monitoring of Busulfan and Combination Chemotherapy in Hodgkin or Non-Hodgkin Lymphoma Undergoing Stem Cell Transplant

    Science.gov (United States)

    2015-08-12

    Adult Grade III Lymphomatoid Granulomatosis; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Childhood Burkitt Lymphoma; Childhood Diffuse Large Cell Lymphoma; Childhood Grade III Lymphomatoid Granulomatosis; Childhood Immunoblastic Large Cell Lymphoma; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Contiguous Stage II Adult Burkitt Lymphoma; Contiguous Stage II Adult Diffuse Large Cell Lymphoma; Contiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Contiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Contiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Contiguous Stage II Adult Lymphoblastic Lymphoma; Contiguous Stage II Grade 1 Follicular Lymphoma; Contiguous Stage II Grade 2 Follicular Lymphoma; Contiguous Stage II Grade 3 Follicular Lymphoma; Contiguous Stage II Mantle Cell Lymphoma; Contiguous Stage II Marginal Zone Lymphoma; Contiguous Stage II Small Lymphocytic Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncontiguous Stage II Adult Burkitt Lymphoma; Noncontiguous Stage II Adult Diffuse Large Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Noncontiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Noncontiguous Stage II Adult Lymphoblastic Lymphoma; Noncontiguous Stage II Grade 1 Follicular Lymphoma; Noncontiguous Stage II Grade 2 Follicular Lymphoma; Noncontiguous Stage II Grade 3 Follicular Lymphoma; Noncontiguous Stage II Mantle Cell Lymphoma; Noncontiguous Stage II Marginal Zone Lymphoma; Noncontiguous Stage II Small Lymphocytic Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult

  19. Fine-needle aspiration diagnosis of extranodal non-Hodgkin′s lymphoma of the tongue

    Directory of Open Access Journals (Sweden)

    Srinivasa Murthy

    2011-01-01

    Full Text Available Primary non-Hodgkin′s lymphoma (NHL of the oral region is rare. Oral manifestation is present in 3-5% of cases of NHL and oral lesions are rarely the initial manifestations. We describe primary NHL, diffuse, mixed, small and large cell type in a 50-year-old female, who presented with mass lesion primarily involving the base of the tongue; initially diagnosed by fine needle aspiration cytology and later confirmed by histopathology and immunohistochemistry. Pertinent literature is being reviewed.

  20. Novel insights into the molecular pathogenesis of gastric MALT lymphoma

    OpenAIRE

    2010-01-01

    Gastric marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) represents a distinct class of extranodal lymphoma that evolves against a background of chronic inflammation induced by persistent infection with the bacterium Helicobacter pylori. In its early stages, MALT lymphoma is an antigen-dependent disease characterised by an indolent clinical course and in most cases is treatable by antibiotic eradication therapy alone. Low grade MALT lymphomas c...

  1. [Change of Plasma Interleukin-17 Level in Patients with Extranodal NK/T-Cell Lymphoma and Its Clinical Significance].

    Science.gov (United States)

    Shang, Chun-Xiang; Ma, Ji-Cheng; Nan, Zheng; Li, Ye; He, Wen-Cai; Pan, Xian-Ying

    2017-06-01

    To investigate the clinical significance of interleukin-17 (IL-17) level in patients with extranodal NK/T-cell lymphoma(ENKTL). Eighty patients with nasal ENKTL who received radiotherapy, chemotherapy or radiotherapy combined with chemotherapy from January 2011 to January 2012 were enrolled in the study. Eighty healthy volunteers were selected as the controls (control group). About 5 ml of peripheral blood was collected from all patients and controls. IL-17 level was determined by ELISA. The age, sex, ECOG score, B symptoms, LDH level, lymph node involvment, Ann Arbor stage, IPI, KPI, peripheral blood lymphocyte and lymph node metastasis, number of lymphocytes and monocytes in peripheral blood were recorded. All patients were followed up for 3 year progression-free survival (PFS) and overall survival (OS). The average IL-17 level in patients with ENKTL was 6.48 pg/ml and the average concentration of IL-17 in control group was 0.56 pg/ml (PKPI, lymphocyte count and monocyte cell count. The sensitivity and specificity of IL-17 were 74.5% and 73.7% respectively, and the optimal threshold was 3.49 pg/ml and AUC was 0.799 (95% CI: 0.688-0.909) (P<0.01). The PFS and OS were longer in the patients with IL-17≤3.49 pg/ml and longer in the patients without lymph node involvement and Ann Arbor I. Multivariate analysis showed that independent predictors of PFS and OS in patients with ENKTL were plasma IL-17 levels and age (P<0.05). ENKTL patients with different clinical characteristics have different levels of IL-17, the different level of IL-17 has different effects on prognosis of patients with ENKTL.

  2. {sup 111}In-DOTA-dPhe{sup 1}-Tyr{sup 3}-octreotide, {sup 111}In-DOTA-lanreotide and {sup 67}Ga citrate scintigraphy for visualisation of extranodal marginal zone B-cell lymphoma of the MALT type: a comparative study

    Energy Technology Data Exchange (ETDEWEB)

    Li, Shuren; Kurtaran, Amir; Li, Mei; Traub-Weidinger, Tatjana; Kienast, Oskar [Department of Nuclear Medicine, University of Vienna, Waehringer Guertel 18-20, 1090, Vienna (Austria); Schima, Wolfgang [Department of Nuclear Medicine, University of Vienna, Waehringer Guertel 18-20, 1090, Vienna (Austria); Department of Radiodiagnostics, University of Vienna (Austria); Angelberger, Peter [Research Center Seibersdorf (Austria); Virgolini, Irene [Institute for Nuclear Medicine, Vienna Hospital Lainz (Austria); Ludwig-Boltzman Institute for Nuclear Medicine, Vienna (Austria); Raderer, Markus [Department of Internal Medicine I, Division of Oncology, University of Vienna (Austria); Dudczak, Robert [Department of Nuclear Medicine, University of Vienna, Waehringer Guertel 18-20, 1090, Vienna (Austria)

    2003-08-01

    Somatostatin receptor (SSTR) scintigraphy and gallium-67 citrate ({sup 67}Ga) scintigraphy have been used for visualisation of Hodgkin's lymphoma and non-Hodgkin's lymphoma. However, experience with B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) type is very limited. The aim of this study was to prospectively compare the {sup 67}Ga scintigraphy results with those obtained by {sup 111}In-DOTA-dPhe{sup 1}-Tyr{sup 3}-octreotide ({sup 111}In-DOTA-TOCT) and {sup 111}In-DOTA-lanreotide ({sup 111}In-DOTA-LAN) scintigraphy in patients with proven MALT-type lymphoma. Comparative scintigraphic examinations using {sup 67}Ga, {sup 111}In-DOTA-TOCT and {sup 111}In-DOTA-LAN were performed in 18 patients (11 female and 7 male, median age 64{+-}15 years) with histologically verified MALT-type lymphomas of various origin. Planar and single-photon emission tomography imaging acquisitions were performed after injection of a mean dose of 185{+-}26 MBq {sup 67}Ga and 165{+-}20 MBq {sup 111}In-DOTA-TOCT or {sup 111}In-DOTA-LAN. All scintigraphic results were correlated with other conventional examinations including gastroscopy, colonoscopy, endosonoscopy, ophthalmologic investigation, CT of the thorax and abdomen and bone marrow biopsy. This comparative study showed that {sup 67}Ga scintigraphy found abnormalities in 10 of 16 patients (63%) and detected 18 of 31 clinically involved sites (58%), but was false positive in three patients. {sup 111}In-DOTA-TOCT found abnormalities in 9 of 15 patients (60%) and detected 15 of 27 clinical lesions (56%); it was false positive in two patients. {sup 111}In-DOTA-LAN scintigraphy showed abnormalities in 7 of 11 patients (64%) and found 12 of 22 clinical lesions (55%). False-positive {sup 111}In-DOTA-LAN scan results were found in two patients. For supra-diaphragmatic lesions, {sup 67}Ga scintigraphy detected 12 of 16 sites (75%). {sup 111}In-DOTA-TOCT scintigraphy revealed 7 of 15 lesions (47%). {sup 111}In

  3. Expression of the activation markers Blimp1, Foxp1 and pStat3 in extranodal diffuse large B-cell lymphomas.

    Science.gov (United States)

    Petrakis, Georgios; Kostopoulos, Ioannis; Venizelos, Ioannis; Lambropoulou, Maria; Vouras, Kyriakos; Vakalopoulou, Sofia; Mandala, Eudokia; Tsatalas, Constantinos; Papadopoulos, Nicolas

    2017-08-01

    Different studies have suggested that the expression of biomarkers related to lymphoid cell activation may provide information on the behavior of DLBCL. Most studies have concentrated on nodal or a mixture of nodal and extranodal lymphomas. The differential expression and potential clinical impact of these markers in a homogeneous group of extranodal DLBCLs are not well defined. In this study, we investigated the expression of three activation markers, Blimp1, Foxp1 and pStat3, in a cohort of 35 extranodal DLBCLs homogeneously treated with R-CHOP. Immunohistochemical stains were evaluated using an immunoreactivity score on representative paraffin sections. Blimp1 was positive in 55% (19/35), Foxp1 in 60% (21/35), and pStat3 in 69% (24/35) of our cases. We did not observe any statistical differences in the expression of these markers in GCB and non-GCB tumors or in gastrointestinal and non-gastrointestinal tumors. Blimp1 expression was negatively correlated with overall survival (OS) (p=0.001) in the whole series and in the non-GCB group (Muris algorithm) (p=0.002). Foxp1 positivity and pStat3 positivity had no impact on the outcome of the patients in the global cohort, but they were associated with a better survival in the non-GCB subgroup (p=0.033, p=0.044 respectively). Multivariate analysis showed that Blimp1 expression but not COO was an independent negative prognostic factor for OS (HR=17.5, 95%, CI=2.2-141.1, p=0.007). Our results suggest that these markers are differentially expressed and have different impacts on outcome in extranodal DLBCLs compared to nodal tumors, emphasizing the need to evaluate separately these and probably other markers in these subsets of tumors.

  4. Treatment options for ocular adnexal lymphoma (OAL

    Directory of Open Access Journals (Sweden)

    Victoria Mary Lendrum Cohen

    2009-11-01

    Full Text Available Victoria Mary Lendrum CohenSt. Bartholomew’s and Moorfields Eye Hospital, London UKAbstract: Most lymphomas that involve the ocular adnexal structure are low grade, B cell, non-Hodgkin’s lymphomas. The treatment depends upon the grade and stage of the disease. High grade lymhoma requires treatment with systemic chemotherapy whereas the localized low grade (extranodal marginal zone lymphoma can be successfully managed with local radiotherapy. Chlamydia psittaci infection is associated with low grade ocular lymphoma; however there is wide geographic variation in the strength of this association. Blanket antibiotic therapy is not advised unless there is proof of an infective agent. The monoclonal antibody, rituximab, may be successful for CD20 positive lymphoma, although it is likely that rituximab will have better long-term results when used in combination with systemic chemotherapy.Keywords: ocular adnexal lymphoma, mucosa associated lymphoid tissue, extranodal marginal zone lymphoma, Chlamydia psittaci, rituximab, radiotherapy, chemotherapy

  5. Long-term outcome for gastric marginal zone lymphoma treated with radiotherapy

    DEFF Research Database (Denmark)

    Wirth, A; Gospodarowicz, M; Aleman, B M P;

    2013-01-01

    We evaluated the long-term results of radiotherapy for patients with gastric marginal zone lymphoma (GMZL).......We evaluated the long-term results of radiotherapy for patients with gastric marginal zone lymphoma (GMZL)....

  6. Marginal Zone Lymphoma Complicated by Protein Losing Enteropathy

    Directory of Open Access Journals (Sweden)

    Nadine Stanek

    2016-01-01

    Full Text Available Protein losing enteropathy (PLE refers to excessive intestinal protein loss, resulting in hypoalbuminemia. Underlying pathologies include conditions leading to either reduced intestinal barrier or lymphatic congestion. We describe the case of a patient with long-lasting diffuse abdominal problems and PLE. Repetitive endoscopies were normal with only minimal lymphangiectasia in biopsies. Further evaluations revealed an indolent marginal zone lymphoma with minor bone marrow infiltration. Monotherapy with rituximab decreased bone marrow infiltration of the lymphoma but did not relieve PLE. Additional treatments with steroids, octreotide, a diet devoid of long-chain fatty-acids, and parenteral nutrition did not prevent further clinical deterioration with marked weight loss (23 kg, further reduction in albumin concentrations (nadir 8 g/L, and a pronounced drop in performance status. Finally, immunochemotherapy with rituximab and bendamustine resulted in hematological remission and remarkable clinical improvement. 18 months after therapy the patient remains free of gastrointestinal complaints and has regained his body weight with normal albumin levels. We demonstrate a case of PLE secondary to indolent marginal zone lymphoma. No intestinal pathologies were detected, contrasting a severe and almost lethal clinical course. Immunochemotherapy relieved lymphoma and PLE, suggesting that a high suspicion of lymphoma is warranted in otherwise unexplained cases of PLE.

  7. Lenalidomide and Combination Chemotherapy (DA-EPOCH-R) in Treating Patients With MYC-Associated B-Cell Lymphomas

    Science.gov (United States)

    2016-08-24

    Adult Grade III Lymphomatoid Granulomatosis; B-cell Chronic Lymphocytic Leukemia; Contiguous Stage II Adult Diffuse Large Cell Lymphoma; Contiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Contiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Contiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Contiguous Stage II Grade 1 Follicular Lymphoma; Contiguous Stage II Grade 2 Follicular Lymphoma; Contiguous Stage II Grade 3 Follicular Lymphoma; Contiguous Stage II Mantle Cell Lymphoma; Contiguous Stage II Marginal Zone Lymphoma; Contiguous Stage II Small Lymphocytic Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncontiguous Stage II Adult Diffuse Large Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Noncontiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Noncontiguous Stage II Grade 1 Follicular Lymphoma; Noncontiguous Stage II Grade 2 Follicular Lymphoma; Noncontiguous Stage II Grade 3 Follicular Lymphoma; Noncontiguous Stage II Mantle Cell Lymphoma; Noncontiguous Stage II Marginal Zone Lymphoma; Noncontiguous Stage II Small Lymphocytic Lymphoma; Progressive Hairy Cell Leukemia, Initial Treatment; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Stage 0 Chronic Lymphocytic Leukemia; Stage I Adult Diffuse Large Cell Lymphoma; Stage I Adult Diffuse Mixed Cell Lymphoma; Stage I Adult Diffuse Small Cleaved Cell Lymphoma; Stage I Adult Hodgkin Lymphoma; Stage I Adult Immunoblastic Large Cell Lymphoma; Stage I Chronic Lymphocytic Leukemia; Stage I Grade 1 Follicular Lymphoma; Stage I Grade 2 Follicular Lymphoma; Stage I Grade 3 Follicular Lymphoma; Stage I Mantle Cell Lymphoma; Stage I Marginal Zone Lymphoma; Stage I Small Lymphocytic Lymphoma; Stage II Adult Hodgkin Lymphoma; Stage II Chronic Lymphocytic

  8. Recidiva tardia de linfoma da zona marginal Late relapse of marginal zone lymphoma

    OpenAIRE

    Rocha,Talita M. B.S.; Bortolheiro,Tereza C.; Eduardo Costa; Daniela Haardt; Roberto P. Paes; Chiattone, Carlos S.

    2009-01-01

    O linfoma de zona marginal é um linfoma de baixo grau com curso clínico indolente e potencial de recidiva.1,2 Apresentamos um caso de recidiva tardia após 25 anos de aparente remissão completa, levantando a possibilidade de recidiva de doença preexistente ou desenvolvimento de novo clone neoplásico.Marginal zone lymphoma is a low grade lymphoma with an indolent course and chance to relapse. We present a case of a patient who suffered relapse after 25 years of apparently complete remission of ...

  9. Clinicopathology, immunophenotype, T cell receptor gene rearrangement, Epstein-Barr virus status and p53 gene mutation of cutaneous extranodal NK/T-cell lymphoma, nasal-type

    Institute of Scientific and Technical Information of China (English)

    WANG Ting-ting; XU Chen; LIU Shan-ling; KAN Bei; RAN Yu-ping; LIU Wei-ping; LI Gan-di

    2013-01-01

    Background Extranodal natural killer/T-cell (NK/T cell) lymphoma,nasal-type,is a rare lymphoma.Skin is the second most common site of involvement after the nasal cavity/nasalpharynx.The aim of this study was to investigate the clinicopathologic features,immunophenotype,T cell receptor (TCR) gene rearrangement,the association with Epstein-Barr virus (EBV) infection and p53 gene mutations of the lymphoma.Methods The clinicopathologic analysis,immunohistochemistry,in situ hybridization for EBER1/2,TCR gene rearrangement by polymerase chain reaction (PCR),mutations of p53 gene analyzed by PCR and sequence analysis were employed in this study.Results In the 19 cases,the tumor primarily involved the dermis and subcutaneous layer.Immunohistochemical staining showed that most of the cases expressed CD45RO,CD56,CD3ε,TIA-1 and GrB.Three cases were positive for CD3 and two cases were positive for CD30.Monoclonal TCRY gene rearrangement was found in 7 of 18 cases.The positive rate of EBER1/2 was 100%.No p53 gene mutation was detected on the exon 4-9 in the 18 cases.Fifteen cases showed Pro (proline)/Arg (arginine) single nucleotide polymorphisms (SNPs) on the exon 4 at codon 72.The expression of p53 protein was 72% (13/18) immunohistochemically.Conclusions Cutaneous NK/T-cell lymphoma is a rare but highly aggressive lymphoma with poor prognosis.No p53 gene mutation was detected on the exon 4-9,and Pro/Arg SNPs on p53 codon 72 were detected in the cutaneous NK/T-cell lymphoma.The overexpression of p53 protein may not be the result of p53 gene mutation.

  10. Clinicopathologic and molecular features of 122 Brazilian cases of nodal and extranodal NK/T-cell lymphoma, nasal type, with EBV subtyping analysis.

    Science.gov (United States)

    Gualco, Gabriela; Domeny-Duarte, Pollyanna; Chioato, Lucimara; Barber, Glen; Natkunam, Yasodha; Bacchi, Carlos E

    2011-08-01

    Extranodal natural killer/T-cell lymphoma, nasal type (NK/TCL) is more prevalent in Asia and in some areas of South and Central America, but it is rarely seen in the United States and Europe. In this study, a series of 122 cases of NK/TCL from Brazil was analyzed with respect to clinicopathologic features. Clinical characteristics and geographic distribution were evaluated in 97 cases of nasal/nasopharyngeal region and 23 cases in extranasal sites including 6 nodal cases. Clinical staging and follow-up information was available in a subset of 21 patients. All cases harbored Epstein-Barr virus (EBV), 95% and 85% expressed cytoplasmic CD3 and CD56, respectively, and all cases were positive for at least 1 marker for cytotoxic granules. The global distribution of EBV subtypes showed predominance of strain subtype A, 89%, and subtype B, 11%. No dual infections were detected. TCR-γ TCR-gene rearrangement was observed in 7 cases; all of them extranodal. Three of TCR-γ(+) cases showed EBV subtype A. Two TCR-γ(+)/CD56(+) cases showed EBV subtype B. Geographic distribution of NK/TCL showed higher frequency in the southeast and northeast regions of Brazil. Striking differences among geographic regions were seen with the vast majority of EBV subtype B (86%) occurring in the south and southeast regions.

  11. Regression of the tumor after withdrawal of cyclosporine in relapsed extranodal natural killer/T cell lymphoma following allogeneic hematopoietic stem cell transplantation.

    Science.gov (United States)

    Kako, Shinichi; Izutsu, Koji; Oshima, Kumi; Sato, Hiroyuki; Kanda, Yoshinobu; Motokura, Toru; Chiba, Shigeru; Kurokawa, Mineo

    2007-10-01

    The prognosis of patients with advanced-stage extranodal natural killer/T cell lymphoma, nasal type (ENKL) has been generally poor, and several anecdotal reports have suggested the role of allogeneic hematopoietic stem cell transplantation (HSCT). A potential advantage of allogeneic HSCT may be the graft-versus-lymphoma (GVL) effect. The susceptibility to the GVL effect, however, has been shown to vary according to histologic subtypes, and it has been hardly documented yet whether ENKL is susceptible to the GVL effect. Here we report a patient with advanced-stage ENKL who underwent allogeneic HSCT from an HLA one-allele mismatched related donor, whose clinical course after HSCT suggested the potent GVL effect against ENKL. A 43-year-old female underwent allogeneic HSCT for advanced-stage, chemorefractory ENKL, and achieved complete response. In 4 months after the transplantation, however, the ENKL relapsed in multiple sites. These lesions markedly responded to the discontinuation of immunosuppressive agents and disappeared. Except for a temporal exacerbation of bronchiolitis obliterans organizing pneumonia, she has been free from disease for more than a year without other treatments against lymphoma. The clinical course of the current patient suggests the potent GVL effect against ENKL. Allogeneic HSCT, including that with reduced-intensity regimens, is a promising treatment option for high-risk ENKL.

  12. Extranodal nasal NK/T-Cell Lymphoma: a rare oral presentation and FASN, CD44 and GLUT-1 Expression

    OpenAIRE

    2013-01-01

    O linfoma de células natural killers (NK)/T extranodal é um tumor maligno agressivo com características clinicopatológicas distintas, caracterizadas por invasão e destruição vasculares, necrose proeminente, fenótipo linfocítico citotóxico e uma forte associação com o vírus Epstein-Barr. Relatamos aqui um caso de linfoma de células NK/T nasal extranodal, envolvendo o seio maxilar, assoalho de órbita, e interessantemente estendendo-se para a cavidade oral através do osso alveolar e mucosa vesti...

  13. Primary thymic extranodal marginal zone B cell lymphoma as an incidental finding in a Caucasian woman

    DEFF Research Database (Denmark)

    Krogh Petersen, Jeanette; Larsen, Thomas Stauffer; Møller, Michael Boe

    2015-01-01

    of muscle and joint pain. An anterior mediastinal mass was detected by a positron emission tomography-CT (PET-CT) scan and thymectomy was performed. The mass was contained within the thymus with a homogeneous pale cut surface with solid areas. Histologically, the typical morphological and immunophenotypic...... features of TML were found, with a prominent lymphoid infiltrate comprising of small-to-medium-sized neoplastic lymphocytes, plasmacytic differentiation and a distorted thymic epithelial network. Postoperative follow-up has indicated an associated undifferentiated connective tissue disease (UCTD...

  14. Extranodal marginal zone lymphoma in the ocular region: clinical, immunophenotypical, and cytogenetical characteristics

    DEFF Research Database (Denmark)

    Sjö, Lene D; Heegaard, Steffen; Prause, Jan U;

    2008-01-01

    the period 1980 to 2005 were reviewed and reclassified according to the World Health Organization (WHO) classification. Cases reclassified as EMZL were selected and reviewed with respect to clinical characteristics and outcome. The presence of translocations involving IGH and/or MALT1 was investigated in 42...... were frequently found at extraocular sites. Five-year progression-free survival and overall survival (OS) rates were 71% and 75%, respectively. Translocations involving the IGH- or MALT1-gene loci were detected in 2 (5%) of 42 specimens. In Cox regression multivariate analysis, IGH...

  15. Reappraisal of Benign Lymphoepithelial Sialadenitis for Evidence of Extranodal Marginal Zone B-Cell Lymphoma

    Science.gov (United States)

    2016-09-28

    chromosome 18 via FISH. No cases revealed translocations involving the MALT1 gene. Epitheliotropic B cells exhibited a CD20, CD79a and PAX 5 positive B-cell...tissue as described by Reed et al. (1993): Two 7-8 µm paraffin sections from each block were deparaffinized in xylene, rehydrated with ethanol, and...amplification products of the first cycles (so-called outer nest) are not amplified in the second cycles (inner nest) ( Reed et al. 1993). Table 6

  16. Rituximab in Treating Patients Undergoing Donor Peripheral Blood Stem Cell Transplant for Relapsed or Refractory B-cell Lymphoma

    Science.gov (United States)

    2015-11-23

    B-cell Adult Acute Lymphoblastic Leukemia; B-cell Childhood Acute Lymphoblastic Leukemia; B-cell Chronic Lymphocytic Leukemia; Childhood Burkitt Lymphoma; Childhood Diffuse Large Cell Lymphoma; Childhood Immunoblastic Large Cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Post-transplant Lymphoproliferative Disorder; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Recurrent/Refractory Childhood Hodgkin Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenström Macroglobulinemia

  17. Prognostic role of ABO blood type in patients with extranodal natural killer/T cell lymphoma, nasal type: a triple-center study.

    Science.gov (United States)

    Li, Ya-Jun; Yi, Ping-Yong; Li, Ji-Wei; Liu, Xian-Ling; Tang, Tian; Zhang, Pei-Ying; Jiang, Wen-Qi

    2017-07-31

    The prognostic significance of ABO blood type for lymphoma is largely unknown. We evaluated the prognostic role of ABO blood type in patients with extranodal natural killer (NK)/T-cell lymphoma (ENKTL). We retrospectively analyzed clinical data of 697 patients with newly diagnosed ENKTL from three cancer centers. The prognostic value of ABO blood type was evaluated using Kaplan-Meier curves and Cox proportional hazard models. The prognostic values of the International Prognostic Index (IPI) and the Korean Prognostic Index (KPI) were also evaluated. Compared with patients with blood type O, those with blood type non-O tended to display elevated baseline serum C-reactive protein levels (P = 0.038), lower rate of complete remission (P = 0.005), shorter progression-free survival (PFS, P 60 years (P KPI in distinguishing between the intermediate-to-low- and high-to-intermediate-risk groups. ABO blood type was an independent predictor of clinical outcome for patients with ENKTL.

  18. Vorinostat and Decitabine in Treating Patients With Advanced Solid Tumors or Relapsed or Refractory Non-Hodgkin's Lymphoma, Acute Myeloid Leukemia, Acute Lymphocytic Leukemia, or Chronic Myelogenous Leukemia

    Science.gov (United States)

    2014-08-26

    Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Blastic Phase Chronic Myelogenous Leukemia; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Secondary Acute Myeloid Leukemia; Splenic Marginal Zone Lymphoma; Stage III Adult Burkitt Lymphoma; Stage III Adult Diffuse Large Cell Lymphoma; Stage III Adult Diffuse Mixed Cell Lymphoma; Stage III Adult Diffuse Small Cleaved Cell Lymphoma; Stage III Adult Immunoblastic Large Cell Lymphoma; Stage III Adult Lymphoblastic Lymphoma; Stage III Grade 1 Follicular Lymphoma; Stage III Grade 2 Follicular Lymphoma; Stage III Grade 3 Follicular Lymphoma; Stage III Mantle Cell Lymphoma; Stage III Marginal Zone Lymphoma; Stage III Small Lymphocytic Lymphoma; Stage IV Adult Burkitt Lymphoma; Stage IV Adult Diffuse Large Cell Lymphoma; Stage IV Adult Diffuse Mixed Cell Lymphoma; Stage IV Adult Diffuse Small Cleaved Cell Lymphoma; Stage IV Adult Immunoblastic Large Cell Lymphoma; Stage IV Adult Lymphoblastic Lymphoma; Stage IV Grade 1 Follicular Lymphoma; Stage IV Grade 2 Follicular Lymphoma; Stage IV Grade 3 Follicular Lymphoma; Stage IV Mantle Cell Lymphoma; Stage IV Marginal Zone Lymphoma

  19. Natural History Study of Monoclonal B Cell Lymphocytosis (MBL), Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL), Lymphoplasmacytic Lymphoma (LPL)/Waldenstrom Macroglobulinemia (WM), and Splenic Marginal Zone Lymphoma (SMZL)

    Science.gov (United States)

    2017-10-05

    B-Cell Chronic Lymphocytic Leukemia; Monoclonal B-Cell Lymphocytosis; Lymhoma, Small Lymphocytic; Chronic Lymphocytic Leukemia; Lymphoplasmacytic Lymphoma; Waldenstrom Macroglobulinemia; Splenic Marginal Zone Lymphoma

  20. Primary mucosa-associated lymphoid tissue thyroid lymphoma: a rare thyroid neoplasm of extrathyroid origin

    Directory of Open Access Journals (Sweden)

    Dimitrios Hadjidakis

    2012-01-01

    Full Text Available Primary thyroid lymphoma is a rare malignancy, representing 2-8% of all thyroid malignancies and 1-2% of all extranodal lymphomas. The majority of cases concern non-Hodgkin`s lymphoma of B cell origin, following by Hodgkin’s disease, T cell lymphomas and rarely marginal zone B-cell mucosa-associated lymphoid tissue (MALT lymphomas. MALT lymphomas have been associated with long-standing autoimmune Hashimoto`s thyroiditis. We present the case of a 44-years-old woman with thyroid MALT lymphoma in the background of multinodular goiter of autoimmune origin.

  1. Clinicopathologic analysis of extranodal malignant lymphoma%淋巴结外恶性淋巴瘤的临床病理研究

    Institute of Scientific and Technical Information of China (English)

    任秋华; 侯慧; 郑静; 李莉; 聂军; 刘沈伟; 景士兵

    2008-01-01

    目的 探讨不同组织淋巴结外恶性淋巴瘤(extranodal malignant lymphoma,ELA)的临床病理特点.方法 结合临床资料,采用组织形态及免疫组织化学相结合的方法 ,对脾、乳腺、扁桃体、皮肤、甲状腺、脑组织的6例病理诊断为淋巴结外恶性淋巴瘤(ELA)的病例进行临床、病理分析.结果 发生在不同部位的淋巴结外恶性淋巴瘤(ELA)临床表现缺乏特异性,与相应部位的癌相似.组织学形态与低分化癌难以鉴别,但通过仔细观察及免疫组织化学可以正确做出诊断.结论 不同部位淋巴结外恶性淋巴瘤(ELA)组织形态学具有独特的组织像,易与其他肿瘤混淆,造成误诊、漏诊,应引起临床及病理医师的高度重视.

  2. Circulating and Tumor-Infiltrating Foxp3+ Regulatory T Cell Subset in Chinese Patients with Extranodal NK/T Cell Lymphoma

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    Rou-Jun Peng, Zhou-Feng Huang, Yi-Lan Zhang, Zhong-Yu Yuan, Yi Xia, Wen-Qi Jiang, Yi-Xin Zeng, Jiang Li

    2011-01-01

    Full Text Available Foxp3+ regulatory T lymphocytes (Tregs usually act as an immune suppressor and correlate with poorer survival in malignancies. This study aims to investigate the distribution and characterization of Foxp3+ subset in peripheral blood mononuclear cells (PBMCs and tumor tissues from extranodal NK/T cell lymphoma (ENKTL. Our study showed the percentage of Foxp3+ subset from PBMC was significantly higher than that of healthy individuals (P<0.001. The Foxp3+ subset from PBMCs expressed CD45RO, CTLA4, GITR, CCR7, and had an IL-10highIFNγ+TGFβ+IL-2lowIL-17low cytokine secreting phenotype. Interestingly, the existence of EBV antigen-specific CD8+Foxp3+ Tregs was discovered in ENKTL. Furthermore, the high density of Foxp3+ TILs was associated with improved progression-free survival (PFS in ENKTL patients (P<0.05. Collectively, our study implicates that EBV antigens could induce antigen-specific CD8+Foxp3+ Tregs in ENKTL, and Foxp3+ TILs is an independent factor for PFS in ENKTL.

  3. Efficacy and tolerance of pegaspargase, gemcitabine and oxaliplatin with sandwiched radiotherapy in the treatment of newly-diagnosed extranodal nature killer (NK)/T cell lymphoma.

    Science.gov (United States)

    Jing, Xiao-Mei; Zhang, Zhi-Hui; Wu, Ping; Zhang, Shi-Chuan; Ren, Yuan-Rong; Xiong, Zhu-Juan; Wei, Wen; Luo, Lei; Li, Li

    2016-08-01

    Extranodal nature killer (NK)/T cell lymphoma (ENKL), nasal type, is a highly aggressive and heterogeneous disease. Here we report a retrospective study of 38 newly-diagnosed ENKL patients treated with pegaspargase, gemcitabine, oxaliplatin (P-Gemox) and sandwiched radiotherapy in our department during 2012-2016. A median of 4 (range, 2-6) (total=141) cycles of P-Gemox were administered. Interim restaging after at least 2 cycles showed complete remission (CR) rate of 23.68%, partial remission (PR) rate of 63.16%, giving an overall response rate (ORR) of 86.84%. On treatment completion, the ORR became 92.1% (CR=86.84%, PR=5.26%). Only one patient experienced disease progression during therapy. Multivariate analysis showed gender was a significant independent factor impacting on CR. Hematologic toxicity was common yet nonhematologic toxicity was mild, both of them can be well controlled by supportive treatments and only one treatment-related death was observed. At a median follow-up of 15.5 months, 4 patients (10.53%) experienced disease progression and died of disease. 1-year progression-free survival (PFS) rate and 1-year overall survival (OS) rate for the whole cohort were 86.7% and 86.6%. The P-Gemox regimen with sandwiched radiotherapy may be a promising option in the treatment of newly-diagnosed ENKL due to its high efficacy yet low toxicity.

  4. Predictive value of pretreatment positron emission tomography/computed tomography in patients with newly diagnosed extranodal natural killer/T-cell lymphoma.

    Science.gov (United States)

    Bai, Bing; Huang, Hui-Qiang; Cai, Qi-Chun; Fan, Wei; Wang, Xiao-Xiao; Zhang, Xu; Lin, Ze-Xiao; Gao, Yan; Xia, Yun-Fei; Guo, Ying; Cai, Qing-Qing; Jiang, Wen-Qi; Lin, Tong-Yu

    2013-03-01

    The role of (18)Fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) in extranodal natural killer/T-cell lymphoma (ENKL) is not well established. This study aimed to investigate the prognostic role of the pretreatment maximum standardized uptake value (SUV(max)) on PET/CT in patients with newly diagnosed ENKL. Among 364 consecutive patients with newly diagnosed ENKL, 81 patients were included and reviewed. The impact of SUV(max) on survival and the relationship between SUV(max) and other clinicopathological parameters were analyzed. The median SUV(max) was 14.6 (range 2.0-45.4). The optimal cutoff value of SUV(max) to predict overall survival (OS) was 15. Patients with high SUV(max) (SUVmax >15) were associated with bulky disease (P KPI, P = 0.046), resistance to primary treatment (P = 0.014), poor OS (P 60 years (P = 0.001), stage III-IV (P = 0.023), SUV(max) >15 (P = 0.020), and bulky disease (>5 cm) (P = 0.002). By using the SUV(max), patients in most subgroups stratified by the KPI or the International Prognostic Index (IPI) were further discriminated in OS with significant statistical difference. Our results suggest the pretreatment SUV(max) is predictive of prognosis in patients with newly diagnosed ENKL. The SUV(max) may provide additional prognostic information for IPI and KPI.

  5. Serum C-reactive protein (CRP) as a simple and independent prognostic factor in extranodal natural killer/T-cell lymphoma, nasal type.

    Science.gov (United States)

    Li, Ya-Jun; Li, Zhi-Ming; Xia, Yi; Huang, Jia-Jia; Huang, Hui-Qiang; Xia, Zhong-Jun; Lin, Tong-Yu; Li, Su; Cai, Xiu-Yu; Wu-Xiao, Zhi-Jun; Jiang, Wen-Qi

    2013-01-01

    C-reactive protein (CRP) is a biomarker of the inflammatory response, and it shows significant prognostic value for several types of solid tumors. The prognostic significance of CRP for lymphoma has not been fully examined. We evaluated the prognostic role of baseline serum CRP levels in patients with extranodal natural killer (NK)/T-cell lymphoma (ENKTL). We retrospectively analyzed 185 patients with newly diagnosed ENKTL. The prognostic value of the serum CRP level was evaluated for the low-CRP group (CRP≤10 mg/L) versus the high-CRP group (CRP>10 mg/L). The prognostic value of the International Prognostic Index (IPI) and the Korean Prognostic Index (KPI) were evaluated and compared with the newly developed prognostic model. Patients in the high-CRP group tended to display increased adverse clinical characteristics, lower rates of complete remission (P60 years, hypoalbuminemia, and elevated lactate dehydrogenase levels were independent adverse predictors of OS. Based on these four independent predictors, we constructed a new prognostic model that identified 4 groups with varying OS: group 1, no adverse factors; group 2, 1 factor; group 3, 2 factors; and group 4, 3 or 4 factors (PKPI in distinguishing between the low- and intermediate-low-risk groups, the intermediate-low- and high-intermediate-risk groups, and the high-intermediate- and high-risk groups. Our results suggest that pretreatment serum CRP levels represent an independent predictor of clinical outcome for patients with ENKTL. The prognostic value of the new prognostic model is superior to both IPI and KPI.

  6. Intensity-modulated radiation therapy followed by GDP chemotherapy for newly diagnosed stage I/II extranodal natural killer/T cell lymphoma, nasal type.

    Science.gov (United States)

    Huang, Yu; Yang, Jianliang; Liu, Peng; Zhou, Shengyu; Gui, Lin; He, Xiaohui; Qin, Yan; Zhang, Changgong; Yang, Sheng; Xing, Puyuan; Sun, Yan; Shi, Yuankai

    2017-09-01

    Extranodal natural killer (NK)/T cell lymphoma, nasal type (ENKTL) is an aggressive non-Hodgkin lymphoma and the majority of ENKTL cases are diagnosed at the localized stage. Radiotherapy in combination with chemotherapy has been used for localized ENKTL, but the optimal combination treatment modality and the best first-line chemotherapy regimen have not been defined. In this retrospective study, 44 patients with newly diagnosed, stages I/II ENKTL were enrolled and received intensity-modulated radiation therapy (IMRT, 50-56 Gy) followed by GDP (gemcitabine, dexamethasone, and cisplatin) chemotherapy. The median number of chemotherapy cycles per patient was 4 (range, 2-6 cycles). At the end of treatment, the overall response rate was 95% (42/44), including 39 patients (89%) who attained complete response. Two patients developed systemic progression after IMRT. With a median follow-up of 37.5 months, the 3-year overall survival (OS) rate and progression-free survival (PFS) rate were 85% (95% CI, 74 to 96%) and 77% (95% CI, 64 to 91%), respectively. Locoregional and systemic failure rates for this treatment were 9% (4/44) and 14% (6/44), respectively. The most common grades 3 to 4 adverse events included leukopenia (37%), neutropenia (34%), and mucositis (25%). No treatment-related deaths were observed. This study suggested high efficacy and low toxicity of IMRT followed by GDP regimen chemotherapy for newly diagnosed stage I/II ENKTL patients. These results require further investigation in prospective trials.

  7. [Parapsoriasis en plaque suspected of progression to mycosis fungoides associated with extranodal malignant B cell lymphoma of the cheek].

    Science.gov (United States)

    Onizuka, T; Satou, S; Koike, M; Ishiyama, T; Tomoyasu, S; Tsuruoka, N; Ohta, H; Fujisawa, R; Yamaguchi, A; Tashiro, T

    1993-06-01

    A 59-year-old female, clinically diagnosed as having parapsoriasis en plaque for ten years, was referred on June 1991 to the Department of Oral Surgery in the Dental School of showa University with a complaint of painless swelling of the left malar area. The swelling was found to have been caused by a tumor. Since the excised tumor was suspected to be a malignant lymphoma, she was admitted to our hospital. The specimen was subsequently confirmed to be a malignant lymphoma of the diffuse large cell type according to the LSG classification, and was immunologically confirmed to be a B cell lymphoma. In addition, the excised skin was suspected of incipient mycosis fungoides. She was classified as having stage IE disease according to her bone marrow biopsy and other examinations. she was treated with combination of chemotherapy (CHOP) and radiation therapy. This subject was interesting because her tumor probably resulted from a parapsoriasis en plaque skin lesion.

  8. The clinical features, management and prognosis of primary and secondary indolent lymphoma of the bone: a retrospective study of the International Extranodal Lymphoma Study Group (IELSG #14 study).

    Science.gov (United States)

    Govi, Silvia; Christie, David; Mappa, Silvia; Marturano, Emerenziana; Bruno-Ventre, Marta; Messina, Carlo; Medina, Elías A Gracia; Porter, David; Radford, John; Heo, Dae Seog; Park, Yeon; Pro, Barbara; Jayamohan, Jayasingham; Pavlakis, Nick; Zucca, Emanuele; Gospodarowicz, Mary; Ferreri, Andrés J M

    2014-08-01

    Indolent lymphomas primarily involving the skeleton (iPBL) represent management and prognosis have not been previously described. Patients with primary and secondary iPBL were selected from an international database of 499 patients with a histopathological diagnosis of non-Hodgkin lymphoma and skeleton involvement, and clinical features, management and prognosis were analyzed. Twenty-six (5%) patients had an iPBL. Ten patients had small lymphocytic lymphoma, 10 had follicular lymphoma and six had lymphoplasmacytic lymphoma. Eleven patients had limited stage and 15 had advanced disease. The overall response rate was 73% (95% confidence interval [CI] = 57-89%). Median follow-up was 58 months, and the 5- and 10-year progression-free survival (PFS) rates were 37 ± 10% and 25 ± 12%, respectively. Nine patients are alive, with 5- and 10-year overall survival (OS) rates of 46 ± 10% and 29 ± 11%, respectively. Patients with small lymphocytic lymphoma showed significantly better outcome than patients with follicular lymphoma. Performance status and stage of disease were independently associated with OS. The prognosis of patients with primary bone lymphoplasmacytic or follicular lymphoma was less favorable.

  9. Primary gastric T cell lymphoma mimicking marginal zone B cell lymphoma of mucosa-associated lymphoid tissue.

    Science.gov (United States)

    Holanda, Danniele; Zhao, Merry Y; Rapoport, Aaron P; Garofalo, Michael; Chen, Qing; Zhao, X Frank

    2008-07-01

    Primary gastric T cell lymphoma is rare and mostly of large cell type. In this paper, we present a case of gastric T cell lymphoma morphologically similar to the gastric marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT). Morphologically, the cells are small with abundant clear cytoplasm. Lymphoepithelial lesions are readily identified with diffuse destruction of gastric glands. Immunohistochemically, the neoplastic cells are CD3+/CD4+/CD8-/Granzyme B-. Molecular studies revealed monoclonal T cell receptor gamma gene rearrangement. Clinically, the patient responded initially to four cycles of R-CHOP, but then progressed. Because peripheral T cell lymphoma is usually associated with a poor prognosis, whereas marginal zone B cell lymphoma is an indolent lymphoproliferative disorder, this morphologic mimicry should be recognized and completely investigated when atypical small lymphoid infiltrates with lymphoepithelial lesions are encountered in the stomach.

  10. Extranodal diffuse large B-cell lymphoma: Experience from a tertiary care oncology center in South India

    Directory of Open Access Journals (Sweden)

    K. C. Lakshmaiah

    2014-01-01

    Full Text Available Aims: Diffuse large B-cell lymphoma (DLBCL is the most common non-Hodgkin′s lymphoma (NHL with frequent extra nodal (EN presentation. The overall occurrence of lymphomas has been increasing; however, those of EN-NHL have been increasing much more rapidly. There is limited data found on EN-DLBCL in the Indian population and hence we carried out this retrospective observational study of primary EN-DLBCL at our center in Southern India. Materials and Methods: A total of 90 consecutive cases diagnosed as EN-DLBCL (according to the standard criteria by tissue biopsy confirmed by immunohistochemistry between 2007 and 2011 were included. Staging workup including computed tomography of neck, thorax and abdomen and pelvis, bone marrow aspiration and biopsy was done and International Prognostic Index (IPI calculated. Staging was according to Cotswold′s modification of Ann Arbor. The actuarial survival analysis was performed by Kaplan-Meier. Data were analyzed using the SPSS (version 16 statistical software. Results: The median age in this study was 49 years (18-88 with results showing EN-DLBCL to be 1.36 times more common in males. Advanced stages were seen in 15 subjects (16.6% and bulky disease in 13 subjects (14.4%. CD20 was positive in 89 (98.8% while 32 had high serum lactate dehydrogenase. According to the IPI most were low-risk-56 (66.6%. Overall response rate for the various combination chemotherapies was 85.7% with complete response in 62.3%. The overall survival range spanned from 2 to 123 months. Univariate analysis showed only bulky disease was associated with inferior survival. Conclusions: EN-DLBCL was present at an early age compared to nodal DLBCL, present more often in early stage and low IPI score. Chemoimmunotherapy with radiotherapy to the EN or bulky site is the standard treatment at present.

  11. The Genetic Deletion of 6q21 and PRDM1 and Clinical Implications in Extranodal NK/T Cell Lymphoma, Nasal Type

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    Li Liang

    2015-01-01

    Full Text Available 6q21 genetic deletion has been frequently detected in extranodal NK/T cell lymphoma, nasal type (EN-NK/T-NT, and PRDM1 is considered as candidate gene. However, direct detection of PRDM1 deletion has not been well documented. We investigated genetic alterations of 6q21 and PRDM1 in 43 cases of EN-NK/T-NT and cell lines by FISH. PRDM1 expression was evaluated by immunohistochemistry and Western blot. The correlation between genetic alteration and PRDM1 expression and the significance in clinic-pathologic were analyzed. Heterozygous deletion of 6q21 and/or PRDM1 was observed in 24 of 43 cases (55.81% of EN-NK/T-NT including 16 cases (37.21% for 6q21 deletion and 19 cases (44.19% for PRDM1 deletion. Similarly, heterozygous codeletion of 6q21 and PRDM1 was identified in NK92 and NKL cells. The heterozygous deletion of 6q21 and/or PRDM1 was correlated with PRDM1 expression. However, genetic deletion of 6q21 and/or PRDM1 was not correlated with clinicopathological features of EN-NK/T-NT, while PRDM1 expression showed positive effect on the outcome of patients as those as disease site, B symptom, and clinical stage. Thus, heterozygous deletion of 6q21 and/or PRDM1 was frequently detected in EN-NK/T-NT and correlated with downregulation of PRDM1. But the prognostic role of genetic deletion needs to be further evaluated in larger cohort.

  12. Concurrent IMRT and weekly cisplatin followed by GDP chemotherapy in newly diagnosed, stage IE to IIE, nasal, extranodal NK/T-Cell lymphoma.

    Science.gov (United States)

    Ke, Q-H; Zhou, S-Q; Du, W; Liang, G; Lei, Y; Luo, F

    2014-12-12

    On the basis of the benefits of frontline radiation in early-stage, extranodal natural killer (NK)/T-cell lymphoma (ENKTL), we conducted the trial of concurrent chemoradiotherapy (CCRT) followed by three cycles of gemcitabine, dexamethasone and cisplatin (GDP). Thirty-two patients with newly diagnosed, stage IE to IIE, nasal ENKTL received CCRT (that is, all patients received intensity-modulated radiotherapy 56 Gy and cisplatin 30 mg/m(2) weekly, 3-5 weeks). Three cycles of GDP (gemcitabine 1000 mg/m(2) intravenously (i.v.) on days 1 and 8, dexamethasone 40 mg orally on days 1-4 and cisplatin 75 mg/m(2) i.v. on day 1 (GDP), every 21 days as an outpatient were scheduled after CCRT. All patients completed CCRT, which resulted in 100% response that included 24 complete responses (CRs) and eight partial responses. The CR rate after CCRT was 75.0% (that is, 24 of 32 responses). Twenty-eight of the 32 patients completed the planned three cycles of GDP, whereas four patients did not because they withdrew (n = 1) or because they had an infection (n = 3). The overall response rate and the CR rate were 90.6% (that is, 29 of 32 responses) and 84.4% (that is, 27 of 32 responses), respectively. Only two patient experienced grade 3 toxicity during CCRT (nausea), whereas 13 of the 30 patients experienced grade 4 neutropenia. The estimated 3-year overall survival and progression-free rates were 87.50% and 84.38%, respectively. In conclusion, CCRT followed by GDP chemotherapy can be a feasible and effective treatment strategy for stage IE to IIE nasal ENKTL.

  13. Primary cutaneous marginal zone B-cell lymphoma: clinical and therapeutic features in 50 cases.

    NARCIS (Netherlands)

    Hoefnagel, J.J.; Vermeer, M.H.; Jansen, P.A.M.; Heule, F.; Voorst Vader, P.C. van; Sanders, C.J.; Gerritsen, M.J.P.; Geerts, M.L.; Meijer, C.J.; Noordijk, E.M.; Willemze, R.

    2005-01-01

    BACKGROUND: Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is a low-grade B-cell lymphoma that originates in the skin, with no evidence of extracutaneous disease. Studies focusing on the optimal treatment of PCMZL have not been published thus far. We describe 50 patients with PCMZL to

  14. Primary cutaneous marginal zone B-cell lymphoma: Clinical and therapeutic features in 50 cases

    NARCIS (Netherlands)

    P.P.W. Hoefnagel (Pepijn); P.M. Noordijk (P.); R. Willemze (Roelof); M.H. Vermeer (Maarten); P.M. Jansen (Pieter); F. Heule (Freerk); P.C. Van Voorst Vader (P.); C.J.G. Sanders (C. J G); M.J.P. Gerritsen (M. J P); M.L. Geerts (M.); C.J.L.M. Meijer (Chris)

    2005-01-01

    textabstractBackground: Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is a low-grade B-cell lymphoma that originates in the skin, with no evidence of extracutaneous disease. Studies focusing on the optimal treatment of PCMZL have not been published thus far. We describe 50 patients with

  15. Primary cutaneous marginal zone B-cell lymphoma - Clinical and therapeutic features in 50 cases

    NARCIS (Netherlands)

    Hoefnagel, JJ; Vermeer, MH; Jansen, PM; Heule, F; Vader, PCV; Sanders, CJG; Gerritsen, MJP; Geerts, ML; Meijer, CJLM; Noordijk, EM; Willemze, R

    Background: Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is a low-grade B-cell lymphoma that originates in the skin, with no evidence of extracutaneous disease. Studies focusing on the optimal treatment of PCMZL have not been published thus far. We describe 50 patients with PCMZL to

  16. Primary Extranodal Non-Hodgkin Lymphoma of the Head and Neck in Patients with Acquired Immunodeficiency Syndrome: A Clinicopathologic Study of 24 Patients in a Single Hospital of Infectious Diseases in Argentina

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    Corti, Marcelo

    2014-04-01

    Full Text Available Introduction Extranodal non-Hodgkin lymphomas (NHLs are commonly described in patients with acquired immunodeficiency syndrome (AIDS and are related with an atypical morphology and aggressive clinical course. AIDS-associated lymphomas are characterized by their rapid progression, frequent extranodal manifestations, and poor outcome. Objective The aim of this article is to remake the clinical features of head and neck (HN NHL in patients with AIDS to facilitate early diagnosis and treatment. Methods We evaluated the epidemiologic, clinical, immunologic, virologic, and histopathologic characteristics of 24 patients with human immunodeficiency virus (HIV/AIDS with primary HN NHL treated at a single institution between 2002 and 2012. Histopathologic diagnosis was made according to the criteria of the World Health Organization Classification of Tumors of Hematopoietic and Lymphoid Tissues. Additional immunohistochemical stains were applied in all cases. Results Eighteen patients (75% were men and the median of age was 39 years. The gingiva and the hard palate were the most common sites of the lesions (15 patients, 62.5%. Lactate dehydrogenase levels were elevated in 16 cases (84%. Bone marrow infiltration was detected only in 4 cases (16.6%. The median CD4 T-cell count was 100 cells/µL. According to the histopathologic evaluation, the most common subtype was diffuse large B-cell lymphoma (12 cases, 50%, followed by plasmablastic lymphoma (9 cases, 37.5% and Burkitt lymphoma (3 cases, 12.5%. Conclusion HN NHL is a severe complication of advanced HIV/AIDS disease. Early diagnosis followed by chemotherapy plus highly active antiretroviral treatment is necessary to improve the prognosis and the survival of these patients.

  17. Bortezomib and Filgrastim in Promoting Stem Cell Mobilization in Patients With Non-Hodgkin Lymphoma or Multiple Myeloma Undergoing Stem Cell Transplant

    Science.gov (United States)

    2016-04-19

    Adult Grade III Lymphomatoid Granulomatosis; B-cell Chronic Lymphocytic Leukemia; Contiguous Stage II Adult Burkitt Lymphoma; Contiguous Stage II Adult Diffuse Large Cell Lymphoma; Contiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Contiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Contiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Contiguous Stage II Adult Lymphoblastic Lymphoma; Contiguous Stage II Grade 1 Follicular Lymphoma; Contiguous Stage II Grade 2 Follicular Lymphoma; Contiguous Stage II Grade 3 Follicular Lymphoma; Contiguous Stage II Mantle Cell Lymphoma; Contiguous Stage II Marginal Zone Lymphoma; Contiguous Stage II Small Lymphocytic Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncontiguous Stage II Adult Burkitt Lymphoma; Noncontiguous Stage II Adult Diffuse Large Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Noncontiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Noncontiguous Stage II Adult Lymphoblastic Lymphoma; Noncontiguous Stage II Grade 1 Follicular Lymphoma; Noncontiguous Stage II Grade 2 Follicular Lymphoma; Noncontiguous Stage II Grade 3 Follicular Lymphoma; Noncontiguous Stage II Mantle Cell Lymphoma; Noncontiguous Stage II Marginal Zone Lymphoma; Noncontiguous Stage II Small Lymphocytic Lymphoma; Progressive Hairy Cell Leukemia, Initial Treatment; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular

  18. Primary site and regional lymph node involvement are independent prognostic factors for early-stage extranodal nasal-type natural killer/T cell lymphoma

    Institute of Scientific and Technical Information of China (English)

    ShaoQing Niu; YuJing Zhang; Yong Yang; YiYang Li; Ge Wen; Liang Wang; ZhiMing Li; HanYu Wang; LuLu Zhang; YunFei Xia

    2016-01-01

    Background: Nasal‑type extranodal natural killer/T‑cell lymphoma (ENKTCL) originates primarily in the nasal cavity or extra‑nasal sites within the upper aerodigestive tract. However, it is unclear whether the primary site can serve as an independent prognostic factor or whether the varying clinical outcomes observed with different primary sites can be attributed merely to their propensities of regional lymph node involvement. The aim of this study was to investigate the prognostic implications of the primary site and regional lymph node involvement in patients with early‑stage nasal‑type ENKTCL. Methods: To develop a nomogram, we reviewed the clinical data of 215 consecutively diagnosed patients with early‑stage nasal‑type ENKTCL who were treated in Sun Yat‑sen University Cancer Center with chemotherapy and radiotherapy between 2000 and 2011. The predictive accuracy and discriminative ability of the nomogram were determined using a concordance index (C‑index) and calibration curve. Results: The 5‑year overall survival (OS) and progression‑free survival (PFS) rates of patients with nasal ENKTCL were higher than those of patients with extra‑nasal ENKTCL (OS: 68.2% vs. 46.0%, P = 0.030; PFS: 53.4% vs. 26.6%, P = 0.010).The 5‑year OS and PFS rates of patients with Ann Arbor stage IE ENKTCL were higher than those of patients with Ann Arbor stage IIE ENKTCL (OS: 66.3% vs. 59.2%, P = 0.003; PFS: 51.4% vs. 40.3%, P = 0.009). Multivariate analysisshowed that age >60 years, ECOG performance status score nasal primary site, and regional lymph node involvement were significantly associated with lower 5‑year OS rate;≥2, elevated lactate dehydrogenase (LDH) level, extra‑age >60 years, elevated LDH level, extra‑nasal primary site, and regional lymph node involvement were significantly associated with lower 5‑year PFS rate. The nomogram included the primary site and regional lymph node involve‑ment based on multivariate analysis. The

  19. A Phase 2 Study of Concurrent Fludarabine and Rituximab for the Treatment of Marginal Zone Lymphomas

    Science.gov (United States)

    Brown, Jennifer R; Friedberg, Jonathan W.; Feng, Yang; Scofield, Sarah; Phillips, Kimberly; Cin, Paola Dal; Joyce, Robin; Takvorian, Ronald W; Fisher, David C; Fisher, Richard I; Liesveld, Jane; Marquis, Diana; Neuberg, Donna; Freedman, Arnold S

    2009-01-01

    SUMMARY The marginal zone lymphomas are a recently defined group of related diseases likely arising from a common cell of origin, the marginal zone B cell. Data on therapy for subtypes other than gastric MALT has been largely limited to retrospective case series. We therefore undertook this prospective phase 2 study of fludarabine and rituximab for the treatment of marginal zone lymphomas. 26 patients were enrolled, 14 with nodal MZL, 8 with MALT lymphomas and 4 with splenic MZL; 81% were receiving initial systemic therapy. Only 58% (95% CI 37–77%) of patients completed the planned six cycles, due to significant hematologic, infectious and allergic toxicity. Four late toxic deaths occurred due to infections (15% (95% CI 4.3–35%), two related to delayed bone marrow aplasia and two related to MDS. Nonetheless, the ORR was 85% (95% CI 65–96%), with 54% CRs. The progression-free survival at 3.1 years of follow-up is 79.5% (95% CI, 63–96%). We conclude that although concurrent fludarabine and rituximab given at this dose and schedule is a highly effective regimen in the treatment of marginal zone lymphomas, the significant hematologic and infectious toxicity observed both during and after therapy is prohibitive in this patient population, emphasizing the need to study marginal zone lymphomas as a separate entity. PMID:19344412

  20. Clinical and pathological features of testicular diffuse large B-cell lymphoma: a heterogeneous disease.

    NARCIS (Netherlands)

    Kuper-Hommel, M.J.; Janssen-Heijnen, M.L.; Vreugdenhil, G.; Krol, A.D.; Kluin-Nelemans, H.C.; Coebergh, J.W.W.; Krieken, J.H.J.M. van

    2012-01-01

    Most testicular lymphomas are of diffuse large B-cell (DLBCL) type with an outcome inferior to nodal DLBCL. Within an apparently homogeneous group of testicular DLBCLs, small cell components, plasmacytoid differentiation and lymphoepithelial lesions (LELs), features of extranodal marginal zone

  1. Clinical and pathological features of testicular diffuse large B-cell lymphoma : a heterogeneous disease

    NARCIS (Netherlands)

    Kuper-Hommel, Marion J. J.; Janssen-Heijnen, Maryska L. G.; Vreugdenhil, Gerard; Krol, Augustinus D. G.; Kluin-Nelemans, Hanneke C.; Coebergh, Jan-Willem W.; van Krieken, J. Han J. M.

    2012-01-01

    Most testicular lymphomas are of diffuse large B-cell (DLBCL) type with an outcome inferior to nodal DLBCL. Within an apparently homogeneous group of testicular DLBCLs, small cell components, plasmacytoid differentiation and lymphoepithelial lesions (LELs), features of extranodal marginal zone lymph

  2. Ophthalmic lymphoma: epidemiology and pathogenesis.

    Science.gov (United States)

    Sjö, Lene Dissing

    2009-02-01

    With a lifetime risk of 1% and 700 new cases per year, Non-Hodgkin lymphoma (NHL) is the seventh most frequent type of cancer in Denmark. The incidence of NHL has increased considerably in Western countries over the last decades; consequently, NHL is an increasing clinical problem. Ophthalmic lymphoma, (lymphoma localized in the ocular region, i.e. eyelid, conjunctiva, lacrimal sac, lacrimal gland, orbit, or intraocularly) is relatively uncommon, accounting for 5%-10% of all extranodal lymphomas. It is, however, the most common orbital malignancy. The purpose of this thesis was to review specimens from all Danish patients with a diagnosis of ophthalmic lymphoma during the period 1980-2005, in order to determine the distribution of lymphoma subtypes, and the incidence- and time trends in incidence for ophthalmic lymphoma. Furthermore, an extended analysis of the most frequent subtype, extranodal marginal zone lymphoma (MALT lymphoma), was done to analyse clinical factors and cytogenetic changes with influence on prognosis. A total of 228 Danish patients with a biopsy-reviewed verified diagnosis of ocular adnexal-, orbital-, or intraocular lymphoma were identified. We found that more than 50% of orbital- and ocular adnexal lymphomas were of the MALT lymphoma subtype, whereas diffuse large B-cell lymphoma (DLBCL) predominated intraocularly (Sjo et al. 2008a). Furthermore, lymphoma arising in the lacrimal sac was surprisingly predominantly DLBCL (Sjo et al. 2006). Incidence rates were highly dependent on patient age. There was an increase in incidence rates for the whole population from 1980 to 2005, corresponding to an annual average increase of 3.4% (Sjo et al. 2008a). MALT lymphoma arising in the ocular region was found in 116 patients (Sjo et al. 2008b). One third of patients had a relapse or progression of disease after initial therapy and relapses were frequently found at extra-ocular sites. Overall survival, however, was not significantly poorer for patients

  3. Primary malignant lymphoma of the parotid gland

    Directory of Open Access Journals (Sweden)

    Sudha H Metikurke

    2012-01-01

    Full Text Available Lymphoma of the salivary gland accounts for 5% of cases of extranodal lymphoma and 10% of malignant salivary gland tumors. Most primary salivary gland lymphomas are B marginal zone lymphomas arising on a background of sialadenitis associated with an autoimmune disorder such as Sjorgen′s syndrome. This report describes a case of primary B-cell lymphoma arising in the parotid gland in a middle-aged female, which was not associated with an autoimmune disorder. Immunohistochemistry studies confirmed the clonal B-cell nature of the tumor. This case highlights the fact that B-cell lymphoma in the salivary gland can go unrecognized due to its non-specific symptoms and requires immunohistochemistry studies for confirmation. We present this case for its rarity.

  4. Molecular Pathogenesis of MALT Lymphoma

    Directory of Open Access Journals (Sweden)

    Katharina Troppan

    2015-01-01

    Full Text Available Approximately 8% of all non-Hodgkin lymphomas are extranodal marginal zone B cell lymphoma of mucosa associated lymphoid tissue (MALT, also known as MALT lymphoma, which was first described in 1983 by Isaacson and Wright. MALT lymphomas arise at a wide range of different extranodal sites, with the highest frequency in the stomach, followed by lung, ocular adnexa, and thyroid, and with a low percentage in the small intestine. Interestingly, at least 3 different, apparently site-specific, chromosomal translocations and missense and frameshift mutations, all pathway-related genes affecting the NF-κB signal, have been implicated in the development and progression of MALT lymphoma. However, these genetic abnormalities alone are not sufficient for malignant transformation. There is now increasing evidence suggesting that the oncogenic product of translocation cooperates with immunological stimulation in oncogenesis, that is, the association with chronic bacterial infection or autoaggressive process. This review mainly discusses MALT lymphomas in terms of their genetic aberration and association with chronic infections and summarizes recent advances in their molecular pathogenesis.

  5. Splenic marginal zone lymphoma with Evans' syndrome, autoimmunity, and peripheral gamma/delta T cells

    OpenAIRE

    2008-01-01

    Splenic marginal zone lymphoma with Evans? syndrome, autoimmunity, and peripheral gamma/delta T cells (Garcia-Mu?oz, Ricardo) Hematology Service, Clinica Universitaria, University of Navarra - Pamplona - SPAIN (Garcia-Mu?oz, Ricardo) Hematology Service, Clinica Universitaria, University of Navarra - Pamplona - SPAIN (Rodriguez-Otero, Paula) Hematology Service, Clinica Universitaria, University of Navarra - Pamplona - SPAIN (Pegenaute, Carlota) Department of...

  6. Linfoma B de zona marginal extranodal tipo MALT de conjuntiva bilateral tratado con radioterapia y anticuerpo monoclonal en un paciente con orbitopatía distiroidea

    Directory of Open Access Journals (Sweden)

    Sara Isabel Plazola Hernández

    2014-04-01

    Full Text Available Una mujer de 42 años con orbitopatía distiroidea presenta aumento de volumen conjuntival bilateral con reporte de linfoma no hodgkiniano de patrón folicular tipo MALT CD45 y 20 positivos. Se trata del primer caso de orbitopatía distiroidea y el desarrollo de un linfoma extranodal de conjuntiva bilateral tratado con rituximab y radioterapia.

  7. Linfoma B de zona marginal extranodal tipo MALT de conjuntiva bilateral tratado con radioterapia y anticuerpo monoclonal en un paciente con orbitopatía distiroidea

    OpenAIRE

    Plazola Hernández, Sara Isabel; Pérez Terán, Saúl Alberto; Moreno Ferreyra, Luis Gerardo

    2014-01-01

    Una mujer de 42 años con orbitopatía distiroidea presenta aumento de volumen conjuntival bilateral con reporte de linfoma no hodgkiniano de patrón folicular tipo MALT CD45 y 20 positivos. Se trata del primer caso de orbitopatía distiroidea y el desarrollo de un linfoma extranodal de conjuntiva bilateral tratado con rituximab y radioterapia.

  8. RNA-binding protein VICKZ is expressed in a germinal center associated pattern among lymphoma subtypes

    DEFF Research Database (Denmark)

    Natkunam, Y.; Vainer, G.; Zhao, S.C.;

    2005-01-01

    to the cytoplasm. Among 868 non-Hodgkin and Hodgkin lymphomas tested by immunohistochemistry on tissue microarrays, staining for VICKZ protein was present in 76% (126/165) of follicular lymphoma, 78% (155/200) of DLBCL, 90% (9/10) of mediastinal large B-cell lymphoma, and 100% (2/2) of Burkitt lymphoma. A subset...... of mantle cell lymphoma (11%, 2/19), extranodal (8%, 2/25), and nodal (20%, 1/5) marginal zone lymphoma and lymphoblastic lymphoma (25%, 4/13), showed VICKZ staining. The majority of lymphocyte predominant Hodgkin (92%, 12/13) and classical Hodgkin (94%, 101/108) lymphoma were found to be positive. Among T......Recent effort in the molecular characterization of diffuse large B-cell lymphoma (DLBCL) has led to the recognition that patients with DLBCL of germinal center origin exhibit a better overall survival. Thus, identification and characterization of markers of germinal center derivation...

  9. Linfomas não-Hodgkin extranodais em Salvador-Bahia: aspectos clínicos e classificação histopatológica segundo a OMS-2001 Extranodal non-Hodgkins lymphomas in Salvador, Brazil: clinical aspects and histopathological classification according to the WHO-2001 guidelines

    Directory of Open Access Journals (Sweden)

    Marinho M. Silva Neto

    2008-02-01

    Full Text Available Linfomas não-Hodgkin (LNH extranodais representam cerca de um terço de todos os linfomas e atualmente apresentam taxa de incidência maior que a de linfomas nodais. Diferenças entre LNH nodais e extranodais incluem etiologia, formas de apresentação e resposta terapêutica, entretanto não dispomos de dados na nossa população. Este estudo teve como objetivo caracterizar os LNH extranodais diagnosticados no Hospital Aristides Maltez, em Salvador-Bahia. Foram avaliados, retrospectivamente, 145 diagnósticos de linfoma não-Hodgkin, segundo a OMS-2001, no período de janeiro de 1999 a julho de 2001. A freqüência de linfomas extranodais foi de 30,3%. A idade média dos pacientes foi de 55,6 anos e a relação homem/mulher foi de 1:1. A maioria dos pacientes apresentava estadios avançados (III ou IV de Ann Arbor, presença de sintomas B, LDH normal, bom desempenho pela escala do ECOG e IPI entre zero e dois. Nove pacientes estão vivos e em remissão completa (22,5% após um seguimento médio de 23 meses. O sítio extranodal mais comumente acometido foram as tonsilas, seguidas pela cavidade oral, pele e trato gastrointestinal, dentre outros. O linfoma difuso de grandes células B foi o mais comum subtipo histológico, seguido pelo linfoma anaplásico de grandes células. Concluímos que o mais freqüente sítio extranodal de apresentação em nosso estudo difere da maioria da literatura, porém nossa freqüência de linfoma extranodal é semelhante à mesma.Extranodal non-Hodgkins lymphomas represent approximately one third of all lymphomas and currently have an incidence higher than nodal lymphomas. Differences in etiology, presentation and outcome of these lymphomas have been reported. However, there are no data in our population. This study was carried out in the Pathological Anatomy Service of Aristides Maltez Hospital in Salvador, Bahia. One hundred and forty-five non-Hodgkins lymphomas cases according to the WHO-2001 classification

  10. Stereotyped patterns of B-cell receptor in splenic marginal zone lymphoma

    KAUST Repository

    Zibellini, S.

    2010-05-29

    Antigen stimulation may be important for splenic marginal zone lymphoma pathogenesis. To address this hypothesis, the occurrence of stereotyped B-cell receptors was investigated in 133 SMZL (26 HCV+) compared with 4,414 HCDR3 sequences from public databases. Sixteen SMZL (12%) showed stereotyped BCR; 7 of 86 (8%) SMZL sequences retrieved from public databases also belonged to stereotyped HCDR3 subsets. Three categories of subsets were identified: i) SMZL-specific subsets (n=5), composed only of 12 SMZL (9 HCV- from our series); ii) Non-Hodgkin\\'s lymphoma-like subsets (n=5), comprising 5 SMZL (4 from our series) clustering with other indolent lymphomas; iii) "CLL-like subsets" (n=6), comprising 6 SMZL (3 from our series) that belonged to known CLL subsets (n=4) or clustered with public CLL sequences. Immunoglobulin 3D modeling of 3 subsets revealed similarities in antigen binding regions not limited to HCDR3. Overall, data suggest that the pathogenesis of splenic marginal zone lymphoma may involve also HCV unrelated epitopes or an antigenic trigger common to other indolent lymphomas. ©2010 Ferrata Storti Foundation.

  11. Utility of baseline, interim and end-of-treatment 18F-FDG PET/CT in extranodal natural killer/T-cell lymphoma patients treated with L-asparaginase/pegaspargase

    Science.gov (United States)

    Chang, Yu; Fu, Xiaorui; Sun, Zhenchang; Xie, Xinli; Wang, Ruihua; Li, Zhaoming; Zhang, Xudong; Sheng, Guangyao; Zhang, Mingzhi

    2017-01-01

    Positron emission tomography-computed tomography (PET/CT) is widely used for initial staging and monitoring treatment responses in Hodgkin and diffuse large B-cell lymphoma. However, its prognostic value in extranodal natural killer (NK)/T-cell lymphoma (ENKL) remains unclear. Here, we conducted a retrospective study to determine the impact of PET/CT in ENKL. Fifty-two patients newly diagnosed with ENKL were enrolled. Baseline maximum standardized uptake values (SUVmax), whole-body metabolic tumor volume (WBMTV) and whole-body total lesion glycolysis (WBTLG) were recorded. Additionally, interim PET/CT (I-PET) and end-of-treatment PET/CT (E-PET) results were scored using a 5-point scale. Patients were divided into groups using baseline parameter cut-off values; significant differences were found in overall survival (OS) and progression-free survival (PFS) between the high and low WBMTV and WBTLG groups and in OS between the two SUVmax groups. Positive I-PET and E-PET results predicted inferior PFS and OS. A multivariate analysis showed that baseline WBTLG, I-PET and E-PET results were associated with PFS and OS, and baseline SUVmax was an independent predictor of OS. Thus, baseline WBTLG, I-PET and E-PET results are good predictors of PFS and OS in ENKL patients who received L-asparaginase/pegaspargase in their first-line treatment, and baseline SUVmax is a valuable tool for assessing OS. PMID:28117395

  12. A phase II prospective study of the "Sandwich" protocol, L-asparaginase, cisplatin, dexamethasone and etoposide chemotherapy combined with concurrent radiation and cisplatin, in newly diagnosed, I/II stage, nasal type, extranodal natural killer/T-cell lymphoma.

    Science.gov (United States)

    Jiang, Ming; Zhang, Li; Xie, Li; Zhang, Hong; Jiang, Yu; Liu, Wei-Ping; Zhang, Wen-Yan; Tian, Rong; Deng, Yao-Tiao; Zhao, Sha; Zou, Li-Qun

    2017-07-25

    Nasal-type, extranodal NK/T cell lymphoma (ENKTCL) is a special type of lymphomas with geographic and racial specificity. Up to now, the standard first-line treatment is still not unified. In our previous report, the "sandwich" protocol produced good results. Continuing to use the "sandwich" mode, a new chemotherapy composed of L-asparaginase, cisplatin, etoposide and dexamethasone (LVDP) plus concurrent chemoradiotherapy (CCRT) was conducted in more patients with newly diagnosed, I/II stage ENKTCL. The results showed that 66 patients were enrolled. Overall response rate was 86.4% including 83.3% complete response and 3.0% partial remission. With the median follow-up of 23.5 months, 3-year overall survival and 3-year progression-free survival were 70.1% and 67.4%, respectively. The survival rate in stage II and extra-cavity stage I was significantly less than that in limited stage I (p < 0.05). Therefore, we thought that the "sandwich" mode was worthy of being generalized and LVDP combined with CCRT was an effective protocol for I/II stage ENKTCL. But this regimen was not suitable for all stage I/II patients and warrants larger sample and layering investigation. This study was a registered clinical trial with number ChiCTR-TNC-12002353.

  13. FOXP1 status in splenic marginal zone lymphoma: a fluorescence in situ hybridization and immunohistochemistry approach.

    Science.gov (United States)

    Baró, Cristina; Espinet, Blanca; Salido, Marta; Colomo, Lluís; Luño, Elisa; Florensa, Lourdes; Ferrer, Ana; Salar, Antonio; Campo, Elias; Serrano, Sergi; Solé, Francesc

    2009-11-01

    Splenic marginal zone lymphoma (SMZL) is a well-recognized entity in which chromosomal aberrations seem to be potential markers in diagnosis, prognosis and disease monitoring. FOXP1 is a transcriptional regulator of B lymphopoiesis that is deregulated in some types of NHL. Translocation t(3;14)(p14;q32) has been described in marginal zone lymphomas but few series have studied FOXP1 involvement in SMZL. We performed cytogenetic, fluorescence in situ hybridization (FISH) and immunohistochemical (IHC) studies in a series of 36 patients in order to study the status of FOXP1 in this entity. According to our results, FOXP1 is not rearranged in SMZL, although we were able to demonstrate gains of FOXP1 gene due to trisomy 3/3p by FISH. FOXP1 protein expression seemed to be not related to any aberration and IHC studies are not conclusive.

  14. 结外NK/T细胞淋巴瘤,鼻型的诊断与治疗进展%Diagnosis and treatment of extranodal NK/T-cell lymphoma, nasal type

    Institute of Scientific and Technical Information of China (English)

    冯帆; 李志铭

    2016-01-01

    Extranodal natural killer (NK)/T-cell lymphoma, nasal type (ENKL) is an uncommon subtype of non-Hodgkin's lymphoma, which is aggressive and has poor prognosis. ENKL occurs predominantly in the nasal cavity and less frequently in the skin and in the gastrointestinal tract. The pathology of this disease is characterized by vascular invasion and destruction. ENKL is strongly associated with the Epstein–Barr virus, which aids in the diagnosis of ENKL. The characteristic markers on the surface of an NK/T cell and the spe-cific genetic variations are also useful in the diagnosis. At present, the treatment of ENKL is still under discussion. Although radiothera-py combined with chemotherapy on patients with early-stage ENKL and L-ASP-based chemotherapy and hematopoietic stem cell trans-plant on patients with advance-stage ENKL have achieved favorable effects, further studies are still necessary to develop the principles and methods of a standardized treatment.%结外NK/T细胞淋巴瘤,鼻型(extranodal natural killer/T-cell lymphoma,nasal type,ENKL)是非霍奇金淋巴瘤(non-hodgkin lymphoma,NHL)的一种少见亚型,其侵袭性强且预后较差。ENKL主要发生于鼻腔,其次是皮肤、胃肠道等。该病以血管的侵犯和组织破坏为主要病理学表现。ENKL与EBV的感染密切相关,EBV水平对其辅助诊断有重要的意义。NK/T细胞表面的特征性标志物和特异性遗传学改变也可以帮助诊断该病。目前对于ENKL的治疗尚在讨论中,虽然对早期患者采用放疗±化疗联合治疗,以及对中晚期患者采用以左旋门冬酰胺酶为基础的化疗和造血干细胞移植得到了一定疗效,但仍需进一步的研究探索以形成规范的治疗原则。

  15. Therapy of gastric mucosa associated lymphoid tissue lymphoma

    Institute of Scientific and Technical Information of China (English)

    Andrea Morgner; Renate Schmelz; Christian Thiede; Manfred Stolte; Stephan Miehlke

    2007-01-01

    Gastric mucosa associated lymphoid tissue (MALT)lymphoma has recently been incorporated into the World Health Organization (WHO) lymphoma classification,termed as extranodal marginal zone B-cell lymphoma of MALT-type. In about 90% of cases this lymphoma is associated with H pylori infection which has been clearly shown to play a causative role in lymphomagenesis.Although much knowledge has been gained in defining the clinical features, natural history, pathology, and molecular genetics of the disease in the last decade, the optimal treatment approach for gastric MALT lymphomas,especially locally advanced cases, is still evolving. In this review we focus on data for the therapeutic, stage dependent management of gastric MALT lymphoma.Hence, the role of eradication therapy, surgery,chemotherapy and radiotherapy is critically analyzed.Based on these data, we suggest a therapeutic algorithm that might help to better stratify patients for optimal treatment success.

  16. Cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) in the treatment of stage IE/IIE extranodal natural killer/T cell lymphoma, nasal type: 13-year follow-up in 135 patients.

    Science.gov (United States)

    Wang, Liang; Xia, Zhong-jun; Huang, Hui-qiang; Lu, Yue; Zhang, Yu-jing

    2012-11-01

    We conducted a retrospective study of 135 patients of stage IE/IIE extranodal natural killer/T cell lymphoma, nasal type (ENKTL) treated with CHOP as induction chemotherapy to find some valuable prognostic factors and analyze the usefulness of International Prognostic Index (IPI) and Korean Prognostic Index (KPI) in predicting prognosis. Most of the patients were in the low-risk group (IPI score 0-1). Complete remission (CR) after induction chemotherapy was achieved in 31.8 % of the patients, which increased to 69.6 % after radiotherapy. The 2-, 5-, and 10-year overall survival (OS) rates were 60, 48, and 43 %, respectively. Patients with better performance status (ECOG 0-1), normal serum LDH level, without local invasiveness, low KPI scores, and IPI score of 0 had significantly better overall survival (P KPI score systems should be improved further to classify patients into different groups, and should be validated in larger prospective trials. Due to the multi-drug resistance mechanism of ENKTL, CHOP is no longer the state of art and novel drugs should be incorporated into future treatments.

  17. Detecting EB virus to determine curative effect in extranodal natu-ral killer/T-cell lymphoma%结外NK/T细胞淋巴瘤检测EB病毒与疗效关系的研究

    Institute of Scientific and Technical Information of China (English)

    刘文硕; 南飞飞; 贾思思; 李素彩; 张明智; 张蕾

    2015-01-01

    目的:探讨血浆中EB病毒阳性在评价结外NK/T细胞淋巴瘤近期疗效和远期疗效中的临床意义。方法:收集2011年1月至2014年4月郑州大学第一附属医院经病理及免疫组化确诊为结外NK/T细胞淋巴瘤的患者109例,应用qRT-PCR方法检测其血浆中EBV-DNA拷贝数,比较EBV阴性和EBV阳性患者对治疗疗效与预后的差异。结果:109例结外NK/T细胞淋巴瘤患者,53例阳性患者中有34例(64.2%)为晚期(Ⅲ~Ⅳ期),56例阴性患者中22例(39.3%)为晚期(Ⅲ~Ⅳ期),EBV阳性组中伴有B症状33例(62.3%),阴性组患者21例(37.5%),EBV阴性患者与EBV阳性患者在分期及B症状方面均具有统计学意义(P<0.05)。34例(60.7%)EBV阴性结外NK/T细胞淋巴瘤患者达到客观缓解率,显著高于EBV阳性患者的客观缓解率(22例,41.5%)(P<0.05)。EBV阴性组与EBV阳性组相比,其2年无进展生存期更高(P<0.05)。结论:检测结外NK/T细胞淋巴瘤患者血浆中EB病毒对评价其近期疗效及2年无进展生存期具有重要的临床意义。%Objective:To investigate the clinical significance of detecting Epstein Barr virus (EBV) infection in evaluating recent curative and long-term effects in patients with extranodal natural killer (NK)/T-cell lymphoma. Methods:The EBV-DNA copies in the plasma of 109 patients, who were pathologically and immunohistochemically diagnosed with extranodal natural killer/T-cell lymphoma in the First Affiliated Hospital of Zhengzhou University between January 2011 and April 2014, were monitored via quantitative re-al-time polymerase chain reaction. Subsequently, the difference in recent curative and long-term effects between EBV positive and EBV negative patients was compared. Results:Among the 109 patients with extranodal NK/T-cell lymphoma, 34 (64.2%) cases of EBV posi-tive patients were at the advanced stage (Ⅲ~Ⅳ stages), and 22 (39.3%) cases of EBV

  18. GDP (Gemcitabine, Dexamethasone, and Cisplatin) Is Highly Effective and Well-Tolerated for Newly Diagnosed Stage IV and Relapsed/Refractory Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type.

    Science.gov (United States)

    Wang, Jing-Jing; Dong, Mei; He, Xiao-Hui; Li, Ye-Xiong; Wang, Wei-Hu; Liu, Peng; Yang, Jian-Liang; Gui, Lin; Zhang, Chang-Gong; Yang, Sheng; Zhou, Sheng-Yu; Shi, Yuan-Kai

    2016-02-01

    This study was conducted to evaluate the effectiveness and tolerance of GDP (gemcitabine, dexamethasone, and cisplatin) regimen in patients with newly diagnosed stage IV and relapsed/refractory extranodal natural killer/T-cell lymphoma, nasal type (ENKTL).The study enrolled 41 ENKTL patients who received GDP regimen at the Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College between January 2008 and January 2015.The disease status was newly diagnosed stage IV in 15 patients and relapsed/refractory in 26 patients. The median number of cycles of chemotherapy per patient was 6 (range, 2-8 cycles). The overall response rate and complete-remission rate were 83.0% (34/41) and 41.5% (17/41), respectively. After a median follow-up of 16.2 months, 1-year progression-free survival rate and 1-year overall survival rate for the whole cohort were 54.5% and 72.7%. Grade 3 to 4 adverse events included neutropenia (34.1%), thrombocytopenia (19.5%), and anemia (14.6%).Our study has suggested high efficacy and low toxicity profile of GDP regimen in patients with newly diagnosed stage IV and relapsed/refractory ENKTL.

  19. The number of extranodal sites assessed by PET/CT scan is a powerful predictor of CNS relapse for patients with diffuse large B-cell lymphoma

    DEFF Research Database (Denmark)

    El-Galaly, Tarec Christoffer; Villa, Diego; Michaelsen, Thomas Yssing

    2017-01-01

    Purpose Development of secondary central nervous system involvement (SCNS) in patients with diffuse large B-cell lymphoma is associated with poor outcomes. The CNS International Prognostic Index (CNS-IPI) has been proposed for identifying patients at greatest risk, but the optimal model is unknow...

  20. Lymphoma of the thyroid gland: a clinicopathologic study over a period of five years in a tertiary care center

    Directory of Open Access Journals (Sweden)

    U. Anjit

    2015-06-01

    Full Text Available Background: Primary Thyroid Lymphoma (PTL is uncommon, accounting for only 5% of thyroid neoplasms and less than 2.5 to 7% of extranodal lymphomas. The study aims to analyze the histopathological findings and to correlate it with clinical features. Methods: This study includes cases of PTL received in the department of pathology in a tertiary care center, Kerala, south India over a period of 5 years. Patient details and follow up data were obtained by communication with treating doctors and reviewing the hospital records. All cases had a minimum follow up of 6 months. Results: 15 cases of PTL were diagnosed over the five year period. In total 72 cases of extranodal lymphomas were diagnosed, thus forming 20.83% of extra nodal lymphomas. Lymphocytic thyroiditis was present in 93.3%. Most of the cases were Extranodal Marginal Zone B Cell Lymphoma (EMZBCL and Diffuse Large B Cell Lymphoma (DLBCL. Conclusion: It is important to consider the diagnosis of primary thyroid lymphoma in patients presenting with an enlarging neck mass especially with the history of Hashimoto's thyroiditis. Random microscopic foci of DLBCL or small areas of MZBL could be overlooked examination or missed with limited sampling. The distinction between MZBL and DLBCL in the thyroid is clinically significant. [Int J Res Med Sci 2015; 3(3.000: 579-582

  1. Evans Syndrome Presented with Marginal Zone Lymphoma and Duodenal Neuroendocrine Tumor in an Elderly Woman

    Directory of Open Access Journals (Sweden)

    Daniele D'Ambrosio

    2016-12-01

    Full Text Available Evans syndrome (ES is an autoimmune disorder characterized by simultaneous or sequential development of autoimmune hemolytic anemia, immune thrombocytopenia, and/or neutropenia. ES can be classified as a primary (idiopathic or secondary (associated with an underlying disease syndrome. We report a case of ES in an elderly patient in the presence of multiple trigger factors such as recent influenza vaccine, marginal zone lymphoma, and neuroendocrine tumor G1. Whether this association is casual or causal remains a matter of speculation. It is however necessary to have a thorough work-up in a newly diagnosed ES and a more accurate search of miscellaneous factors especially in elderly patients.

  2. High-Dose Busulfan and High-Dose Cyclophosphamide Followed By Donor Bone Marrow Transplant in Treating Patients With Leukemia, Myelodysplastic Syndrome, Multiple Myeloma, or Recurrent Hodgkin or Non-Hodgkin Lymphoma

    Science.gov (United States)

    2010-08-05

    Accelerated Phase Chronic Myelogenous Leukemia; Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Megakaryoblastic Leukemia (M7); Adult Acute Monoblastic Leukemia (M5a); Adult Acute Monocytic Leukemia (M5b); Adult Acute Myeloblastic Leukemia With Maturation (M2); Adult Acute Myeloblastic Leukemia Without Maturation (M1); Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With T(15;17)(q22;q12); Adult Acute Myeloid Leukemia With T(16;16)(p13;q22); Adult Acute Myeloid Leukemia With T(8;21)(q22;q22); Adult Acute Myelomonocytic Leukemia (M4); Adult Acute Promyelocytic Leukemia (M3); Adult Erythroleukemia (M6a); Adult Nasal Type Extranodal NK/T-cell Lymphoma; Adult Pure Erythroid Leukemia (M6b); Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Burkitt Lymphoma; Childhood Acute Erythroleukemia (M6); Childhood Acute Lymphoblastic Leukemia in Remission; Childhood Acute Megakaryocytic Leukemia (M7); Childhood Acute Monoblastic Leukemia (M5a); Childhood Acute Monocytic Leukemia (M5b); Childhood Acute Myeloblastic Leukemia With Maturation (M2); Childhood Acute Myeloblastic Leukemia Without Maturation (M1); Childhood Acute Myeloid Leukemia in Remission; Childhood Acute Myelomonocytic Leukemia (M4); Childhood Acute Promyelocytic Leukemia (M3); Childhood Chronic Myelogenous Leukemia; Childhood Myelodysplastic Syndromes; Chronic Phase Chronic Myelogenous Leukemia; Cutaneous B-cell Non-Hodgkin Lymphoma; De Novo Myelodysplastic Syndromes; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Peripheral T-Cell Lymphoma; Post-transplant Lymphoproliferative Disorder; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent

  3. A Case of Multiple Extranodal NK/T-cell Lymphoma,Nasal Type%多发性结外鼻型NK/T细胞淋巴瘤1例

    Institute of Scientific and Technical Information of China (English)

    孟慧敏; 王琳; 刘宏杰; 郭在培

    2011-01-01

    患者男,60岁,藏族.全身斑块、结节及肿块6月,肿块破渍伴疼痛2月.皮损病理组织示:表皮变薄,基底层液化变性,瘤细胞在真皮及皮下脂肪层呈弥漫性浸润,瘤细胞形态不一,核深染,可见血管中心性浸润及凝固性坏死.免疫组化标记瘤细胞CD3,CD3e,CD56,TIA-1,粒酶B均为(+),CD30约10%(+).CD8(-),CD79a(-),EBER1/2原位杂交(+).TCR-γ重排未见明确克隆性重排条带.诊断:皮肤结外鼻型NK/T细胞淋巴瘤.%A 60-year-old Tibetan nationality male patient presented with an over six month's history of plaque, nodules and lumps all over his body. Two months ago, the lumps developed to ulceration and accompanied pain. The pathologic features consist of thinning epidermis, liquefaction degeneration of basal cell, diffuse infiltrations of tumor cells in dermis and subcutaneous lipid tissue, which with various shapes and nucleolus hyperchromatism, blood vessels with centricity infiltrations, coagulation necrosis. The immunohistochemitry mark showed the tumor cells were positive with CD3, CD3ε, CD56, TIA-1, GrB and 1/2EBER in situ hybridization, about 10% tumor cells positive with CD30,but negative with CD8 and CD79a, TCR-rearrangement were not identify clone rearrange stripes. Extranodal nasal-type NK/T-cell lymphoma was diagnosed.

  4. Comparative Study of L-Asparaginase-Based LOP Regimen Over CHOP Regimen Before Radiotherapy for Stage IIE Extranodal Nasal Type NK/T Cell Lymphoma: A Study of 2 Centers.

    Science.gov (United States)

    Huang, Limin; Yuan, Bin; Wu, Haixia; Chu, Hongliang; Liu, Yayun; Wu, Shuang; Li, Hong; Lu, He; Chen, Hui

    2017-03-01

    In this study we evaluated the efficacy of an L-asparaginase-based LOP (L-asparaginase, vincristine, and dexamethasone) regimen in extranodal Natural Killer (NK)/T-cell lymphoma (ENKTL) patients in the Guizhou province of China. Forty-eight patients were treated with the LOP (L-asparaginase, vincristine and dexamethasone) regimen chemotherapy (CT) and 32 patients with the CHOP (cyclophosphamide, tetrahydropyanyl adriamycin, vincristine, and prednisone) regimen. These patients then received involved-field radiotherapy (RT) with the doses of DT = 49-59 Gy. A significant improvement of clinical end points with the LOP regimen was noticed compared with the CHOP regimen: 33 (68.8%) versus 16 (50.0%) for complete responses; 10 (20.8%) versus 5 (15.6%) for partial responses. There were statistical differences in objective response rates (43 [89.6%] for LOP vs. 21 [65.6%] for CHOP; P = .009), 3 years of overall survival (42 [87.5%] for LOP vs. 20 [62.5%] for CHOP; P = .006) and progression-free survival (32 [79.2%] for LOP vs. 16 [50.0%] for CHOP; P = .007). The results showed that the LOP regimen is safe and much more efficient than the CHOP regimen for stage IIE ENKTL patients. They indicate that the LOP regimen is a satisfying alternative protocol among the other L-asparaginase-based regimens reported so far, such as SMILE (dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide), GELOX (gemcitabine, oxaliplatin, and L-asparaginase), CHOP-L, and sandwich (CT, then RT, then CT). Copyright © 2017 Elsevier Inc. All rights reserved.

  5. Prognostic value of whole-body metabolic tumour volume and total lesion glycolysis measured on {sup 18}F-FDG PET/CT in patients with extranodal NK/T-cell lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Choon-Young; Hong, Chae Moon; Kim, Do-Hoon; Son, Seung Hyun; Jeong, Shin Young; Lee, Sang-Woo; Lee, Jaetae; Ahn, Byeong-Cheol [Kyungpook National University School of Medicine and Hospital, Department of Nuclear Medicine, Daegu (Korea, Republic of)

    2013-09-15

    The aim of this study was to determine whether maximum standardized uptake value (SUVmax), whole-body metabolic tumour volume (WBMTV), and whole-body total lesion glycolysis (WBTLG) measured on pretreatment {sup 18}F-FDG PET/CT can predict prognosis in patients with extranodal natural killer/T-cell lymphoma (ENKTL). We conducted a retrospective analysis of 20 patients with newly-diagnosed ENKTL who underwent pretreatment {sup 18}F-FDG PET/CT. WBMTV and WBTLG were measured automatically using the boundaries of voxels presenting SUV > 3.0. Uni- and multivariate analyses for survival and disease progression were performed using clinical variables and PET parameters (SUVmax, WBMTV, and WBTLG). During the follow-up period (median 26.3 months), 12 patients showed disease progression and 10 patients died from the disease. Receiver operating characteristic curve analysis showed cut-off values for SUVmax, WBMTV and WBTLG of 8.1, 14.4 cm{sup 3} and 52.7, respectively. Univariate analysis showed that the International Prognostic Index (IPI) score and PET parameters were significant predictors of overall survival (OS) and progression-free survival (PFS). Multivariate analysis, even after adjustment for the IPI score, showed that high WBMTV was the best predictor of OS and PFS, and high SUVmax and WBTLG were significant predictors of PFS. Our results suggested that the use of PET parameters together with the IPI score may be useful for detailed prediction of prognosis in ENKTL patients. Therefore, despite a lower IPI score, patients with high PET parameter values might be considered candidates for aggressive therapy to improve clinical outcomes. (orig.)

  6. Post-treatment plasma EBV-DNA positivity predicts early relapse and poor prognosis for patients with extranodal NK/T cell lymphoma in the era of asparaginase.

    Science.gov (United States)

    Wang, Liang; Wang, Hua; Wang, Jing-hua; Xia, Zhong-jun; Lu, Yue; Huang, Hui-qiang; Jiang, Wen-qi; Zhang, Yu-jing

    2015-10-06

    Circulating Epstein-Barr virus (EBV) DNA is a biomarker of EBV-associated malignancies. Its prognostic value in early stage NK/T-cell lymphoma (NKTCL) in the era of asparaginase was investigated. 68 patients were treated with a median of 4 cycles of asparaginase-based chemotherapy followed by a median of 54.6 Gy (range 50-60 Gy) radiation. The amount of EBV-DNA was prospectively measured in both pretreatment and post-treatment plasma samples by real-time quantitative PCR. At the end of treatment, complete response (CR) rate was 79.4%, and overall response rate (ORR) was 88.2%. Patients with negative pretreatment EBV-DNA had a higher CR rate (96.0% vs. 69.8%, p = 0.023). The 3-year progression-free survival (PFS) rate and overall survival (OS) rate was 71% and 83%, respectively. In multivariate survival analysis, post-treatment EBV-DNA positivity and treatment response (non-CR) were prognostic factors for both worse PFS and OS (p EBV-DNA positivity correlated with inferior PFS and OS (both p EBV-DNA, negative post-treatment EBV-DNA correlated with better PFS and OS (both p EBV-DNA positivity can predict early relapse and poor prognosis for patients with early stage NKTCL in the era of asparaginase, and may be used as an indicator of minimal residual disease.

  7. Paraneoplastic Syndrome in Splenic Marginal Zone Lymphoma: A Rare Phenomenon of Paraplegia as an Atypical Presenting Manifestation

    Directory of Open Access Journals (Sweden)

    Jessica Schering

    2016-01-01

    Full Text Available We describe a case presenting complaint of complete lower body paraparesis, which was discovered to have splenic marginal zone lymphoma (SMZL. While paraneoplastic syndromes are more common in tumors, such as small cell lung cancer, very few reports exist on this condition with SMZL. We describe such a rare entity with a clinical course spanning twenty-four months after diagnosis.

  8. [Clinical characteristics of splenic marginal zone lymphoma with abnormal complete blood count].

    Science.gov (United States)

    Yang, Shen-Miao; Jiang, Qian; Jiang, Bin; Chen, Ding-Bao; Wang, Jing; Jiang, Hao; Lu, Jin; Lu, Xi-Jing; Bao, Li; Shi, Hong-Xia; Liu, Yan-Rong; Huang, Xiao-Jun

    2013-02-01

    The aim of this study was to investigate the clinical and laboratorial characteristics of splenic marginal zone lymphoma (SMZL) with an abnormal complete blood count (CBC). Data of 19 newly diagnosed SMZL patients with abnormal CBC were analyzed retrospectively. Seven patients were diagnosed by using splenic histology, 12 patients who did not undergo splenectomy were diagnosed on the basis of typical clinical presentation and cytologic, immunophenotypic and histologic characteristics of peripheral blood and bone marrow, according to SBLG guidelines. The results showed that leukocytosis (≥ 10.0×10(9)/L) was seen in 5 cases (26.3%); leukocytopenia (complete response was achieved in 9 out of 11 (81.8%) patients. It is concluded that SMZL with abnormal CBC has a higher incidence of cytopenia, bone marrow involvement and autoimmune phenomena. Therapeutic strategies consisting of Rituximab show a better efficacy.

  9. Interference from lupus anticoagulant on von Willebrand factor measurement in splenic marginal zone lymphoma

    DEFF Research Database (Denmark)

    Vinholt, Pernille J; Nybo, Mads

    2015-01-01

    We present a case concerning a patient with splenic marginal zone lymphoma (SMZL) and isolated prolonged activated partial thromboplastin time (aPTT) caused by lupus anticoagulant. Von Willebrand factor (VWF) activity and antigen were immeasurable by latex particle immunoturbidimetric assays......, and several coagulation factor levels were decreased. However, VWF activity and antigen were normal when analyzed by other methods. Also, coagulation factor levels were normal if an aPTT reagent with low lupus anticoagulant sensitivity or a chromogenic method was applied. Altogether, the initial findings were...... because of lupus anticoagulant interference and in fact, the patient had normal VWF activity and coagulation status. Interference of lupus anticoagulant in clot-based assays is well known but has not previously been described in VWF assays. This is furthermore the first report in which lupus anticoagulant...

  10. Serum EBV EA-IgA and VCA-IgA antibodies can be used for risk group stratification and prognostic prediction in extranodal NK/T cell lymphoma: 24-year experience at a single institution.

    Science.gov (United States)

    Huang, Yuhua; Rao, Huilan; Yan, Shumei; Wang, Fang; Wu, Qinian; Feng, Yanfen; Zhang, Yujing

    2017-08-01

    Although extranodal NK/T cell lymphoma (ENKTCL) is consistently associated with Epstein-Barr virus (EBV) infection, the manifestation and prognostic value of serum EBV antibodies still remain unknown. One hundred and forty-one patients with ENKTCL were evaluated for serum EBV EA-IgA and VCA-IgA antibodies levels in the past 24 years in our institution. Their correlation with clinicopathological features, plasma EBV DNA load, and patients' outcomes was analyzed. EBV EA-IgA ≥1:10 and VCA-IgA ≥1:160 were found in 18.4 and 16.3% of patients, respectively. They correlated with adverse ENKTCL profile and inferior overall survival (OS) and progression-free survival (PFS). EA-IgA ≥1:10 was an independent prognostic factor on OS (RR = 2.276, p = 0.008) and associated with lower complete response (CR) rate (34.8 vs 70.6%, p = 0.001) and higher relapse rate in CR patients (62.5 vs 34.7%, p = 0.016). In subgroup analysis, both EA-IgA ≥1:10 and VCA-IgA ≥1:160 significantly correlated with inferior OS and PFS in patients with stage I/II, IPI score 0-1, plasma EBV DNA (+), and CR. Patients with plasma EBV DNA (+) and EA-IgA ≥1:10 (or VCA-IgA ≥1:160) had significantly shorter periods of OS and PFS in comparison with other corresponding groups. Elevated serum EBV EA-IgA and VCA-IgA levels were related to adverse ENKTCL profile and correlated with poor treatment response, early relapse, and poor prognosis in patients with ENKTCL. These findings provide convincing evidence for the use of serum EBV EA-IgA and VCA-IgA antibodies for risk group stratification and prognostic prediction in ENKTCL.

  11. Treatment of splenic marginal zone lymphoma of the CNS with high-dose therapy and allogeneic stem cell transplantation

    Directory of Open Access Journals (Sweden)

    Busemann Christoph

    2012-10-01

    Full Text Available Abstract Therapy of indolent lymphomas with involvement of the central nervous system (CNS has not been standardized so far. A 42-year old male patient presented with neurological signs because of leukemic splenic marginal zone lymphoma (SMZL manifested in bone marrow, lymph nodes and CNS. Due to the aggressiveness of the disease and the young age of the patient, an intensive immunochemotherapy followed by high-dose therapy with busulfan, thiotepa and fludarabine and subsequent unrelated allogeneic stem cell transplantation (alloSCT was performed. The haemopoietic stem cells engrafted in time and the patient is doing well (ECOG 0 without evidence for active lymphoma three years after transplantation. Highly sensitive tests by specific quantitative real-time polymerase chain reaction for presence of lymphoma cells in blood and bone marrow indicated also a molecular remission. The reported case shows the feasibility of high-dose therapy and allogeneic stem cell transplantation in high-risk patients with CNS-involvement of indolent non-Hodgkin’s lymphoma. In addition, the case supports the hypothesis that the graft-versus lymphoma effect after alloSCT is also active within the CNS.

  12. Clinicopathological profile of gastrointestinal lymphomas in Kashmir

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    Mehnaaz Sultan Khuroo

    2016-01-01

    Full Text Available Background: The histological categorization of lymphoma has been a source of controversy for many years for both clinicians and pathologists. Clinicopathologic information of gastrointestinal lymphomas in Indian subcontinent is lacking. We studied histopathological spectrum of Primary Gastrointestinal Lymphomas (PGIL and attempted to classify the G.I. lymphomas based on the recent WHO classification in to major histological types and immunological categories. Material and Methods: This study was done to evaluate the clinicopathological pattern of 100 cases with a histopathological diagnosis of primary gastrointestinal lymphoma at a tertiary care hospital. All patients of primary gastrointestinal lymphomas were included with the help of medical records over a 11-years period that is, January 2005 to December 2015. Results: The study included 100 cases (60 males, 40 females; mean age 51.43 years; age range 4.5-90 years . The disease involved stomach in 82 (82%, small intestine in 8 (8%, large bowel and rectum in 8 (8%, gall bladder in 1 (1% and oesophagus in 1 (1%. 82 (82% of the 100 cases were Diffuse Large B cell lymphomas; 12 (12% were Extra Nodal Marginal Zone Lymphomas (ENMZL of MALT type 2 (2% IPSID 2 (2% of Mantle cell lymphoma morphology, 1 (1% Burkitt's and 1(1% enteropathy associated T cell lymphoma. The commonest presenting symptom was abdominal pain. 99 (99% of 100 tumours were classified as B-cell lymphomas immunohistochemically and majority exhibited monoclonal light chain restriction on kappa/lambda staining. In addition; Burkitt's lymphoma showed positivity for CD 10. One tumour (1% showed positivity for T-cell markers. The data demonstrated that primary GI NHL is more common among males, mainly in their fifth decade. Abdominal pain is the most common presenting symptom, with stomach being the most commonly involved site. Diffuse large cell lymphoma is the most frequent histologic subtype, followed by extranodal marginal-zone B

  13. Atypical Marginal Zone Hyperplasia Is a Mimic for Lymphoma in Pediatric Transplant Recipients: Report of Two Patients.

    Science.gov (United States)

    Caltharp, Shelley A; Qayed, Muna; Park, Sunita I

    2015-01-01

    Atypical marginal zone hyperplasia (AMZH) of mucosa-associated lymphoid tissue (MALT) closely resembles lymphoma in that it shows expansion of the marginal zones with prominent intraepithelial B lymphocytes, is immunoglobulin light-chain restricted, and may show aberrant CD43 expression. However, unlike lymphoma, it does not show rearrangement of the immunoglobulin heavy chain gene (immunoglobulin H [IgH]) by polymerase chain reaction (PCR), and it behaves in a benign fashion. We identified AMZH in 2 pediatric solid organ transplant recipients who presented with adenotonsillar hypertrophy. To date, the patients have experienced a self-limited course in the absence of treatment or reduction of immunosuppression. Atypical marginal zone hyperplasia is a pitfall for posttransplant lymphoproliferative disorder and MALT lymphoma in the pediatric solid organ transplant population. In transplant patients with a lambda-restricted B-cell clone and marginal zone hyperplasia in native MALT sites, PCR for IgH and IgK gene rearrangement is essential to prevent misdiagnosis.

  14. Employment of Oligodeoxynucleotide plus Interleukin-2 Improves Cytogenetic Analysis in Splenic Marginal Zone Lymphoma

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    Antonella Bardi

    2011-01-01

    Full Text Available To compare the efficiency of novel mitogenic agents and traditional mitosis inductors, 18 patients with splenic marginal zone lymphoma (SMZL were studied. Three cultures using oligodeoxynucleotide (ODN plus interleukin-2 (IL-2, or TPA, or LPS were setup in each patient. Seventeen/18 cases with ODN+IL2 had moderate/good proliferation (94,4% as compared with 10/18 cases with TPA and LPS (55% (P=.015; 14/18 (77,7% cases with ODN+IL2 had sufficient good quality of banding as compared with 8/18 cases (44,4% with TPA and LPS. The karyotype could be defined from ODN+IL2-stimulated cultures in all 18 patients, 14 of whom (77,7% had a cytogenetic aberration, whereas clonal aberrations could be documented in 9 and in 3 cases by stimulation with LPS and TPA, respectively. Recurrent chromosome aberrations in our series were represented by aberrations of chromosome 14q in 5 patients, by trisomy 12 and 7q deletion in 4 cases each, and by abnormalities involving 11q and 13q in two cases each. These findings show that stimulation with ODN+IL2 offers more mitotic figures of better quality and results in an increased rate of clonal aberrations in SMZL, making this method ideal for prospective studies aiming at the definition of the prognostic impact of cytogenetic aberrations in this disorder.

  15. Splenic Marginal Zone Lymphoma in the Setting of Noncirrhotic Portal Hypertension.

    Science.gov (United States)

    Ratnayake, Saman; Ammar, Ali; Rezvani, Rodd; Petersen, Greti

    2015-01-01

    We present a case of a 65-year-old Hispanic man with a history of disseminated cutaneous coccidioidomycosis who presented to the emergency room for progressively worsening abdominal pain associated with shortness of breath. The patient was found to have pleural effusion and moderate ascites on physical examination. Abdominal ultrasound and computed tomography scan were consistent with moderate ascites and portal hypertension but negative for both liver cirrhosis and for venous or arterial thrombosis. Cytology of ascitic fluid was suggestive of portal hypertension and was negative for infection. Subsequent, thoracentesis was suggestive of exudative effusion and also negative for infection. Liver biopsy confirmed the absence of cirrhosis. Complete blood count indicated pancytopenia, whereas bone marrow biopsy and flow cytometry were suggestive of marginal zone lymphoma (MZL). Clinically, the patient's shortness of breath was resolved by thoracentesis and paracentesis; however, his abdominal pain persisted. A diagnosis of idiopathic noncirrhotic portal hypertension in the setting of splenic MZL was made. The patient was transferred to a higher level of care for splenectomy; however, he missed multiple appointments. Since discharge, the patient has been seen in the outpatient setting and states that he is controlling his disease with diet and exercise; however, he continues to complain of intermittent shortness of breath with exertion.

  16. Employment of Oligodeoxynucleotide plus Interleukin-2 Improves Cytogenetic Analysis in Splenic Marginal Zone Lymphoma

    Science.gov (United States)

    Bardi, Antonella; Cavazzini, Francesco; Rigolin, Gian Matteo; Tammiso, Elisa; Volta, Eleonora; Pezzolo, Elisa; Formigaro, Luca; Sofritti, Olga; Daghia, Giulia; Ambrosio, Cristina; Rizzotto, Lara; Abass, Awad E.; D'Auria, Fiorella; Musto, Pellegrino; Cuneo, Antonio

    2011-01-01

    To compare the efficiency of novel mitogenic agents and traditional mitosis inductors, 18 patients with splenic marginal zone lymphoma (SMZL) were studied. Three cultures using oligodeoxynucleotide (ODN) plus interleukin-2 (IL-2), or TPA, or LPS were setup in each patient. Seventeen/18 cases with ODN + IL2 had moderate/good proliferation (94, 4%) as compared with 10/18 cases with TPA and LPS (55%) (P = .015); 14/18 (77, 7%) cases with ODN + IL2 had sufficient good quality of banding as compared with 8/18 cases (44, 4%) with TPA and LPS. The karyotype could be defined from ODN + IL2-stimulated cultures in all 18 patients, 14 of whom (77, 7%) had a cytogenetic aberration, whereas clonal aberrations could be documented in 9 and in 3 cases by stimulation with LPS and TPA, respectively. Recurrent chromosome aberrations in our series were represented by aberrations of chromosome 14q in 5 patients, by trisomy 12 and 7q deletion in 4 cases each, and by abnormalities involving 11q and 13q in two cases each. These findings show that stimulation with ODN + IL2 offers more mitotic figures of better quality and results in an increased rate of clonal aberrations in SMZL, making this method ideal for prospective studies aiming at the definition of the prognostic impact of cytogenetic aberrations in this disorder. PMID:21629757

  17. Splenic Marginal Zone Lymphoma in the Setting of Noncirrhotic Portal Hypertension

    Directory of Open Access Journals (Sweden)

    Saman Ratnayake MD

    2015-09-01

    Full Text Available We present a case of a 65-year-old Hispanic man with a history of disseminated cutaneous coccidioidomycosis who presented to the emergency room for progressively worsening abdominal pain associated with shortness of breath. The patient was found to have pleural effusion and moderate ascites on physical examination. Abdominal ultrasound and computed tomography scan were consistent with moderate ascites and portal hypertension but negative for both liver cirrhosis and for venous or arterial thrombosis. Cytology of ascitic fluid was suggestive of portal hypertension and was negative for infection. Subsequent, thoracentesis was suggestive of exudative effusion and also negative for infection. Liver biopsy confirmed the absence of cirrhosis. Complete blood count indicated pancytopenia, whereas bone marrow biopsy and flow cytometry were suggestive of marginal zone lymphoma (MZL. Clinically, the patient’s shortness of breath was resolved by thoracentesis and paracentesis; however, his abdominal pain persisted. A diagnosis of idiopathic noncirrhotic portal hypertension in the setting of splenic MZL was made. The patient was transferred to a higher level of care for splenectomy; however, he missed multiple appointments. Since discharge, the patient has been seen in the outpatient setting and states that he is controlling his disease with diet and exercise; however, he continues to complain of intermittent shortness of breath with exertion.

  18. CAR-pNK Cell Immunotherapy in CD7 Positive Leukemia and Lymphoma

    Science.gov (United States)

    2016-12-04

    Acute Myeloid Leukemia; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma; T-cell Prolymphocytic Leukemia; T-cell Large Granular Lymphocytic Leukemia; Peripheral T-cell Lymphoma, NOS; Angioimmunoblastic T-cell Lymphoma; Extranodal NK/T-cell Lymphoma, Nasal Type; Enteropathy-type Intestinal T-cell Lymphoma; Hepatosplenic T-cell Lymphoma

  19. Therapy and Prognosis Analysis of 47 Patients with Extranodal NK/T-Cell Lymphoma%47例结外NK/T细胞淋巴瘤治疗和预后分析

    Institute of Scientific and Technical Information of China (English)

    张松松; 于力; 魏敏; 李红华; 靖彧; 李菲; 黄文荣; 陆晓林; 刘占祥; 周颖

    2011-01-01

    本研究探讨结外NK/T细胞淋巴瘤的临床特征、治疗和预后情况.对中国人民解放军总医院血液科自1995年10月到2008年12月收治的47例结外NK/T细胞淋巴瘤患者进行回顾性分析.运用Kaplan-Meier方法行生存分析,COX回归分析模型对预后进行多变量分析.采用的临床参数分别为CD56、Ann Arbor分期、国际预后指标和B组症状.结果表明,全组2年、5年总生存(OS)率分别为91%和71%.单纯放疗患者的累积生存率较单纯化疗患者明显提高,二者相比有统计学差异;而放疗与放化疗结合治疗之间无统计学差异.COX回归分析模型多变量分析显示,CD56、Ann Arbor分期是独立的预后因素;单纯化疗中使用CHOP方案治疗的患者以分期为单因素分析显示,分期≥ⅢE期为预后的因素之一;以B症状为单因素分析3种疗法的生存曲线显示,B症状亦为预后因素之一.结论:单纯放疗的近期疗效明显优于单纯化疗.放化疗结合对近期生存率没有显著提高;CD56阴性和Ann Arbor分期较早是患者预后良好的指标,改善病人的一般状况可以使患者的预后更佳.%This study was purposed to explore the clinical characteristics, therapy and prognosis of patients with extranodal NK/T cell lymphoma (ENKL). 47 patients with ENKL from October 1995 to December 2008 in our hospital were analyzed retrospectively. The survival of patients was analyzed by using Kaplan-Meier methods, the prognosis of patients was evaluated by multivariate analysis using COX regression model. The clinical parameters used included CD56, Ann Arbor stage, international prognostic index (IPI) and B symptom. The results showed that the 2- year and 5-year overall survival (OS) rates were 91% ,71% respectively. Multivariant analysis by COX regression showed the CD56 and Ann Arbor stage were independent prognostic factors. Single factor analysis with staging in CHOP chemotherapy group indicated that more than stage

  20. Intravascular large B cell lymphoma

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    Ricardo García-Muñoz

    2014-01-01

    Full Text Available Intravascular large B cell lymphoma (IVBCL is a rare type of extranodal large B cell lymphoma characterized by selective growth of lymphoma cells within the microvasculature. We present an illustrative case of intravascular B cell lymphoma suspected by the presence of a very small monoclonal B cell population identified by immunophenotype and polymerase chain reaction in bone marrow. The diagnosis was confirmed by skin biopsy.

  1. Bilateral Lacrimal Gland Lymphoma in Sjögren Syndrome

    Science.gov (United States)

    Ozsan, Nazan; Sahin, Fahri

    2016-01-01

    A 31-year-old female with Primary Sjögren Syndrome (pSS) presented with bilateral puffiness around the eye for 3 years. The lacrimal glands were hypertrophic and edematous bilaterally. Schirmer 1 score was 2 and 1 mm and tear-film break-up time was 3 and 4 seconds, in the right and the left eyes, respectively. An incisional biopsy from the left lacrimal gland revealed diffuse and intense CD20, CD5, and bcl-2 positivity with negative cyclin D1 and CD23 which supported lymphoma. Upon haematology consultation extranodal marginal zone lymphoma diagnosis was made. CHOP (cyclophosphamide, doxorubicin, vincristine, and methyl prednisolone) treatment was initiated. In conclusion, pSS is a well known autoimmune disease in which increased rate of lymphoma is present. Early detection with histopathologic confirmation and multidisciplinary approach with ophthalmology, rheumatology, and haematology are mandatory in these patients.

  2. Bilateral Lacrimal Gland Lymphoma in Sjögren Syndrome

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    Melis Palamar

    2016-01-01

    Full Text Available A 31-year-old female with Primary Sjögren Syndrome (pSS presented with bilateral puffiness around the eye for 3 years. The lacrimal glands were hypertrophic and edematous bilaterally. Schirmer 1 score was 2 and 1 mm and tear-film break-up time was 3 and 4 seconds, in the right and the left eyes, respectively. An incisional biopsy from the left lacrimal gland revealed diffuse and intense CD20, CD5, and bcl-2 positivity with negative cyclin D1 and CD23 which supported lymphoma. Upon haematology consultation extranodal marginal zone lymphoma diagnosis was made. CHOP (cyclophosphamide, doxorubicin, vincristine, and methyl prednisolone treatment was initiated. In conclusion, pSS is a well known autoimmune disease in which increased rate of lymphoma is present. Early detection with histopathologic confirmation and multidisciplinary approach with ophthalmology, rheumatology, and haematology are mandatory in these patients.

  3. Primary Pancreatic Lymphomas

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    Muhammad Wasif Saif

    2006-05-01

    Full Text Available Extranodal non-Hodgkin’s lymphomas (NHLs represent up to 30-40% of all NHL cases. The gastrointestinal tract is the most commonly involved extranodal site; accounting for about half of such cases [1]. Stomach and the small intestine constitute the most common gastrointestinal sites. Secondary invasion of the pancreas from contiguous, retroperitoneal lymph node disease is the prevalent mode of involvement. Secondary involvement of the pancreas from the duodenum or adjacent peripancreatic lymphadenopathy is well-known. Primary pancreatic lymphoma (PPL is an extremely rare disease [2]. PPL can present as an isolated mass mimicking pancreatic carcinoma. However, unlike carcinomas, PPL are potentially treatable [3].

  4. Splenectomy with chemotherapy vs surgery alone as initial treatment for splenic marginal zone lymphoma

    Institute of Scientific and Technical Information of China (English)

    Rajko Milosevic; Milena Todorovic; Bela Balint; Miodrag Jevtic; Miodrag Krstic; Elizabeta Ristanovic; Nebojsa Antonijevic; Mirjana Pavlovic; Maja Perunicic; Milan Petrovic; Biljana Mihaljevic

    2009-01-01

    AIM:To evaluate the clinical characteristics of splenic marginal-zone lymphoma (SMZL) following antigen expression and the influence of therapeutic approaches on clinical outcome and overall survival (OS).METHODS:A total of 30 patients with typical histological and immunohistochemical SMZL patterns were examined.Splenectomy plus chemotherapy was applied in 20 patients,while splenectomy as a single treatment-option was performed in 10 patients.Prognostic factor and overall survival rate were analyzed.RESULTS:Complete remission (CR) was achieved in 20 (66.7%),partial remission (PR) in seven (23.3%),and lethal outcome due to disease progression occurred in three (10.0%) patients.Median survival of patients with a splenectomy was 93.0 mo and for patients with splenectomy plus chemotherapy it was 107.5 mo (Log rank=0.056,P>0.05).Time from onset of first symptoms to the beginning of the treatment (mean 9.4 mo) was influenced by spleen dimensions,as measured by computerized tomography and ultra-sound (t=2.558,P=0.018).Strong positivity (+++) of CD20 antigen expression in splenic tissue had a positive influence on OS (Log rank = 5.244,P 0.05) effects on the OS.The expression of other antigens (immunohistochemistry) also had no effect on survival-rate,as measured by a χ2 test (P > 0.05).CONCLUSION:Initial splenectomy combined with chemotherapy has been shown to be beneficial due to its advanced remission rate/duration;however,a larger controlled clinical study is required to confirm our findings.

  5. Autoimmune hemolytic anemia as a risk factor of poor outcome in patients with splenic marginal zone lymphoma.

    Science.gov (United States)

    Fodor, Aniko; Molnar, Miklos Zsolt; Krenacs, Laszlo; Bagdi, Eniko; Csomor, Judit; Matolcsy, Andras; Demeter, Judit

    2009-12-01

    Splenic marginal zone lymphoma is a rare disease, accounting for 1% of all lymphomas. We reviewed our single center experience of 13 patients with splenic marginal zone lymphoma (SMZL). Based on the prognostic model developed by Intergruppo Italiano Linfomi, 31% (4/13) of our patients had good, 38% (5/13) had intermediate and 31% (4/13) had a poor prognosis. The presence of two out of three prognostic factors (anemia, elevated LDH, low serum albumin) assignes the patient into the high risk category. In patients with anemia and an elevated LDH due to hemolysis, the outcome seems to be especially poor. Three out of 13 (23%) cases were complicated by autoimmune hemolytic anemia. All patients with autoimmune hemolytic anaemia (AIHA) died 7-28 months after the diagnosis. The mean follow-up time of those nine patients who are still alive is longer than 5 years (36-100 months). Patients with AIHA had significantly (p < 0.001) worse survival than those without AIHA. The main finding of our study is that the presence of AIHA is an adverse prognostic factor in SMZL.

  6. Prevalence of Achromobacter xylosoxidans in pulmonary mucosa-associated lymphoid tissue lymphoma in different regions of Europe.

    Science.gov (United States)

    Adam, Patrick; Czapiewski, Piotr; Colak, Seba; Kosmidis, Perikles; Tousseyn, Thomas; Sagaert, Xavier; Boudova, Ludmila; Okoń, Krzysztof; Morresi-Hauf, Alicia; Agostinelli, Claudio; Pileri, Stefano; Pruneri, Giancarlo; Martinelli, Giovanni; Du, Ming-Qing; Fend, Falko

    2014-03-01

    Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) comprises 7-8% of B-cell lymphomas and commonly originates from a background of long-standing chronic inflammation. An association with distinct bacteria species has been confirmed for several anatomical sites of MALT lymphoma. For pulmonary MALT lymphoma, however, a clear link with an infectious agent or autoimmune disorder has not yet been reported. Using a 16S rRNA gene-based approach, we have recently identified Achromobacter (Alcaligenes) xylosoxidans in eight of nine cases of pulmonary MALT lymphoma. A. xylosoxidans is a gram-negative betaproteobacterium with low virulence, but high resistance to antibiotic treatment. To further examine a potential association with A. xylosoxidans, 124 cases of pulmonary MALT lymphoma and 82 control tissues from six European countries were analysed using a specific nested PCR. Although prevalence rates for A. xylosoxidans varied significantly from country to country, they were consistently higher for MALT lymphoma as compared to controls. Overall, 57/124 (46%) pulmonary MALT lymphomas and 15/82 (18%) control tissues were positive for A. xylosoxidans (P = 0·004). Whether the significant association of A. xylosoxidans with pulmonary MALT lymphoma demonstrated in our study points to a potential causal role in the pathogenesis of this lymphoma will require further studies.

  7. Correlation of {sup 18}F-FDG PET/CT and Endoscopic Findings of Twin Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma of the Stomach: Report of a Case

    Energy Technology Data Exchange (ETDEWEB)

    Bahk, Yong Whee; Choi, Jin Wook [SungAe Hospital, Seoul (Korea, Republic of)

    2008-02-15

    Mucosa-associated lymphoid tissue (MALT) type lymphoma arises from extranodal marginal zone B-cell. It is etiologically associated with Helicobacter pylori infection and, hence, can be cured by antibiotic treatment. MALT type lymphoma is the most common variety of gastric lymphoma that is rare in the stomach). The published data of clinical studies on the usefulness of 18F-FDG PET in the diagnosis of MALT type lymphoma varied according to authors. Thus, the result of Hoffmann et al. was discouraging whereas a high diagnostic accuracy was reported by Ambrosini et al. The latter group further went to suggest that higher 18F-FDG uptake in gastric MALT type lymphoma would positively relate to the aggressiveness of neoplasm. The clinical studies conducted by other groups on MALT lymphomas of the stomach, lung, orbit and parotid gland and the stomach, lung, parotid gland, skin, orbit, mandible, esophagus and uterus confirmed that 18F-FDG scan is valuable.

  8. Primary endobronchial marginal zone B-cell lymphoma of bronchus-associated lymphoid tissue: CT findings 7 patients

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Ra Gyoung; Kim, Mi Young; Song, Jae Woo; Chae, Eun Jin; Choi, Chang Min; Jang, Se Jin [University of Ulsan College of Medicine, Seoul (Korea, Republic of)

    2013-04-15

    To investigate CT and 1{sup 8F}-fluorodeoxyglucose (1{sup 8F}-FDG) positron-emission tomography/CT findings of primary endobronchial marginal zone B-cell lymphoma of the bronchus-associated lymphoid tissue (BALT). From June 2006 through April 2012, seven patients (six female, one male; age range, 21-61 years; mean age, 49 years) were examined who were pathologically diagnosed with the primary endobronchial marginal zone B-cell lymphoma of BALT. We evaluated the locations and characteristics of the lesions on CT and 1{sup 8F}-FDG-PET/CT scans. The lesions were classified into the following three patterns: 1) solitary intraluminal nodule; 2) several tiny nodular protrusions; and 3) diffuse wall thickening. A solitary intraluminal nodule was observed in four patients (57.1%), several tiny nodular protrusion in two patients (28.6%), and diffuse wall thickening in one patient (14.3%). The lesions were categorized into 3 major locations: confined to the trachea (n 3), confined to the lobar bronchus (n = 2), and diffuse involvement of the trachea and both main bronchi (n = 2). All lesions demonstrated homogeneous iso-attenuation as compared with muscle on pre- and post-enhancement scans. Secondary findings in the lungs (n = 3; 42.9%) included postobstructive lobar atelectasis (n = 1), air trapping (n = 1), and pneumonia (n = 1). On 1{sup 8F}-FDG-PET/CT (n = 5), 4 lesions showed homogeneous uptake with maximum standardized uptake values (mSUV), ranging 2.3-5.7 (mean mSUV: 3.3). One lesion showed little FDG uptake. Primary endobronchial marginal zone B-cell lymphoma of the BALT manifests as three distinct patterns on CT, with the solitary intraluminal nodule presenting as the main pattern. Most lesions demonstrate homogeneous but weak FDG uptake on 1{sup 8F}-FDG-PET/CT.

  9. Primary B-Cell Mucosa-Associated Lymphoid Tissue Lymphoma of the Hard Palate and Parotid Gland: Report of One Case and Review of the Literature

    Science.gov (United States)

    Yonal-Hindilerden, Ipek; Hindilerden, Fehmi; Arslan, Serkan; Turan-Guzel, Nalan; Dogan, Ibrahim Oner; Nalcaci, Meliha

    2016-01-01

    A 61-year-old woman was admitted to our hospital with an ulcerated palate mass and swelling of the right parotid gland. Incisional biopsy from the hard palate revealed an extranodal marginal zone B-cell lymphoma, also called mucosa-associated lymphoid tissue (MALT) lymphoma. Final diagnosis was MALT lymphoma of the parotid gland with concomitant involvement of an extremely seldom site of involvement: the hard palate. To our knowledge, this report illustrates the first case of MALT lymphoma of the hard palate and parotid gland without an underlying autoimmune disease. Rituximab-based combination regimen (R-CHOP) provided complete remission with total regression of mass lesions at the hard palate and parotid gland. At 44-month follow-up, there is no disease relapse. We adressed the manifestations and management of MALT lymphoma patients with involvement of salivary gland and oral cavity. PMID:27738485

  10. Morphological and immunophenotypical features of hairy cell leukaemia involving lymph nodes and extranodal tissues.

    Science.gov (United States)

    Cortazar, Jacqueline M; DeAngelo, Daniel J; Pinkus, Geraldine S; Morgan, Elizabeth A

    2017-07-01

    Hairy cell leukaemia (HCL) is a rare B cell neoplasm that mainly affects bone marrow (BM), peripheral blood (PB) and spleen. Involvement of lymph nodes and extranodal structures is considered infrequent. Herein we describe our institutional experience of nodal (n = 10) and extranodal (n = 3) HCL during a 30-year period. Ten patients had prior evidence of HCL within the BM or PB at a median 35.8 months before nodal/extranodal diagnosis (range: <1-175 months), and HCL was diagnosed concurrently within the bone marrow of one additional patient. Nodal involvement showed distinct architectural patterns, including diffuse (62% of cases), sinusoidal (25%) and nodular (13%). Extranodal involvement was characterized as diffuse infiltration through underlying structures in all cases. Morphological features ranged from classic 'fried-egg' cytology to a plasmacytoid appearance. Nodal/extranodal disease showed an overlapping immunophenotypical profile with other small B cell lymphomas, including expression of cyclin D1 (70%), CD43 (55%), CD10 (38%) and CD5 (8%). Rates of both CD43 and CD10 reactivity were higher than described previously in leukaemic HCL, suggesting that expression may be enriched in cases with extramedullary extension. Although uncommon, HCL should be considered in the differential diagnosis of small B cell neoplasms involving nodal/extranodal sites, given the therapeutic implications. In particular, recent discoveries including detection of the BRAF(V)(600E) mutation in nearly all cases of HCL and the availability of an antibody to CD103 for use in paraffin-embedded tissues will facilitate the distinction of HCL from other small B cell lymphomas in the nodal/extranodal setting. © 2017 John Wiley & Sons Ltd.

  11. Clinical analysis of 27 cases with nasal type extranodal NK/T cell lymphoma%结外鼻型NK/T细胞淋巴瘤27例临床分析

    Institute of Scientific and Technical Information of China (English)

    李楠; 李彩霞; 刘红; 叶璐; 马超; 吴德沛

    2011-01-01

    Objective To analyze the clinical characteristic of the nasal type extranodal NK/T cell lymphoma (ENKL) as well as the therapeutic effects for different treatment methods and element that would affect prognosis.Methods Review and analyze the 27 patients with pathologically confirmed ENKL patients received radiotherapy (3 cases),chemotherapy (9 cases),combination of radiotherapy and chemotherapy (15 cases),3 of whom received auto-HSCT.Detailed B symptoms,lactate dehydrogenase (LDH),performance status (PS) scores,international prognostic index (IPI),Ann Arbor stage and therapeutic modality were included in univariate analysis.Results The average overall survival time was 32 (2-42) months.The 1,2and 3 year overall survival (OS) rates were 79.5 %,71.6 %,53.7 %,respectively.The 2-year overall survival of patients who was early stage (stage Ⅰ + Ⅱ ) was 83.3 %,and those who was advanced stage(stage Ⅲ+Ⅳ) was 62.3 % (P =0.368).Among the patients in early stage,after radiotherapy or chemotherapy,4 cases achieved OR (overall response) which is complete remission (CR) or partial remission (PR),9/10 in combination of radiotherapy and chemotherapy achieved OR. While among those in their advantage stage,the OR in chemotherapy is 2 cases,2/5 in combination of radiotherapy and chemotherapy.In univariate analysis,age and short-term response were main factors of survival (P <0.05).Conclusion To treat the nasal ENKL in the early stage,radiotherapy can achieve a better effect,while combination of radiotherapy and chemotherapy is an important method for patients in advantage stage.Age and short-term response may be.prognostic factors of nasal type ENKL.%目的 分析结外鼻型NK/T细胞淋巴瘤(ENKL)的临床特征、不同治疗方法的疗效及影响预后的因素.方法 回顾性分析27例ENKL患者,予单纯放疗3例,单纯化疗9例,其余15例采用放化疗联合治疗,其中3例患者行自体造血干细胞移植(auto-HSCT).对B组症状、乳酸脱

  12. [Central nervous system relapse in diffuse large B cell lymphoma: Risk factors].

    Science.gov (United States)

    Sancho, Juan-Manuel; Ribera, Josep-Maria

    2016-01-15

    Central nervous system (CNS) involvement by lymphoma is a complication associated, almost invariably, with a poor prognosis. The knowledge of the risk factors for CNS relapse is important to determine which patients could benefit from prophylaxis. Thus, patients with very aggressive lymphomas (such as lymphoblastic lymphoma or Burkitt's lymphoma) must systematically receive CNS prophylaxis due to a high CNS relapse rate (25-30%), while in patients with indolent lymphoma (such as follicular lymphoma or marginal lymphoma) prophylaxis is unnecessary. However, the question about CNS prophylaxis in patients with diffuse large B-cell lymphoma (DLBCL), the most common type of lymphoma, remains controversial. The information available is extensive, mainly based on retrospective and heterogeneous studies. There seems that immunochemotherapy based on rituximab reduces the CNS relapse rate. On the other hand, patients with increased serum lactate dehydrogenase plus more than one extranodal involvement seem to have a higher risk of CNS relapse, but a prophylaxis strategy based only on the presence of these 2 factors does not prevent all CNS relapses. Patients with involvement of testes or breast have high risk of CNS relapse and prophylaxis is mandatory. Finally, CNS prophylaxis could be considered in patients with DLBCL and renal or epidural space involvement, as well as in those cases with MYC rearrangements, although additional studies are necessary. Copyright © 2015 Elsevier España, S.L.U. All rights reserved.

  13. Bilateral primary breast lymphoma

    Institute of Scientific and Technical Information of China (English)

    Jung Im Yi; Byung Joo Chae; Ja Seong Bae; Bong Joo Kang; Ahwon Lee; Byung Joo Song; Sang Seol Jung

    2010-01-01

    @@ Primary breast lymphoma (PBL) is rare, accounting for 0.04%-0.50% of breast malignancies and 1.7% of extranodal lymphoma.1,2 The originally described diagnostic criteria for PBL2 remains the standard definition for this disease. These criteria are breast location as the clinical site of presentation, absence of history of previous lymphoma or evidence of widespread disease at diagnosis, close association of lymphoma with breast tissue in pathologic specimens, and involvement of ipsilateral lymph nodes if they develop simultaneously with PBL.

  14. Gastrointestinal lymphomas in a North American population: clinicopathologic features from one major Central-Midwestern United States tertiary care medical center

    Directory of Open Access Journals (Sweden)

    Warrick Joshua

    2012-06-01

    Full Text Available Abstract Background Gastrointestinal (GI lymphomas are very common types of extranodal lymphomas, and we hypothesize there are regional differences in subtype, distribution in the GI tract, and epidemiological features among the different populations. Methods We retrospectively evaluated the clinical, molecular and histologic features of North American primary and secondary GI lymphomas diagnosed from 2000–2009 seen at our institution. We utilized immunohistochemistry and fluorescence in situ hybridization to further evaluate a subset of the gastric lymphomas. Results Extranodal marginal zone lymphomas of mucosal associated lymphoid tissue (MALTs and diffuse large B cell lymphomas (DLBCLs were the most common subtypes of GI lymphomas. Select gastric DLBCLs (N = 6 and MALTs (N = 13 were further examined for API2-MALT1 and IGH translocations, and P16 and P53 protein expression. Gastric MALTs showed frequent API2-MALT1 (38% but not IGH translocations (0%, and the DLBCLs showed neither translocation. Expression of P16 and P53 proteins and the proliferative index were compared between high grade gastric lymphomas (gastric DLBCLs and low grade gastric lymphomas (gastric MALTs. P53 overexpression (P = 0.008 and a high proliferation index [Ki-67] (P = 0.00042 were significantly associated with gastric DLBCL, but no statistically significant difference was observed in P16 expression (p = 0.108 between gastric DLBCL and gastric MALT. Conclusion Our study revealed that GI lymphomas from a Central-Midwestern North American population showed differences and similarities to non-North American cohorts. In addition, API2-MALT1, P16 and P53 abnormalities occurred frequently in gastric lymphomas from this North American population. Virtual slides The virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1415505838687793

  15. Results of a randomized trial of chlorambucil versus fludarabine for patients with untreated Waldenström macroglobulinemia, marginal zone lymphoma, or lymphoplasmacytic lymphoma.

    Science.gov (United States)

    Leblond, Véronique; Johnson, Steve; Chevret, Sylvie; Copplestone, Adrian; Rule, Simon; Tournilhac, Olivier; Seymour, John Francis; Patmore, Russell D; Wright, David; Morel, Pierre; Dilhuydy, Marie-Sarah; Willoughby, Sara; Dartigeas, Caroline; Malphettes, Marion; Royer, Bruno; Ewings, Maeve; Pratt, Guy; Lejeune, Julie; Nguyen-Khac, Florence; Choquet, Sylvain; Owen, Roger G

    2013-01-20

    Treatment options for patients with Waldenström macroglobulinemia (WM) and closely related disorders include alkylating agents, purine analogs, and monoclonal antibodies. No large randomized studies have yet been reported comparing any of these approaches. The randomized WM1 study (Trial Comparing Chlorambucil to Fludarabine in Patients With Advanced Waldenström Macroglobulinemia) was undertaken in 101 centers in five countries enrolling 414 eligible patients (339 with WM, 37 with non-mucosa-associated lymphoid tissue marginal zone lymphoma, and 38 with lymphoplasmacytic lymphoma) who were randomly assigned to receive chlorambucil or fludarabine. The primary end point was the overall response rate (ORR). On the basis of intent-to-treat analysis, the ORR was 47.8% (95% CI, 40.9% to 54.8%) in the fludarabine arm versus 38.6% (95% CI, 32.0% to 45.7%) in the chlorambucil arm (P = .07). With a median follow-up of 36 months (interquartile range, 18 to 58 months), median progression-free survival (PFS), and duration of response (DR) were significantly improved in the fludarabine arm compared with the chlorambucil arm: PFS, 36.3 versus 27.1 months (P = .012) and DR, 38.3 versus 19.9 months (P chlorambucil arm (95% CI, 61.6 to 79.8 months; P = .014). Grade 3 to 4 neutropenia was significantly higher among patients treated with fludarabine (36%) compared with patients treated with chlorambucil (17.8%; P chlorambucil arm with 6-year cumulative incidence rate of 20.6% versus 3.7% in the fludarabine arm (P = .001). In the complete intent-to-treat study population, fludarabine significantly improved PFS compared with chlorambucil, and in patients with WM, it improved OS.

  16. Ocular Adnexal Lymphoma Presenting as Refractory Unilateral Open-angle Glaucoma.

    Science.gov (United States)

    Sears, Nathaniel C; Singh, Annapurna; Singh, Arun D

    2016-08-01

    We report a single case of masquerade glaucoma caused by increased episcleral venous pressure from adnexal lymphoma. The patient presented as a referral for unilateral glaucoma with intraocular pressures (IOPs) consistently >40 mm Hg (right eye). We present data conclusively demonstrating extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue in the involved eye, and provide an account of the treatment of the tumor with sustained regression and complete resolution of his elevated IOP. We conclude with a discussion of the proposed mechanism. This case serves as a reminder that unilateral open-angle glaucoma can be a sign of indolent ocular adnexal lymphoma. The case also provides a useful model for increased IOP with orbital lesions.

  17. Indolent B-Cell Lymphoid Malignancy in the Spleen of a Man Who Handled Benzene: Splenic Marginal Zone Lymphoma.

    Science.gov (United States)

    Lee, Jihye; Kang, Young Joong; Ahn, Jungho; Song, Seng-Ho

    2017-09-01

    We present the case of a 45-year-old man with a history of benzene exposure who developed splenic marginal zone lymphoma. For 6 years, he had worked in an enclosed space cleaning instruments with benzene. He was diagnosed with splenic marginal zone lymphoma 19 years after retirement. During his time of working in the laboratory in the 1980s, working environments were not monitored for hazardous materials. We indirectly estimated the cumulative level of past benzene exposure using job-exposure matrices and technical assumptions. Care must be taken in investigating the relevance of occupational benzene exposure in the occurrence of indolent B-cell lymphoma. Because of the long latency period and because occupational measurement data do not exist for the period during the patient's exposure, the epidemiological impact of benzene exposure may be underestimated.

  18. Gastric marginal zone lymphoma of mucosa-associated lymphoid tissue and signet ring cell carcinoma, synchronous collision tumour of the stomach: a case report.

    Science.gov (United States)

    George, Smiley Annie; Junaid, T A

    2014-01-01

    To report a rare case of synchronous marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) signet ring cell carcinoma occurring as a collision tumour in the stomach. A 53-year-old man was diagnosed initially with signet ring cell carcinoma of the stomach. The microscopy of the subsequent total gastrectomy revealed a collision tumour of MALT lymphoma and signet ring cell carcinoma associated with Helicobacter pylori gastritis. This case highlighted the importance of a careful evaluation of the accompanying lymphoid population in the biopsy samples of gastric adenocarcinoma and underlined the need for multiple endoscopic biopsies to detect these rare synchronous tumours. © 2013 S. Karger AG, Basel.

  19. Clinicopathological Analysis of Pulmonary Marginal Zone B-cell Lymphoma of 
Mucosa-associated Lymphoid Tissue

    Directory of Open Access Journals (Sweden)

    Jing ZENG

    2011-05-01

    Full Text Available Background and objective As a rare disease, pulmonary marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (PMZL-MALT, is often misdiagnosed. The aim of this study is to summarize the clinical and pathological features of this disease and improve the awareness of doctors. Methods Seven cases (female 5, male 2 diagnosed of PMZL-MALT in West China Hospital between November 2008 and November 2010, were analyzed retrospectively, including their symptoms, radiological findings, pathological examinations, treatment and prognosis. Results The median age of the patients were 62 years old (range 34-79 years. Six patients suffered from cough and sputum. Pulmonary consolidation was the most frequent manifestation, leading a misdiagnosis of pneumonia with CT examinations. Pathological diagnosis was obtained via fiberoptic bronchoscopy in six patients and percutaneous pulmonary biopsy for the rest one. In the seven cases, immunohistochemical results showed CD20(+, CD79a(+, while CD3 epsilon(-, CD5(-, CyclinD1(-, CD10(-, Bcl-2(- and CD30(-. Additionally, the expression of Ki-67 was below 10%. Further PCR analysis showed evidence of immunoglobulin heavy chain gene rearrangement in tissues from six subjects. Based on the disease location and patients’ wishes, compared with two cases just receiving symptomatic treatments, the other five ones took in chemotherapies. Conclusion Since there were no specific clinical features for patients of PMZL-MALT, histopathological examination was the only effective means to confirm the diagnosis.

  20. Prevalence of Borrelia burgdorferi infection in a series of 98 primary cutaneous lymphomas.

    Science.gov (United States)

    Ponzoni, Maurilio; Ferreri, Andrés J M; Mappa, Silvia; Pasini, Elisa; Govi, Silvia; Facchetti, Fabio; Fanoni, Daniele; Tucci, Alessandra; Vino, Arianna; Doglioni, Claudio; Berti, Emilio; Dolcetti, Riccardo

    2011-01-01

    Borrelia burgdorferi has been variably associated with different forms of primary cutaneous lymphoma. Differences in prevalence rates among reported studies could be a result of geographic variability or heterogeneity in the molecular approaches that have been employed. In the present study, we investigated the prevalence of Borrelia burgdorferi sensu lato DNA in diagnostic tissue samples from fresh cutaneous biopsies of 98 primary cutaneous lymphomas and 19 normal skin controls. Three different polymerase chain reaction (PCR) protocols targeting the hbb, flagellin, and Osp-A genes were used. Direct sequencing of both sense and antisense strands of purified PCR products confirmed the specificity of the amplified fragments. Sequence specificity was assessed using the Basic Local Alignment Search Tool, and MultAlin software was used to investigate the heterogeneity of target gene sequences across the different samples. Borrelia DNA was not detected in 19 controls, 23 cases of follicular lymphoma, 31 cases of extranodal marginal zone lymphoma, or 30 cases of mycosis fungoides. A single case of 14 diffuse large B-cell lymphoma cases was positive for B. burgdorferi. This study does not support a pathogenic role of B. burgdorferi in primary cutaneous B- and T-cell lymphomas from areas nonendemic for this microorganism and the consequent rationale for the adoption of antibiotic therapy in these patients.

  1. Dendritic Cell Therapy, Cryosurgery, and Pembrolizumab in Treating Patients With Non-Hodgkin Lymphoma

    Science.gov (United States)

    2017-01-26

    Aggressive Non-Hodgkin Lymphoma; Indolent Non-Hodgkin Lymphoma; Recurrent Adult Non-Hodgkin Lymphoma; Recurrent Diffuse Large B-Cell Lymphoma; Recurrent Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mediastinal (Thymic) Large B-Cell Cell Lymphoma; Recurrent T-Cell Non-Hodgkin Lymphoma; Small Lymphocytic Lymphoma

  2. A Case of Contiguous Primary Hepatic Marginal Zone B-Cell Lymphoma and Hemangioma Ultimately Diagnosed Using Contrast-Enhanced Ultrasonography

    Directory of Open Access Journals (Sweden)

    Kazue Shiozawa

    2015-02-01

    Full Text Available Primary hepatic marginal zone B-cell malignant lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma is extremely rare. We present a case in which a lesion was diagnosed as 2 contiguous tumors (MALT lymphoma and hemangioma using contrast-enhanced ultrasonography (US with sonazoid. There has been no previous case of contiguous hepatic MALT lymphoma and hemangioma. The present case was a female with no medical history. We detected a snowman-like appearance, which was a tumor of 15 mm in diameter with hypo- and hyper-echogenicities in the lateral and medial parts, respectively, in the Couinaud's segment (S6 of the liver on US. The tumor appeared as a single lesion with a low-density area in the unenhanced phase and prolonged enhancement in the equilibrium phases on dynamic CT. On MRI, the whole lesion showed a low-intensity signal on T1-weighted imaging, but isointensity in the lateral part and high intensity in the medial part were seen on T2-weighted imaging. On contrast-enhanced US, the lateral hypoechoic region was homogenously hyperenhanced in the early vascular phase, and the contrast medium was washed out after about 30 s; in contrast, the medial hyperechoic region was gradually stained from the margin toward the central region. The tumor showed a defect in both hypo- and hyperechoic regions in the postvascular phase. Hemangioma was suspected for the medial part based on the typical image findings, but the lateral part was not given a diagnosis. Thus, surgical resection was performed. The medial part was a hemangioma, and the lateral part was a MALT lymphoma by histopathological findings.

  3. Primary Gallbladder Lymphoma in a Male Patient with No Risk Factors Detected Incidentally by CT Colonography

    Science.gov (United States)

    Karia, Monil; Mitsopoulos, Grigorios; Patel, Ketan; Rafique, Akkib; Sheth, Hemant

    2015-01-01

    Primary gallbladder lymphoma, although rare, usually presents in females with symptoms mimicking cholecystitis. We present a rare case of primary gallbladder in an 81-year-old male with no risk factors whose only symptom was weight loss. Routine blood tests including liver function tests were unremarkable. A CT colonography was carried out to exclude colonic malignancy. Unilateral gallbladder wall thickening and lymphadenopathy were incidentally detected and confirmed by ultrasound and a decision for the patient to undergo laparoscopic cholecystectomy and intraoperative cholangiogram was made. Histology confirmed extranodal marginal zone lymphoma with follow-up staging and biopsy of the bone marrow not demonstrating spread. Cholecystectomy was therefore deemed curative and no adjuvant therapy was necessary. Thickening of the gallbladder wall on any imaging with or without symptoms should not be ignored or assumed to be cholecystitis, even in males with no risk factors. In these patients urgent cholecystectomy with intraoperative cholangiogram is indicated with histology and haematology follow-up. PMID:26587306

  4. An unusual presentation of gastric mucosa-associated lymphoid tissue (MALT-type lymphoma

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    Bikram Shrestha

    2016-09-01

    Full Text Available Mucosa-associated lymphoid tissue (MALT-type lymphoma is a relatively rare disease; nevertheless, it is the third most common lymphoma type, accounting for 5–7% of all non-Hodgkin lymphomas. Case series and retrospective analysis published in the literature have suggested that extra gastrointestinal (GI MALT-type lymphoma can occur simultaneously with MALT-type lymphoma involving the GI tract. We report the case of a healthy, 64-year-old Caucasian male who presented with progressive fatigue, non-productive cough, and worsening exertional shortness of breath for 3 months who was subsequently diagnosed with gastric extra-nodal marginal zone B-cell lymphoma or MALToma with simultaneous metastasis to the lung (bronchi based on biopsy reports. Case presentation: A 64-year-old Caucasian male presented to the emergency room complaining of progressive fatigue for 3 months which had progressed to the point of hindering his usual activities of daily living (ADL. He had recently visited his primary care provider for evaluation of a non-productive cough and exertional shortness of breath. A chest radiography obtained at the time showed bilateral infiltrates. He was then treated for atypical pneumonia but his symptoms unfortunately did not improve. Initial investigations in the emergency room revealed severe anemia and a positive stool guaiac test. Imaging showed bilateral pulmonary infiltrates and an irregular gastric mass. Gastric and transbronchial biopsies were suggestive of extra-nodal marginal zone B-cell lymphoma with simultaneous metastasis to the bronchi. He was treated symptomatically with transfusion of packed red blood cells (PRBC and intravenous iron followed by radiotherapy. Helicobacter pylori infection was ruled out eliminating the possibility of treating him with eradication therapy. Conclusion: Although the stomach is the most common and most extensively studied site of involvement of MALT lymphomas, they can also emerge in many other

  5. Entospletinib and Obinutuzumab in Treating Patients With Relapsed Chronic Lymphocytic Leukemia, Small Lymphocytic Lymphoma, or Non-Hodgkin Lymphoma

    Science.gov (United States)

    2017-07-24

    Anemia; B-Cell Prolymphocytic Leukemia; Fatigue; Fever; Grade 1 Follicular Lymphoma; Grade 2 Follicular Lymphoma; Grade 3a Follicular Lymphoma; Hairy Cell Leukemia; Lymphadenopathy; Lymphocytosis; Lymphoplasmacytic Lymphoma; Mantle Cell Lymphoma; Marginal Zone Lymphoma; Night Sweats; Recurrent Chronic Lymphocytic Leukemia; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Small Lymphocytic Lymphoma; Richter Syndrome; Splenomegaly; Thrombocytopenia; Weight Loss

  6. Primary gastrointestinal lymphoma

    Institute of Scientific and Technical Information of China (English)

    Prasanna Ghimire; Guang-Yao Wu; Ling Zhu

    2011-01-01

    Gastrointestinal tract is the most common extranodal site involved by lymphoma with the majority being non-Hodgkin type. Although lymphoma can involve any part of the gastrointestinal tract, the most frequent sites in order of its occurrence are the stomach followed by small intestine and ileocecal region. Gastrointestinal tract lymphoma is usually secondary to the widespread nodal diseases and primary gastrointestinal tract lymphoma is relatively rare. Gastrointestinal lymphomas are usually not clinically specific and indistinguishable from other benign and malignant conditions. Diffuse large B-cell lymphoma is the most common pathological type of gastrointestinal lymphoma in essentially all sites of the gastrointestinal tract, although recently the frequency of other forms has also increased in certain regions of the world. Although some radiological features such as bulky lymph nodes and maintenance of fat plane are more suggestive of lymphoma, they are not specific,thus mandating histopathological analysis for its definitive diagnosis. There has been a tremendous leap in the diagnosis, staging and management of gastrointestinal lymphoma in the last two decades attributed to a better insight into its etiology and molecular aspect as well as the knowledge about its critical signaling pathways.

  7. Primary cutaneous B-cell lymphoma other than marginal zone: clinicopathologic analysis of 161 cases: Comparison with current classification and definition of prognostic markers.

    Science.gov (United States)

    Lucioni, Marco; Berti, Emilio; Arcaini, Luca; Croci, Giorgio A; Maffi, Aldo; Klersy, Catherine; Goteri, Gaia; Tomasini, Carlo; Quaglino, Pietro; Riboni, Roberta; Arra, Mariarosa; Dallera, Elena; Grandi, Vieri; Alaibac, Mauro; Ramponi, Antonio; Rattotti, Sara; Cabras, Maria Giuseppina; Franceschetti, Silvia; Fraternali-Orcioni, Giulio; Zerbinati, Nicola; Onida, Francesco; Ascani, Stefano; Fierro, Maria Teresa; Rupoli, Serena; Gambacorta, Marcello; Zinzani, Pier Luigi; Pimpinelli, Nicola; Santucci, Marco; Paulli, Marco

    2016-10-01

    Categorization of primary cutaneous B-cell lymphomas (PCBCL) other than marginal zone (MZL) represents a diagnostic challenge with relevant prognostic implications. The 2008 WHO lymphoma classification recognizes only primary cutaneous follicular center cell lymphoma (PCFCCL) and primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT), whereas the previous 2005 WHO/EORTC classification also included an intermediate form, namely PCDLBCL, other. We conducted a retrospective, multicentric, consensus-based revision of the clinicopathologic characteristics of 161 cases of PCBCL other than MZL. Upon the histologic features that are listed in the WHO classification, 96 cases were classified as PCFCCL and 25 as PCDLBCL-LT; 40 further cases did not fit in the former subgroups in terms of cytology and/or architecture, thus were classified as PCDLBCL, not otherwise specified (PCDLBCL-NOS). We assigned all the cases a histogenetic profile, based on the immunohistochemical detection of CD10, BCL6, and MUM1, and a "double hit score" upon positivity for BCL2 and MYC. PCDLBCL-NOS had a clinical presentation more similar to PCFCCL, whereas the histology was more consistent with the picture of a diffuse large B-cell lymphoma, as predominantly composed of centroblasts but with intermixed a reactive infiltrate of small lymphocytes. Its behavior was intermediate between the other two forms, particularly when considering only cases with a "non-germinal B-cell" profile, whereas "germinal center" cases resembled PCFCCL. Our data confirmed the aggressive behavior of PCDLBC-LT, which often coexpressed MYC and BCL2. The impact of single factors on 5-year survival was documented, particularly histogenetic profile in PCDLBCL and BCL2 translocation in PCFCCL. Our study confirms that a further group-PCDLBCL-NOS-exists, which can be recognized through a careful combination of histopathologic criteria coupled with adequate clinical information.

  8. Bone marrow histopathology in the diagnostic evaluation of splenic marginal-zone and splenic diffuse red pulp small B-cell lymphoma: a reliable substitute for spleen histopathology?

    Science.gov (United States)

    Ponzoni, Maurilio; Kanellis, George; Pouliou, Evi; Baliakas, Panagiotis; Scarfò, Lydia; Ferreri, Andrés J M; Doglioni, Claudio; Bikos, Vasilis; Dagklis, Antonis; Anagnostopoulos, Achilles; Ghia, Paolo; Stamatopoulos, Kostas; Papadaki, Theodora

    2012-11-01

    Primary splenic small B-cell lymphomas mostly comprise the distinct entity of splenic marginal-zone lymphoma (SMZL) and the provisional category of splenic lymphoma/leukemia unclassifiable, mainly represented by the hairy cell leukemia variant and splenic diffuse red pulp small B-cell lymphoma (SDRL). Until recently, histopathologic examination of splenectomy specimens was considered mandatory for the diagnosis of SMZL. However, nowadays, mainly because of advances in chemoimmunotherapy, splenectomy is performed much less frequently. We evaluated the diagnostic efficacy of bone marrow biopsy (BMB) histopathology in the diagnostic approach toward SMZL and SDRL and tested whether it may serve as a substitute for spleen histopathology in the differential diagnosis between these 2 entities. To this end, we conducted a paired assessment of BMB and spleen diagnostic samples from 46 cases with a diagnosis of SMZL (n=32) or SDRL (n=14) based on spleen histopathology. We demonstrate that detailed immunohistopathologic BMB evaluation offers adequate evidence for the confirmation of these entities and their differential diagnosis from other small B-cell lymphoma histotypes. Notably, the immunophenotypical profile of SMZL and SDRL was identical in both BMB and spleen specimens for 21 evaluated markers. Paired assessment of BMB and spleen specimens did not identify discriminating patterns of BMB infiltration, cytology, and/or immunohistology between SMZL and SDRL. Accordingly, bone marrow histopathology contributes significantly in confirming the diagnosis of SMZL and SDRL. However, presently it is not possible to distinguish SMZL from SDRL on the basis of BMB evaluation alone; hence, histopathologic examination of the spleen remains the "gold standard" approach.

  9. Obinutuzumab, Venetoclax, and Lenalidomide in Treating Patients With Relapsed or Refractory B-cell Non-Hodgkin Lymphoma

    Science.gov (United States)

    2017-03-01

    B-Cell Lymphoma, Unclassifiable, With Features Intermediate Between Diffuse Large B-Cell Lymphoma and Burkitt Lymphoma; Grade 1 Follicular Lymphoma; Grade 2 Follicular Lymphoma; Grade 3a Follicular Lymphoma; Recurrent Burkitt Lymphoma; Recurrent Diffuse Large B-Cell Lymphoma; Recurrent Follicular Lymphoma; Recurrent Marginal Zone Lymphoma; Refractory Burkitt Lymphoma; Refractory Diffuse Large B-Cell Lymphoma; Refractory Follicular Lymphoma; Transformed Recurrent Non-Hodgkin Lymphoma

  10. Comparison of Native Escherichia Coli L-Asparaginase Versus Pegylated Asparaginase, in Combination with Ifosfamide, Methotrexate, Etoposide, and Prednisolone (IMEP), in Extranodal NK/T Cell Lymphoma, Nasal Type (NTCL).

    Science.gov (United States)

    Kim, Hyun Jee; Ock, Chan-Young; Kim, Tae Min; Lee, Sung Hee; Lee, Ju-Yeun; Jung, Sun Hoi; Cho, Yoon Sook; Kim, Miso; Keam, Bhumsuk; Kim, Dong-Wan; Kim, Il Han; Heo, Dae Seog

    2017-07-03

    The aim of this study was to compare asparaginase-related toxicities in two asparaginase preparations, namely native Escherichia coli (E. coli) L-asparaginase (L-ASP) and pegylated asparaginase (PEG-ASP) in combination with ifosfamide, methotrexate, etoposide, and prednisolone (IMEP) in NK/T-cell lymphoma (NTCL). A total of 41 NTCL patients who received IMEP plus native E. coli L-ASP or PEG-ASP at Seoul National University Hospital were included in this study between January 2013 and March 2016. IMEP/ASP treatment consisted of ifosfamide, methotrexate, etoposide, plus native E. coli L-ASP (6000 IU/m2 on days 1, 3, 5, 7, 9, and 11) or PEG-ASP (2500 IU/m2 on day 1) every 3 weeks. ASP-related toxicities, toxicity patterns, length of hospital stay and clinical outcomes were compared between the different treatment groups. The frequency of ASP-related toxicities was similar between the IMEP plus native E. coli L-ASP group and the PEG-ASP group apart from hypofibrinogenemia (native E. coli L-ASP vs. PEG-ASP group, 86.4% vs. 36.8%, p=0.001). Although post-treatment transaminase and albumin levels were significantly high and low, respectively, hepatotoxicity gradients before and after treatment did not differ significantly between the groups. Since PEG-ASP was given at an outpatient clinic in some patients, length of hospital stay was significantly shorter in the IMEP plus PEG-ASP group (median 4.0 vs. 6.0 days, p=0.002). A favorable tendency of clinical outcomes was observed in NTCL patients treated with IMEP plus PEG-ASP (complete remission rate, 73.7% vs. 45.5%, p=0.067). IMEP plus PEG-ASP showed similar ASP-related toxicities, shorter length of hospital stay, and a trend towards improved clinical outcomes compared with IMEP plus native E. coli L-ASP in NTCL.

  11. Primary thymic mucosa-associated lymphoid tissue lymphoma with multiple thin walled lung cysts: case report and literature review

    Institute of Scientific and Technical Information of China (English)

    Lung-Yun Kang; Szu-Pei Ho; Yi-Pin Chou

    2013-01-01

    Mucosa-associated lymphoid tissue (MALT) lymphoma of the thymus is rare.We reported a case of a 37-year-old Chinese female with Sj(o)gren's syndrome and hyperglobulinemia.She suffered from chronic cough for 3 weeks.Chest computed tomography (CT) demonstrated a multiloculated cystic mass in mediastinum prevascular space and multiple lung cysts.Laboratory exam of autoimmune markers showed positive of antinuclear antibody (ANA),Sj(o)gren's syndrome A (SSA),Sj(o)gren's syndrome B (SSB),and rheumatoid factors (RF).Thymectomy with lymph node dissection was performed.The pathology report revealed thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue.Under immunohistochemical stains,CD20 and Bcl-2 were positive.No evidence of recurrence of disease was found.

  12. Immunohistochemical comparison of CD5, lambda, and kappa expression in primary and recurrent buccal mucosa-associated lymphoid tissue (MALT) lymphomas.

    Science.gov (United States)

    Tanaka, Toshiaki; Kitabatake, Kenichirou; Iino, Mituyoshi; Goto, Kaoru

    2011-09-06

    Mucosa-associated lymphoid tissue (MALT) lymphoma is a type of extranodal marginal zone B-cell lymphoma and is a distinct subtype of non-Hodgkin's lymphoma.Primary MALT lymphomas can also occur in the oral cavity, although their appearance in this location is rare. The neoplastic cells of which MALT lymphomas are composed express B-cell antigens and show monotypic immunoglobulin expression with light-chain restriction.Although neoplastic MALT lymphoma cells do not express CD5, previous studies have shown that CD5 positive MALT lymphomas are more prone to dissemination than those that do not express CD5. Moreover, there are some reports that describe kappa- and lambda- dual light chain expression in B cell malignant neoplasms.A 66-year-old Japanese woman with swelling of the right buccal mucosa was referred to our hospital. The lesion was excised and was pathologically diagnosed as a MALT lymphoma tumor with a t(11;18)(q21;q21) chromosome translocation.Swelling of the right buccal mucosa recurred 2 years later. The recurrent tumor was then excised and pathologically diagnosed as MALT lymphoma.Immunohistochemical examination of CD5, lambda, and kappa expressions revealed that the primary tumor was positive for CD5, kappa, and lambda, but the recurrent tumor was weakly positive for CD5 and kappa.With respect to lambda positivity, the recurrent tumor showed negativity.Our study suggests that immunohistochemical expression of CD5, kappa, and lambda in oral MALT lymphoma have the risk of recurrence.We first described the recurrence of CD5 positive MALT lymphoma in the oral cavity and compared the immunohistochemical expressions of CD5, lambda, and kappa between the primary and recurrent tumors.

  13. Primary lymphoma of the colon Linfoma primario de colon

    OpenAIRE

    Marta Pascual; Blanca Sánchez-González; Mar García; Miguel Pera; Luis Grande

    2013-01-01

    Background: primary colorectal lymphoma is a very rare disease, representing less than 0.5 % of all primary colorectal neoplasms. The gastrointestinal tract is the most frequently involved site of all extranodal lymphomas, the most common type of that is non-Hodgkin's lymphoma. Early diagnosis is often difficult because of unspecific symptoms. Therapeutic approaches have classically included radical resection, chemotherapy and radiotherapy. Materials and methods: we present our experience in ...

  14. Lymphoma of uterine cervix: magnetic resonance imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Kanaan, Daniel; Constantino, Carolina Pesce Lamas; Souza, Rodrigo Canellas de, E-mail: daniel.kanaan@hotmail.com [Department of Radiology, Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil); Parente, Daniella Braz [Instituto D' Or de Pesquisa e Ensino, Rio de Janeiro, RJ (Brazil)

    2012-05-15

    Lymphoma of the cervix is a rare disease. About 1.0% to 1.5% of extranodal lymphomas originates in the female genital tract. The clinical presentation of this condition is nonspecific and magnetic resonance imaging is important for diagnostic elucidation. The present report describes the case of a 80-year-old patient with lumbar pain, whose magnetic resonance imaging showed a large uterine mass. The final diagnosis was lymphoma. (author)

  15. Primary Hepatosplenic Large B-Cell Lymphoma

    Directory of Open Access Journals (Sweden)

    M.R. Morales-Polanco

    2008-03-01

    Full Text Available Diffuse large B-cell lymphoma is the most common form of lymphoma. It usually begins in the lymph nodes; up to 40% may have an extranodal presentation. According to a definition of primary extranodal lymphoma with presentation only in extranodal sites, there are reports of large B-cell lymphomas limited to liver or spleen as separate entities, and to date there have been only three documented cases of primary hepatosplenic presentation. This paper reports a fourth case. Due to a review of the literature and the clinical course of the case reported, we conclude that primary hepatosplenic large B-cell lymphoma has been found predominantly in females older than 60 years. The patients reported had <2 months of evolution prior to diagnosis, prominent B symptoms, splenomegaly in three and hepatomegaly in two, none with lymph node involvement. All had thrombocytopenia and abnormal liver function tests; three had anemia and elevated serum lactic dehydrogenase levels, two with hemophagocytosis in bone marrow. Because of the previously mentioned data, it can be stated that primary hepatosplenic lymphoma is an uncommon and aggressive form of disease that requires immediate recognition and treatment.

  16. Primary pediatric gastrointestinal lymphoma

    Directory of Open Access Journals (Sweden)

    Ranjana Bandyopadhyay

    2011-01-01

    Full Text Available Background: Primary non-Hodgkin′s lymphoma (NHL of the gastrointestinal (GI tract is the most common extranodal lymphoma in pediatric age group. Yet, the overall incidence is very low. The rarity of the disease as well as variable clinical presentation prevents early detection when the possibility of cure exists. Materials and Methods: We studied six cases of primary GI NHL in pediatric age group with reference to their clinical presentation, anatomic distribution and histopathologic characteristics. Results: All were males except one. Intestinal obstruction was the presenting feature in 50%. Half the cases showed ileocaecal involvement, while large bowel was involved in 16%. Histology showed four cases of diffuse large B-cell lymphoma (DLBCL, one case of Burkitt lymphoma, and one Burkitt-like lymphoma. Immunohistochemistry for Tdt, CD20, CD3, CD30, bcl2, bcl6 confirmed the morphological diagnosis. Conclusion: Pediatric GI lymphoma commonly involves the ileocaecal region and presents with intestinal obstruction. A higher prevalence of DLBCL is found compared to other series. A high proliferative index is useful in differentiating Burkitt-like lymphoma from DLBCL.

  17. Nasal-Type Extranodal Natural Killer/T-cell Neurolymphomatosis Confined to the Lumbar Nerve Roots: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jong Chun; Mun, Sung Hee; Lee, Young Hwan [Catholic University, Daegu (Korea, Republic of)

    2009-11-15

    Neurolymphomatosis refers to lymphoma that has infiltrated the peripheral nervous system and this is the least common clinical presentation of nervous system lymphoma. Most neurolymphomatosis is due to B-cell non-Hodgkin lymphoma, and most patients show lymphomatous infiltration in the meninges and brain parenchyma, in addition to peripheral nervous system involvement. We diagnosed a case of neurolymphomatosis that was confined to the right 4th and 5th lumbar nerve roots without involvement of the meninges or brain parenchyma in a patient with the nasal-type extranodal natural killer/T-cell lymphoma. We made this diagnosis based on the MRI and 18F-FDG PET-CT findings and the clinical manifestations.

  18. Outcome prediction by extranodal involvement, IPI, and R-IPI in the PET/CT and rituximab era

    DEFF Research Database (Denmark)

    El-Galaly, Tarec Christoffer; Villa, Diego; Alzahrani, Musa

    2015-01-01

    with an inferior PFS and OS. The IPI, R-IPI, and NCCN-IPI were predictive of PFS and OS, and the two latter could identify a very good prognostic subgroup with 3-year PFS and OS of 100%. CONCLUSIONS: PET/CT-ascertained extranodal involvement in DLBCL is common and involvement of >2 extranodal sites is associated......PURPOSE: 18F-fluorodeoxyglucose PET/CT (PET/CT) is the current state-of-the-art in the staging of diffuse large B-cell lymphoma (DLBCL) and has a high sensitivity for extranodal involvement. Therefore, reassessment of extranodal involvement and the current prognostic indices in the PET/CT era...... met the inclusion criteria. With a median follow-up of 2.4 years, the 3-year overall (OS) and progression-free survival (PFS) were 73% and 69%, respectively. The Ann Arbor classification had no prognostic impact in itself with the exception of stage IV disease (HR 2.14 for PFS, P

  19. Fluorescence in situ hybridization (FISH analysis of primary ocular adnexal MALT lymphoma

    Directory of Open Access Journals (Sweden)

    Harada Mine

    2006-10-01

    Full Text Available Abstract Background It remains unknown whether primary ocular adnexal extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma is a homogeneous entity, as there are few reports of the results of cytogenetic or molecular analyses of these tumors. Methods We performed interphase fluorescence in situ hybridization (FISH analysis to detect translocations and aneuploidy in 34 cases of primary ocular adnexal MALT lymphoma, and reviewed the histopathological findings. Correlations between the results of FISH analysis, the histopathological features and the clinical data were also analyzed. Results Among the 34 cases, FISH analysis revealed t(14;18(q32;q21 in one case, trisomy 3 in 21 cases (62%, and trisomy 18 in 16 cases (47%. The cases with trisomy 18 had significantly more prominent lymphoepithelial lesions (LELs and less nodularity in the tumors. In regard to the clinical correlations, tumors with trisomy 18 were observed predominantly in females and younger patients; also, in the majority of the cases, the tumor was of conjunctival origin. All the cases with recurrence showed trisomy 18 in the tumor. Conclusion Primary ocular adnexal MALT lymphoma is a significantly heterogeneous entity. Cases with trisomy 18 may have unique clinicopathological features.

  20. 4D-Listmode-PET-CT and 4D-CT for optimizing PTV margins in gastric lymphoma. Determination of intra- and interfractional gastric motion

    Energy Technology Data Exchange (ETDEWEB)

    Reinartz, Gabriele; Haverkamp, Uwe; Wullenkord, Ramona; Lehrich, Philipp; Kriz, Jan; Eich, Hans Theodor [University Hospital Muenster, Department of Radiation Oncology, Muenster (Germany); Buether, Florian [University of Muenster, European Institute for Molecular Imaging (EIMI), Muenster (Germany); Schaefers, Klaus [University of Muenster, European Institute for Molecular Imaging (EIMI), Muenster (Germany); DFG EXC 1003, Cluster of Excellence ' Cells in Motion' , Muenster (Germany); Schaefers, Michael [University of Muenster, European Institute for Molecular Imaging (EIMI), Muenster (Germany); University Hospital Muenster, Department of Nuclear Medicine, Muenster (Germany); DFG EXC 1003, Cluster of Excellence ' Cells in Motion' , Muenster (Germany)

    2016-05-15

    New imaging protocols for radiotherapy in localized gastric lymphoma were evaluated to optimize planning target volume (PTV) margin and determine intra-/interfractional variation of the stomach. Imaging of 6 patients was explored prospectively. Intensity-modulated radiotherapy (IMRT) planning was based on 4D/3D imaging of computed tomography (CT) and positron-emission tomography (PET)-CT. Static and motion gross tumor volume (sGTV and mGTV, respectively) were distinguished by defining GTV (empty stomach), clinical target volume (CTV = GTV + 5 mm margin), PTV (GTV + 10/15/20/25 mm margins) plus paraaortic lymph nodes and proximal duodenum. Overlap of 4D-Listmode-PET-based mCTV with 3D-CT-based PTV (increasing margins) and V95/D95 of mCTV were evaluated. Gastric shifts were determined using online cone-beam CT. Dose contribution to organs at risk was assessed. The 4D data demonstrate considerable intra-/interfractional variation of the stomach, especially along the vertical axis. Conventional 3D-CT planning utilizing advancing PTV margins of 10/15/20/25 mm resulted in rising dose coverage of mCTV (4D-Listmode-PET-Summation-CT) and rising D95 and V95 of mCTV. A PTV margin of 15 mm was adequate in 3 of 6 patients, a PTV margin of 20 mm was adequate in 4 of 6 patients, and a PTV margin of 25 mm was adequate in 5 of 6 patients. IMRT planning based on 4D-PET-CT/4D-CT together with online cone-beam CT is advisable to individualize the PTV margin and optimize target coverage in gastric lymphoma. (orig.) [German] Zur Optimierung des Sicherheitsabstandes beim Planungszielvolumen (PTV) und zur Erfassung der intra-/interfraktionellen Variation des Magens wurden neue Protokolle fuer die Bildverarbeitung in der Radiotherapie lokalisierter Magenlymphome evaluiert. Die Bildgebung von 6 Patienten wurde prospektiv untersucht. Die Planung der intensitaetsmodulierten Strahlentherapie (IMRT) basierte auf 4D-/3D-Bildgebung von Computertomographie (CT) und Positronenemissionstomographie

  1. Clinicopathologic features of gastrointestinal schwannomas coexisting extranodal marginal zone lympoma of mucosa associated lymphoid tissue lymphoma of rectum%直肠碰撞瘤临床病理观察

    Institute of Scientific and Technical Information of China (English)

    崔华娟; 王卓才; 赖日权; 彭大云; 陈敬文

    2013-01-01

    目的 提高对直肠碰撞瘤的认识.方法 对1例直肠胃肠神经鞘瘤与黏膜相关淋巴组织结外边缘区B细胞性淋巴瘤(MALT)形成的碰撞瘤,进行组织形态学观察及免疫组化检测,并复习相关文献.结果 患者女性,65岁.体检发现下腹部肿块.CT提示直肠右侧壁可见2.8 cm ×1.9 cm大小软组织影,邻近直肠管腔受压变窄.在直肠肿瘤切除标本中,同一肿物内可见胃肠神经鞘瘤和MALT淋巴瘤2种成分.2种肿瘤成分界限清楚,形成碰撞瘤,其中淋巴瘤成分包裹神经鞘瘤.神经鞘瘤S-100、PDG9.5、nestin和GFAP(-);淋巴瘤LCA、CD20和CD79α(+),CD3和CD45RO(-).基因重排显示B细胞单克隆性增生.IgH/MALT1 t(14;18) (q32;q21)及API2/MALT1 t(11;18) (q21;q21)染色体异位(-).c-kit及PDGFRA基因均为野生型.结论 直肠胃肠神经鞘瘤与淋巴瘤形成的碰撞瘤罕见.应与胃肠间质瘤及淋巴组织反应性增生鉴别.

  2. Bezafibrate and medroxyprogesterone acetate target resting and CD40L-stimulated primary marginal zone lymphoma and show promise in indolent B-cell non-Hodgkin lymphomas.

    Science.gov (United States)

    Hayden, Rachel E; Kussaibati, Racha; Cronin, Laura M; Pratt, Guy; Roberts, Claudia; Drayson, Mark T; Bunce, Christopher M

    2015-04-01

    B cell non-Hodgkin lymphomas (B-NHLs) are the most common adult hematological cancers and many remain incurable. Development of chemotherapy regimens is confounded by the prevalence of B-NHL in older, frailer patients and the chemo-protective tumor microenvironment. Although biological therapies such as rituximab have significantly improved outcomes and selective kinase inhibitors are showing promise, the rate of new drug discovery remains disappointing, the treatments expensive and long-term benefits uncertain. An alternative strategy is redeployment of available, inexpensive and non-toxic drugs. Here, we demonstrate the antiproliferative and mitochondrial superoxide (MSO) driven pro-apoptotic activities of bezafibrate (BEZ) and medroxyprogesterone acetate (MPA) against B-NHL cells, with a bias toward MZL, in the presence and absence of the microenvironmental signal CD40L. Our study is the first to confirm the presence of CD40L within the lymph node of B-NHL and its capacity to drive B-NHL proliferation. These findings implicate BEZ + MPA as a potential therapeutic strategy in B-NHL.

  3. Expression of human leukocyte antigens in diffuse large B cell lymphomas

    NARCIS (Netherlands)

    Riemersma, Sietske Annette

    2006-01-01

    Diffuse large B cell lymphoma is the most common type of non-Hodgkin lymphoma of which 40% present at extra-nodal sites including immune privileged sites such as the testis and the central nervous system (CNS). Loss of Human Leucocyte Antigen (HLA) expression has been described in many different

  4. Risk of second malignant neoplasms among long-term survivors of early stage extranodal nasal-type natural killer/T-cell lymphoma%早期结外鼻型NK/T细胞淋巴瘤长期生存患者第二原发肿瘤发病风险

    Institute of Scientific and Technical Information of China (English)

    陈波; 李晔雄; 王维虎; 金晶; 王淑莲; 刘跃平; 宋永文; 房辉; 任骅

    2015-01-01

    Objective To estimate risk and incidence of second malignant neoplasms (SMN) among long-term survivors of early stage extranodal nasal-type natural killer/T-cell lymphoma (NKTCL).Methods Between January 1983 and December 2007,174 patients with stage Ⅰ E and Ⅱ E NKTCL survived 3 or more years after treatment.Of them,50 patients were treated with radiotherapy alone,120 patients with combined modality therapy,and 4 patients with chemotherapy alone.The China 2010 population census data and Segi' s world population data were used for calculating the age-standardized cancer incidence rates.Results Median follow-up time was 8.3 years (3.1-35.6 years) for all patients.Nine (5.2 %) SMN were recorded.The median time to SMN was 12.6 years (0.9-18.5 years) from diagnosis of NKTCL.Seven patients had solid tumors,and 2 had other type of malignant lymphomas.The cumulative incidence rates at 5-year,10-year and 15-year were 1.2 %,2.4 % and 13.7 %,respectively.The crude incidence was 531.6/105 person-years,the age-standardized rates by Chinese standard population (ASR China) and world standard population (ASR world) were 294.5/105 and 243.7/105,respectively,and the cumulative incidence rate (0-74 years old) was 22.4 %.All of them were higher than the cancer incidence rates for general population in China in 2010.Conclusions A frequency of SMN in patients with NKTCL is higher than that expected in the general population.The patients have more risk for SMN during 10 to 15 years after diagnosis of NKTCL.Patients with long-term survival are at higher risk of SMN and should be carefully followed-up.%目的 分析早期结外鼻型NK/T细胞淋巴瘤(NKTCL)经治疗后生存超过3年患者第二原发恶性肿瘤(SMN)的发病风险.方法 1983年1月至2007年12月共174例ⅠE~ⅡE期NKTCL患者,经治疗后生存超过3年,其中50例接受单纯放疗,120例接受综合治疗,4例接受单纯化疗.采用中国2000年人口年龄构成比和国际通用

  5. Hodgkin's Lymphoma

    Science.gov (United States)

    ... behavior. Your type determines your treatment options. Classical Hodgkin's lymphoma Classical Hodgkin's lymphoma is the more common ... Hodgkin's lymphoma Lymphocyte-rich Hodgkin's lymphoma Lymphocyte-predominant Hodgkin's lymphoma This much rarer type of Hodgkin's lymphoma ...

  6. Cutaneous natural killer/T-cell lymphoma.

    Science.gov (United States)

    Radonich, Michael A; Lazova, Rossitza; Bolognia, Jean

    2002-03-01

    Lymphomas are classified as either Hodgkin's or non-Hodgkin's. The 2 subtypes of non-Hodgkin's lymphoma that can present primarily in the skin are cutaneous T-cell lymphoma and cutaneous B-cell lymphoma, both of which tend to be low-grade malignant neoplasms. Recently another distinct subtype of lymphoma was discovered, the natural killer (NK)/T-cell lymphoma, which can involve the skin in a primary or secondary fashion. The NK/T-cell subtype of lymphoma is characterized by the expression of the NK-cell antigen CD56. These CD56(+) lymphomas are further subdivided into nasal NK/T-cell lymphomas that commonly present as midfacial destructive disease and non-nasal NK/T-cell lymphomas that often arise in extranodal locations, including the skin. We report a case of aggressive NK-cell leukemia/lymphoma with numerous secondary cutaneous lesions and review the clinical and histopathologic spectrum of non-nasal CD56(+) lymphomas, with an emphasis on the dermatologic findings.

  7. A Case of Successful Remission of Extensive Primary Gastric Diffuse Large B Cell Lymphoma: Radiologic, Endoscopic and Pathologic Evidence

    Directory of Open Access Journals (Sweden)

    Mike M. Bismar

    2014-04-01

    Full Text Available Though rare amongst stomach neoplasms, primary gastric diffuse large B cell lymphoma is one of the commonest extranodal non-Hodgkin lymphomas. If left untreated, it can have a devastating progression and life-threatening consequences. We present the case of a successfully treated large antral ulcer confirmed to be large B cell lymphoma as evidenced by radiologic, endoscopic and histopathologic findings. A brief discussion about the types of gastric lymphoma, their Helicobacter pylori relation and therapeutic modalities follows.

  8. Yttrium Y 90 Ibritumomab Tiuxetan, Fludarabine, Radiation Therapy, and Donor Stem Cell Transplant in Treating Patients With Relapsed or Refractory Non-Hodgkin's Lymphoma

    Science.gov (United States)

    2016-03-21

    B-cell Chronic Lymphocytic Leukemia; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma; Waldenström Macroglobulinemia

  9. Primary gastrointestinal non-Hodgkin lymphoma in adults: clinicopathologic and survival characteristics.

    Science.gov (United States)

    Radić-Kristo, Delfa; Planinc-Peraica, Ana; Ostojić, Slobodanka; Vrhovac, Radovan; Kardum-Skelin, Ika; Jaksić, Branimir

    2010-06-01

    Primary non-Hodgkin lymphomas of gastrointestinal tract (PGI-NHL) are the most common extranodal lymphomas with an increasing incidence. The incidence, clinicopathologic characteristics, treatment and survival were assessed in 39 successive, newly diagnosed PGI-NHL patients (23 male and 16 female) treated at "Merkur" University Hospital. The aim of the study was to precisely evaluate their characteristics and compare them with the results reported from other similar studies. The most common site of PGI-NHL was stomach (n = 29, 74%), followed by small intestine (n = 5, 13%), and colon and rectosigmoid (n = 5, 13%). According to the Ann Arbor classification, 34 (87%) patients had stage IE and IIE, and five patients (12%) stage IIIE and IVE. According to World Health Organization (WHO) classification, 29 (87%) patients had diffuse large B-cell lymphoma (DLCBL), two had mantle cell lymphoma, and seven (18%) had marginal zone B-cell lymphoma-mucosa associated tissue (MALT). Twenty-six (66%) patients underwent surgical resection followed by chemotherapy, ten (26%) were treated with chemotherapy alone, and three (8%) were treated surgically. Complete remission was achieved in 28 (72%) and partial remission in seven (18%) patients. Four (10%) patients had progressive disease. In our patients, the major prognostic factor for outcome was the stage of disease. Patients with localized lymphoma (stage IE and IIE) had a significantly longer overall survival: 85% at five years and 65% at ten years. Patients with extended disease (stage IIIE and IVE) had overall survival less than 33%. The prognostic power of erythrocyte sedimentation rate (ESR), total protein, serum albumin, LDH concentration and activity was analyzed. Of these parameters, only LDH had a statistically significant effect on overall survival. In conclusion, our patient group was comparable to other literature reports on PGI-NHL patients according to clinicopathologic characteristics. Disease stage and LDH were the

  10. Benign lymphoepithelial lesion,lymphoepithelial carcinoma,MALT lymphoma and their association%良性淋巴上皮病、淋巴上皮癌、MALT淋巴瘤的关系

    Institute of Scientific and Technical Information of China (English)

    李江

    2007-01-01

    良性淋巴上皮病(benign lymphoepithelial lesion)、淋巴上皮癌(lymphoepithelial carcinoma)、黏膜相关淋巴组织型结外边缘区B细胞淋巴瘤(MALT淋巴瘤)(extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoidtissue,MALT lymphoma)是3种性质不同但又存在某些关联的病变,本文对3种病变的病因、组织病理学表现及三者之间的关系进行了介绍,并结合叶为民等论文中的一些内容进行了简要探讨.

  11. Primary central nervous system lymphoma in an immunocompetent patient

    OpenAIRE

    Málaga-Zenteno, José; Médico Asistente, Servicio de Hematología, Hospital Nacional Carlos Alberto Seguín Escobedo, EsSalud, Arequipa, Perú.; Mamani-Quispe, Jersson Alonso; Estudiante de Medicina Humana, Centro de Investigación y Estudios Médicos (CIEM), Universidad Católica Santa María, Arequipa, Perú. Sociedad Científica Médico Estudiantil Peruana (SOCIMEP).; Fuentes Fuentes, Mariela; Médico Asistente, Servicio de Hematología, Hospital Nacional Carlos Alberto Seguín Escobedo, EsSalud, Arequipa, Perú.; Suclla-Velásquez, José Alonso; Estudiante de Medicina Humana, Centro de Investigación y Estudios Médicos (CIEM), Universidad Católica Santa María, Arequipa, Perú. Sociedad Científica Médico Estudiantil Peruana (SOCIMEP).; Meza Aragón, Julio; Médico Asistente, Servicio de Neurocirugía, Hospital Nacional Carlos Alberto Seguín Escobedo, EsSalud, Arequipa, Perú.

    2012-01-01

    Primary central nervous system lymphoma (PCNSL) constitutes 2% of extranodal lymphomas and 0,3%-1,5% of all intracranial neoplasms in immunocompetent patients, being more frequent after the sixth decade of life. We report a case of a 76 year-old man with no antecedents who started his disease with march instability, difficulty to move left side of his body with brachial predominance, holocraneal headache and dizziness. He arrived at emergency with Glasgow 14 and right eyelid ptosis. He had le...

  12. Ill-fitting dentures as primary presentation of mantle cell lymphoma: A case report and literature review of the primary mantle cell lymphomas of the hard palate

    Directory of Open Access Journals (Sweden)

    Ömür Dereci

    2015-01-01

    Full Text Available Mantle cell lymphoma (MCL is a subtype of B-cell non-Hodgkin′s lymphoma seen predominantly in males. Common extra-nodal sites of involvement of MCL are Waldeyer′s ring, gastrointestinal tract, bone marrow and peripheral blood. The extra-nodal palatal localization of MCL is quite uncommon. MCL is seen in predominantly older patients, therefore undiagnosed MCL patients are likely to have total prosthesis. In this study, a case of MCL, initially presenting as palatal swelling was reported with relevant literature review and the possible role of dental professionals in the diagnosis of this rare entity was discussed.

  13. Primary Hepatic Lymphoma: A Challenging Diagnosis

    Directory of Open Access Journals (Sweden)

    D. Myoteri

    2014-01-01

    Full Text Available Introduction. Primary hepatic lymphoma is an unusual malignancy and is very difficult to diagnose promptly. An intrigue case presenting with cholestatic jaundice is reviewed and main disease characteristics are further discussed. Case Report. A 70-year-old male presented with dull right upper quadrant abdominal pain and mild cholestatic jaundice. Initial evaluation revealed mildly elevated liver function tests and normal tumor markers, while imaging with an abdominal CT-scan showed multiple hypodense nodules in both liver lobes. First impression of metastatic deposits from gastrointestinal origin was not confirmed by endoscopic means. After CT-guided biopsy, primary diffuse large B-cells non-Hodgkin lymphoma was revealed. Appropriate chemotherapy improved patient’s condition markedly. Discussion. Primary hepatic lymphoma is a rare form of extranodal lymphomas, accounting for less than 1% of all extranodal lymphomas in general. In order to define the condition as PHL, liver has to be the only site of lymphoma occurrence or to be involved in a major degree with minimal nonliver disease. Most PHLs are of B-cell origin with large cells as the main cell type.

  14. Centrofacial angiocentric lymphoma.

    Science.gov (United States)

    Peral-Cagigal, Beatriz; Galdeano-Arenas, María; Crespo-Pinilla, Juan Ignacio; García-Cantera, José Miguel; Sánchez-Cuéllar, Luis Antonio; Verrier-Hernández, Alberto

    2005-01-01

    The centrofacial angiocentric lymphoma is a rare lymphoid neoplasm, with an often-difficult diagnosis due to the non-specific clinical picture. On many occasions it is necessary to perform various biopsies to reach the correct diagnosis. This lymphoma is an aggressive Non-Hodgkin's (NHL) type, which is normally found in the upper respiratory tract (predominantly in the nasal cavity), and has an ominous prognosis, as the average survival rate is between 12 and 18 months (1). It is predominantly found in subjects of oriental and South American extraction, who are between the ages of 50 and 60 years and with a slight tendency towards males (2:1). This is the case study of a female Ecuadorian patient who was referred to our department with a hemifacial edema, chocolate- like rhinorrhea and nasal respiratory obstruction, which had been treated with antibiotics and anti-inflammatories for a month without success. After performing a number of diagnostic tests, it was found histologically that the patient had an extranodal T-cell lymphoma of the nasal type (also known as T-cell angiocentric lymphoma).

  15. NON HODGKIN'S LYMPHOMA OF STOMACH WITH SPONTANEOUS PERFORATION: A CASE REPORT WITH REVIEW OF LITERATURE

    Directory of Open Access Journals (Sweden)

    Rishikant

    2014-01-01

    Full Text Available Stomach is the commonest extranodal site of lymphomas. A case of Non - Hodgkin’s Lymphoma of stomach is reported here for its unusual way of presentation. Fifty year old male presented with features of perforation peritonitis , which on exploration turned out to be a Non - Hodgkin’s lymphoma of stomach with perforation. Usually gastric lymphomas are diagnosed on upper gastrointestinal endoscopic examination and biopsy in patients with epigastric pain , early satiety and weight loss. Detailed review of literature denotes that spontaneous perforation in a case of lymphoma of the gastrointestinal tract is uncommon , although many cases of perforation after chemo or radiotherapy are reported.

  16. Primary endotracheal non-Hodgkin's lymphoma in a Chinese woman: a case report

    Institute of Scientific and Technical Information of China (English)

    ZHANG Wei-dong; LI Shi-yue; OUYANG Ming; ZHONG Nan-shan

    2005-01-01

    @@ Most patients with non-Hodgkin's lymphoma (NHL) present with peripheral lymph node enlargement, with or without systemic symptoms. NHL -05-also involve mediastinal, intra-abdominal and pelvic lymph nodes with resulting symptoms. They -05-involve only an extranodal site, such as part of the gastrointestinal tract, lung, brain or testis. Extranodal presentation is more common in NHL than in Hodgkin's disease. Primary endotracheobronchial involvement in non-Hodgkin's lymphoma is a rare presentation. From 1989 to the present, only 3 cases of primary tracheal NHL were reported in Medline.

  17. Linfomas de la órbita y anexos oculares: Correlación clínico patológica de 25 casos Orbital and ocular adnexal lymphomas: Clinico-pathological correlation in 25 cases

    Directory of Open Access Journals (Sweden)

    Erica A. Rojas Bilbao

    2010-08-01

    Full Text Available Se evaluaron las características clínicas, histológicas y la evolución de una cohorte de pacientes con linfomas de la órbita y anexos oculares. Entre 1995 y 2008 se estudiaron 25 casos de linfomas de la órbita y anexos oculares en un centro oncológico de referencia. En cada caso se analizó el inmunofenotipo usando un panel de anticuerpos monoclonales (CD45, CD20, CD3, CD5, CD23, BCL2, BCL6, BCL10, Ki67, CD30, CD15, BCL1, Kappa, Lambda, CD138. Las lesiones fueron evaluadas utilizando el sistema de clasificación de linfomas (OMS, 2008. Se analizaron 23 linfomas primarios y dos secundarios. Los subtipos histológicos fueron: 16 linfomas B de la zona marginal asociados a las mucosas (MALT, cuatro linfomas difusos de células grandes B, dos linfomas foliculares y un paciente con linfoma Hodgkin. De los 25 casos estudiados, 22 presentaron estadios localizados. El linfoma MALT fue el subtipo más frecuente. En este estudio se observó enfermedad localizada en la mayoría de los casos y con baja progresión a distancia.Clinical, histological features and outcome of a cohort of patients with orbital and adnexal lymphoproliferative tumors were evaluated. Twenty-five cases in an oncologic referral center from 1995 to 2008, were included in the study. Each case had detailed immunophenotypic analysis using a panel of monoclonal antibodies (CD45, CD20, CD3, CD5, CD23, BCL2, BCL6, BCL10, Ki67, CD30, CD15, BCL1, Kappa, Lambda, CD138. Lesions were classified by using WHO (2008 lymphomas classification. Twenty-three patients were found to have primary and two secondary lymphomas. Histological subtypes were: 16 patients with marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma, four diffuse large B cell lymphomas, two mantle cell lymphomas, two follicular lymphomas, and one Hodgkin lymphoma. Among the 25 patients studied, 22 had localized stage. Extranodal marginal zone lymphoma was the most frequent type of primary orbital

  18. Distribution and ZAP-70 expression of WHO lymphoma categories in Shanxi, China: a review of 447 cases using a tissue microarray technique.

    Science.gov (United States)

    Wang, Jinfen; Young, Lillian; Win, William; Taylor, Clive R

    2005-12-01

    This study aims to assess the distribution of lymphoma subtypes in Shanxi, China, according to the World Health Organization (WHO) classification, and to compare the relative distribution with other areas of the world. H&E-stained tissue sections from the archives of the Shanxi Tumor Hospital, China, were reviewed and 447 cases with sufficient materials were selected for detailed study. A panel of antibodies and probes was assembled, including antibodies to ALK1, bcl-6, CDs 1alpha, 3, 4, 5, 7, 8, 10, 15, 20, 23, 30, 43, 56, 68, 79alpha, and 99, cyclin D1, EMA, kappa, lambda, LMP1, PAX5, TdT, Vs38C and ZAP70, plus EBER RNA probe by in situ hybridization. The 447 lymphoma cases, subtyped according to the WHO classification, were assembled in triplicate into 11 tissue microarrays and examined with the panel of markers described. Among the 447 cases, 385 (82.6%) were confirmed to be non-Hodgkin lymphomas (NHL) and 62 (13.9%) were Hodgkin lymphomas of classic type (CHL). Of the NHL cases, 68.6% were B-cell lymphomas and 30.6% T/NK-cell lymphomas. Histiocytic neoplasms accounted for only three cases (0.8%). Diffuse large B-cell lymphomas (DLBCL) were the most common subtype (35.1%), followed by peripheral T-cell lymphomas unspecified (PTun, 12.0%), extranodal marginal zone B-cell lymphomas (MALT lymphomas, 11.7%), follicular lymphomas (FL, 8.6%), T-lymphoblastic lymphomas (T-LBL, 7.0%), anaplastic large cell lymphomas (ALCL, 4.2%), B small lymphocytic lymphomas (B SLL, 3.6%), and mantle cell lymphomas (MCL, 2.6%). Of 263 B-cell neoplasms, 105 (39.9%) expressed immunoglobulin light chain, including 52 kappa and 53 lambda, detectable in paraffin sections. The incidence of DLBCL was similar to many Western countries and Asia. The frequency of FL was, however, much lower than the usual pattern in Western countries, although NK/T-cell lymphomas were more common (30.6%), similar to other countries in Asia, including Japan and Korea. With regard to markers of EBV infection, 8

  19. Clinicopathologic features of lymphoma: analyses of 664 cases%664例淋巴瘤临床病理分析

    Institute of Scientific and Technical Information of China (English)

    宋琳; 邢晓明; 冉雯雯; 李宏; 邵玉铭; 李玉军

    2013-01-01

    目的 根据WHO(2008版)造血和淋巴肿瘤分类标准,探讨青岛地区淋巴瘤的病理类型及分布特点.方法 收集2008年8月~2011年8月青岛大学医学院附属医院诊治664例淋巴瘤,复习其临床资料、HE及免疫组化切片.按WHO(2008版)分类新标准进行病理诊断及分类.结果 664例淋巴瘤中,非霍奇金淋巴瘤(non-Hodgkin's lymphoma,NHL)621例(93.52%),霍奇金淋巴瘤(Hodgkin's lymphoma,HL)43例(6.48%).NHL中B细胞淋巴瘤485例(78.10%),T和NK细胞淋巴瘤136例(21.90%).NHL中,发病构成比居前5位分别为弥漫性大B细胞淋巴瘤-非特殊类型(diffuse large B-cell lymphoma-unspecified,DLBCL-NOS)277例(44.61%)、黏膜相关淋巴组织结外边缘区淋巴瘤(extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type,MALT)48例(7.73%)、结外NK/T细胞淋巴瘤45例(7.25%)、外周T细胞淋巴瘤-非特殊类型(peripheral T-cell lymphoma-unspecified,PTCL-NOS)33例(5.31%)、慢性淋巴细胞性白血病/小淋巴细胞淋巴瘤(chronic lymphocytic leukemia/small lymphocytic lymphoma,CLL/SLL)32例(5.15%).HL中以结节硬化型经典型霍奇金淋巴瘤(nodular sclerosis classical Hodgkin's lymphoma,NSCHL)为多(18例,41.86%).NHL和HL患者中,男女比例为1.52:1,平均年龄53岁(2~95岁).NHL(115例,18.52%)和HL(26例,60.47%)起病部位均以颈部淋巴结为多.结论 664例淋巴瘤中,NHL发病远多于HL,NHL中以DLBCL-NOS多见,HL中以NSCHL多见.大部分淋巴瘤类型以男性发病为多,起病部位以颈部淋巴结为多.%Purpose To study the pathologic types and characteristics of lymphoma based on the Word Health Organization classification of tumors of hematopoietic and lymphoid tissues ( 2008 ) in Qingdao area.Methods Clinical data of lymphoma from August 2008 to August 2011 in the affiliated hospital of Qingdao University Medical College were collected and reviewed, including morphological, immunological and clinical characteristics.According to

  20. Development of primary central nervous system lymphoma in a systemic lupus erythematosus patient after treatment with mycophenolate mofetil and review of the literature.

    Science.gov (United States)

    Balci, M A; Pamuk, G E; Unlu, E; Usta, U; Pamuk, O N

    2017-01-01

    Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma and four cases of PCNSL have previously been described in association with mycophenolate mofetil. We report the fifth case of PCNSL in a patient with lupus nephropathy while on mycophenolate mofetil treatment.

  1. CLINCOPATHOLOGIC STUDY OF LYMPHOMA: a relook

    Directory of Open Access Journals (Sweden)

    Malathi

    2014-10-01

    Full Text Available INTRODUCTION: Lymphomas are heterogeneous group of malignant lympho-proliferative disorders. Broadly categorized into Non-Hodgkin’s lymphoma [NHLs] and Hodgkin’s lymphoma [HL]. Decades back one of the challenging topics in morphologic pathology was accurate diagnosis and classification of lymphoma. Studies on lymphoma with clinicopathologic correlation were found to be much significant. STUDY OBJECTIVES: This was a retrospective study aimed to describe lymphomas on histo morphology and thus classify NHLs using working formulation for clinical usage (1982 and HL using Rye (1966 classification respectively. To attempt clinicopathologic correlation. METHODS: The study was done in the department of pathology, Mahadevappa Rampure Medical College, Gulbarga during the period 1989-1999. Formalin fixed paraffin wax embedded tissue blocks previously diagnosed as lymphomas in the department were used. Morphologic details by light microscopy on haematoxylin and eosin (H&E stained sections were noted. Clinical history of each case were analysed from hospital records. Clinical and pathologic correlation was done. Special stains Reticulin (Gomori and Periodic acid Schiff’s stain [PAS] was done in relevant cases. RESULTS: Study of total 102 cases of lymphoma it was observed NHLs formed 70 cases with an incidence of 68.3%. HL accounted for 32 cases with incidence of 37.2%. NHLs commonly presented in fifth decade 24.2%, followed by fourth and sixth decade. Sex distribution showed Male: Female ratio as 2.5:1. Majority of cases presented with cervical and axillary lymphadenopathy at 41% and 20% respectively. The most frequent grade was clinically aggressive intermediate grade NHLs seen in 80% of all cases. 20 cases of NHLs were of extranodal in origin. The most common site was gastrointestinal tract (60% and head and neck region with (30%. The major histologic type in both nodal and extranodal NHLs was diffuse small cleaved cell type (DSCC. HL showed sex

  2. Cellular Immunotherapy Following Chemotherapy in Treating Patients With Recurrent Non-Hodgkin Lymphomas, Chronic Lymphocytic Leukemia or B-Cell Prolymphocytic Leukemia

    Science.gov (United States)

    2016-07-29

    Post-transplant Lymphoproliferative Disorder; B-Cell Prolymphocytic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; B-Cell Lymphoma, Unclassifiable, With Features Intermediate Between Diffuse Large B-Cell Lymphoma and Burkitt Lymphoma; B-Cell Lymphoma, Unclassifiable, With Features Intermediate Between Diffuse Large B-Cell Lymphoma and Classical Hodgkin Lymphoma; Recurrent Lymphoplasmacytic Lymphoma

  3. T-Cell Lymphomas in South America and Europe

    Directory of Open Access Journals (Sweden)

    Monica Bellei

    2012-01-01

    Full Text Available Peripheral T-cell lymphomas are a group of rare neoplasms originating from clonal proliferation of mature post-thymic lymphocytes with different entities having specific biological characteristics and clinical features. As natural killer cells are closely related to T-cells, natural killer-cell lymphomas are also part of the group. The current World Health Organization classification recognizes four categories of T/natural killer-cell lymphomas with respect to their presentation: disseminated (leukemic, nodal, extranodal and cutaneous. Geographic variations in the distribution of these diseases are well documented: nodal subtypes are more frequent in Europe and North America, while extranodal forms, including natural killer-cell lymphomas, occur almost exclusively in Asia and South America. On the whole, T-cell lymphomas are more common in Asia than in western countries, usually affect adults, with a higher tendency in men, and, excluding a few subtypes, usually have an aggressive course and poor prognosis. Apart from anaplastic lymphoma kinase-positive anaplastic large cell lymphoma, that have a good outcome, other nodal and extranodal forms have a 5-year overall survival of about 30%. According to the principal prognostic indexes, the majority of patients are allocated to the unfavorable subset. In the past, the rarity of these diseases prevented progress in the understanding of their biology and improvements in the efficaciousness of therapy. Recently, international projects devoted to these diseases created networks promoting investigations on T-cell lymphomas. These projects are the basis of forthcoming cooperative, large scale trials to detail biologic characteristics of each sub-entity and to possibly individuate targets for new therapies.

  4. Does Delayed-Time-Point Imaging Improve 18F-FDG-PET in Patients With MALT Lymphoma?

    Science.gov (United States)

    Mayerhoefer, Marius E.; Giraudo, Chiara; Senn, Daniela; Hartenbach, Markus; Weber, Michael; Rausch, Ivo; Kiesewetter, Barbara; Herold, Christian J.; Hacker, Marcus; Pones, Matthias; Simonitsch-Klupp, Ingrid; Müllauer, Leonhard; Dolak, Werner; Lukas, Julius; Raderer, Markus

    2016-01-01

    Purpose To determine whether in patients with extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue lymphoma (MALT), delayed–time-point 2-18F-fluoro-2-deoxy-d-glucose-positron emission tomography (18F-FDG-PET) performs better than standard–time-point 18F-FDG-PET. Materials and Methods Patients with untreated histologically verified MALT lymphoma, who were undergoing pretherapeutic 18F-FDG-PET/computed tomography (CT) and consecutive 18F-FDG-PET/magnetic resonance imaging (MRI), using a single 18F-FDG injection, in the course of a larger-scale prospective trial, were included. Region-based sensitivity and specificity, and patient-based sensitivity of the respective 18F-FDG-PET scans at time points 1 (45–60 minutes after tracer injection, TP1) and 2 (100–150 minutes after tracer injection, TP2), relative to the reference standard, were calculated. Lesion-to-liver and lesion-to-blood SUVmax (maximum standardized uptake values) ratios were also assessed. Results 18F-FDG-PET at TP1 was true positive in 15 o f 23 involved regions, and 18F-FDG-PET at TP2 was true-positive in 20 of 23 involved regions; no false-positive regions were noted. Accordingly, region-based sensitivities and specificities were 65.2% (confidence interval [CI], 45.73%–84.67%) and 100% (CI, 100%-100%) for 18F-FDG-PET at TP1; and 87.0% (CI, 73.26%–100%) and 100% (CI, 100%-100%) for 18F-FDG-PET at TP2, respectively. FDG-PET at TP1 detected lymphoma in at least one nodal or extranodal region in 7 of 13 patients, and 18F-FDG-PET at TP2 in 10 of 13 patients; accordingly, patient-based sensitivity was 53.8% (CI, 26.7%–80.9%) for 18F-FDG-PET at TP1, and 76.9% (CI, 54.0%–99.8%) for 18F-FDG-PET at TP2. Lesion-to-liver and lesion-to-blood maximum standardized uptake value ratios were significantly lower at TP1 (ratios, 1.05 ± 0.40 and 1.52 ± 0.62) than at TP2 (ratios, 1.67 ± 0.74 and 2.56 ± 1.10; P = 0.003 and P = 0.001). Conclusions Delayed–time-point imaging

  5. A Case of p63 Positive Diffuse Large B Cell Lymphoma of the Bladder

    Science.gov (United States)

    Jones, Carol

    2016-01-01

    Diffuse large B cell lymphoma (DLBCL), currently the most common type of non-Hodgkin lymphoma (NHL), is an aggressive B cell neoplasm that typically presents in older adults as a rapidly enlarging mass. The enlarging mass typically represents a lymph node, although extranodal disease can occur in a significant percentage (40%) of cases. The most common extranodal sites of involvement include the gastrointestinal tract and skin; primary bladder lymphoma represents only 0.2% of extranodal non-Hodgkin lymphomas. We report a case of diffuse large B cell lymphoma occurring in the bladder of an 83-year-old gentleman with an initial presentation of hematuria. This neoplasm displayed large, atypical cells with vesicular chromatin and prominent nucleoli that involved the bladder mucosa with invasion into muscularis propria, prostate, and urethra. Positive staining for p63 initially raised suspicion for poorly differentiated urothelial carcinoma; however, lack of staining for pancytokeratin and positive staining for LCA, CD20, CD79a, and PAX-5 confirmed the diagnosis of diffuse large B cell lymphoma. Though it does not occur in all cases, p63 can be positive in a significant percentage of cases of DLBCL; therefore, a diagnosis of lymphoma remains an important entity on the differential diagnosis of aggressive and particularly poorly differentiated neoplasms. PMID:27648316

  6. CT diagnosis of 52 patients with lymphoma in abdominal lymph nodes

    Institute of Scientific and Technical Information of China (English)

    Ri-Sheng Yu; Wei-Min Zhang; Yi-Qing Liu

    2006-01-01

    AIM: To assess CT manifestations and its diagnostic value for lymphoma in the abdominal lymph nodes (LALN).METHODS: CT findings in 52 cases of LALN proved by surgery or biopsy, including Hodgkin's disease (HD) in 16 cases and non-Hodgkin's lymphoma (NHL) in 36 cases,were retrospectively analyzed.RESULTS: (1) CT manifestations based on distribution of the lesions of LALN: Solitary mass type was found in 10 cases, including solitary, round, uniform-density,enlarged lymph nodes in 3 cases; and multiple, enlarged lymph nodes fusing into singular lobular mass in 7cases. Thirty-four cases of multiple-nodular type showed multiple, round, enlarged lymph nodes with uniform density and clear margins. Vessels-embedded signs,including mesenteric vessels, renal vessels, abdominal aorta or inferior vena cava, were seen in 6 cases, and duodenum-embedded signs were seen in 2 cases. Eight cases of diffuse type showed characteristic "cobblestone signs". (2) CT manifestations correlated with pathological type: CT manifestations of 12 cases of HD were different from those of 40 cases of NHL in distribution, size,quantity and fused lesion of enlarged lymph nodes. (3)Twenty-eight cases of 52 patients were accompanied with extra-nodal lymphoma in the abdomen, especially gastrointestinal lymphoma, which had characteristic CT findings. (4) In follow-up examinations, CT images showed uniform, heterogeneous or rim enhancement in 15 cases, and occasional calcifications accompanied with reduction of the lesion size and quantity in 12 cases, whereas the lesions disappeared in 3 cases after treatment.CONCLUSION: CT images show many characteristic manifestations valuable for qualitative diagnosis of LALN,and it is also helpful for pathological classification of LALN and therapeutic evaluation in follow-up of patients.

  7. Primary Gallbladder Lymphoma in a Male Patient with No Risk Factors Detected Incidentally by CT Colonography

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    Monil Karia

    2015-01-01

    Full Text Available Primary gallbladder lymphoma, although rare, usually presents in females with symptoms mimicking cholecystitis. We present a rare case of primary gallbladder in an 81-year-old male with no risk factors whose only symptom was weight loss. Routine blood tests including liver function tests were unremarkable. A CT colonography was carried out to exclude colonic malignancy. Unilateral gallbladder wall thickening and lymphadenopathy were incidentally detected and confirmed by ultrasound and a decision for the patient to undergo laparoscopic cholecystectomy and intraoperative cholangiogram was made. Histology confirmed extranodal marginal zone lymphoma with follow-up staging and biopsy of the bone marrow not demonstrating spread. Cholecystectomy was therefore deemed curative and no adjuvant therapy was necessary. Thickening of the gallbladder wall on any imaging with or without symptoms should not be ignored or assumed to be cholecystitis, even in males with no risk factors. In these patients urgent cholecystectomy with intraoperative cholangiogram is indicated with histology and haematology follow-up.

  8. Cyclin D3 expression in non-Hodgkin lymphoma. Correlation with other cell cycle regulators and clinical features

    DEFF Research Database (Denmark)

    Møller, Michael Boe; Nielsen, O; Pedersen, Niels Tinggaard

    2001-01-01

    analyzed immunohistochemically for cyclin D3 expression. In 43 lymphomas (21.7%), cyclin D3 was overexpressed. T-cell lymphomas more frequently overexpressed cyclin D3 than B-cell lymphomas. Furthermore, cyclin D3-overexpressing indolent lymphomas were associated with higher proliferation rate, higher p21......Waf1 expression, lower p27Kip1 expression, and altered p53. Cyclin D3 overexpression identified a subgroup of patients with indolent B-cell lymphoma with adverse clinical features: patients were older, more frequently had "B" symptoms and extranodal involvement, and were more frequently in the high...

  9. Prognostic factors in non-Hodgkin lymphomas

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    Karin Zattar Cecyn

    2000-01-01

    Full Text Available CONTEXT: In Hodgkin's disease, each clinical or pathologic stage can be related to the extent of the area involved and predicts the next anatomical region at risk for tumor dissemination. OBJECTIVE: To determine the best prognostic factors that could predict survival in non-Hodgkin lymphoma cases. DESIGN: A retrospective study. LOCATION: Department of Hematology and Transfusion Medicine, Universidade Federal de São Paulo - Escola Paulista de Medicina. PARTICIPANTS: 142 patients with non-Hodgkin lymphoma diagnosed between February 1988 and March 1993. MAIN MEASUREMENTS: Histological subset, Sex, Age, Race, B symptoms, Performance status, Stage, Extranodal disease, Bulk disease, Mediastinal disease, CNS involvement, BM infiltration, Level of DHL, Immunophenotype. RESULTS: In the first study (113 patients, the following variables had a worse influence on survival: yellow race (P<0.1; ECOG II, III e IV (P<0.1 and extranodal disease (P<0.1 for high grade lymphomas; constitutional symptoms (P<0.1, ECOG II, III e IV (P<0.1 and involvement of CNS (P<0.1 for intermediate grade and the subtype lymphoplasmocytoid (P=0.0186 for low grade lymphomas. In the second survey (93 patients, when treatment was included, the variables related to NHL survival were: CNS involvement (P<0.1 for high grade lymphomas, constitutional symptoms (P<0.1, ECOG II, III, IV (P=0.0185 and also CNS involvement (P<0.1 for the intermediate group. There were no variables related to the survival for low-grade lymphomas. CONCLUSIONS: The intermediate grade lymphomas were more compatible with data found in the literature, probably because of the larger number of patients. In this specific case, the treatment did not have an influence on the survival.

  10. The Evolving Role of Medical Imaging in Lymphoma Management: The Clinician's Perspective.

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    Svoboda, Jakub; Schuster, Stephen J

    2012-01-01

    Hodgkin and non-Hodgkin lymphomas are a heterogeneous group of hematologic neoplasms which arise from malignant lymphocytes. Imaging plays an important role in management of lymphoma patients during diagnosis, staging, and response assessment. Functional imaging may also provide prognostic information and improve the ability to detect extranodal disease. This article provides an overview of the evolving role of various imaging techniques in lymphoma from the clinician's perspective. It serves as an introduction to the other articles in this issue that focus on specific areas of lymphoma imaging.

  11. Primary Pancreatic Lymphoma in a Patient with Maturity Onset Diabetes of the Young type 3

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    Valentina Bozzoli

    2012-01-01

    Full Text Available Primary pancreatic lymphoma (PPL is an extremely rare disease which occurs in pancreas, accounts for less than 1% of extra-nodal malignant lymphomas and 0,5% of cases of pancreatic masses. We report the case of PPL in a 15 year-old boy suffering from Maturity onset Diabetes of the young type 3 (MODY3 diagnosed at the age of 1 year

  12. [Muscle tissue lymphoma presenting only with fever of unknown origin: a case report and literature review].

    Science.gov (United States)

    Shen, Xiaodong; Chen, Xin; Xiao, Hongju; Liu, Gang; Zhai, Yongzhi; Xu, Baixuan; Shi, Huaiyin; Li, Tanshi; Zhu, Haiyan

    2015-06-01

    Extra-nodal malignant lymphoma is often characterized by a lack of typical symptoms and positive results of auxiliary examinations, which make diagnosis difficult. In some cases, fever can be the only clinical manifestation. For the lymphoma patients presenting with persistent fever with a duration over 3 weeks, characteristics of fever including time of fever attack, fever type and effects of drugs may have significant value in the diagnosis, especially in the early stage of the disease or in rare cases.

  13. ENFERMEDAD RENAL POLIQUISTICA ASOCIADA A LINFOMA EXTRANODAL EN UN CANINO

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    Diego Echeverry B

    2006-12-01

    Full Text Available Objetivo: Presentar el caso de un paciente canino, con enfermedad renal poliquística, asociada alinfoma extranodal. Materiales y métodos: Se estudió el caso de un paciente canino de raza SiberianHusky de 7 años de edad, al que se le encontraron múltiples nódulos renales bilaterales, condiagnóstico histopatológico de linfoma extranodal. Resultados: Se expone el caso clínico de unpaciente canino de raza Siberian Husky, que es presentado a consulta dermatológica por exhibirlesiones alopécicas multifocales. Una vez realizado el examen físico sistemático se evidenció demanera incidental nefromegalia. Los exámenes paraclínicos e histopatológicos aplicadosdemostraron la presencia de enfermedad renal poliquística asociada a linfoma extranodal, comoconsecuencia se presento falla renal crónica. Conclusiones: El linfoma extranodal renal es unaentidad patológica de rara presentación, clínicamente cursa con signos muy similares a otrasalteraciones tumorales, su diagnóstico suele ser histopatológico.

  14. Radiation Therapy in a Case of Orbital Lymphoma

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    Molinaro MA

    2008-07-01

    Full Text Available Introduction: Orbital lymphoma is a rare presentation of extranodal non-Hodgkin's lymphoma, accounting for less than 1% of the total. The role of radiotherapy in its management is well-established. This is a report of a case of orbital lymphoma Materials and Methods: Clinical records and pathologic material of a patient woman of 62 age treated with local radiotherapy for localized orbital lymphoma. Treatment consisted of 36 Gy in 1.8-Gy fractions of irradiation using 6-MV photons with complex treatment planning for retrobulbar lesions. Median follow-up was 18 months Results: Local control was 100%. Cataracts was not observed in 8 of the treated eyes.Conclusion: In this case, localized orbital MALT lymphoma was well controlled with radiotherapy.

  15. Expression of CD56 and Epstein-Barr virus in nasal/nasopharyngeal lymphoma

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    Lee, Seung Sook; Cho, Kyung Ja [Korea Cancer Center Hospital, Seoul (Korea, Republic of)

    1997-12-01

    We examined malignant lymphomas and polymorphic reticulosis of nasal cavity, nasopharynx, and palate, diagnosed at Korea Cancer Center Hospital from 1987 to 1996. With immunophenotypic study, we reclassified nasal/nasopharyngeal lymphomas into three categories: CD56-positive T/NK lymphoma, CD56-negative lymphoma and B-cell lymphoma. Malignant lymphomas of nasal cavity, nasopharynx and palate were 95 patient, that comprised 11% of the total lymphoma cases, and it was the most common extranodal lymphoma. Twenty-five percent were B-cell lymphomas and 75 % were T/NK lymphomas. According to site, nasal cavity was the most frequent and 91 % of nasal cavity lymphomas were T/NK type. CD56-positive T/NK comprised 82 % of total T/NK lymphomas and CD56-negative cases were 18 %. In 89 % of total T/NK lymphomas, many tumor cells expressed EBER-1 in their nuclei (CD56+ T/NK lymphoma: 97 % of EBV expression, CD56-T-cell lymphoma; 60%). Only one case (5%) of B-cell lymphoma showed EBER-1 positivity in a few cells. CD56+ T/NK lymphomas showed significantly more angiocentricity and severe necrosis than CD56- cases. Although it has no statistical significance, T/NK lymphomas has a tendency to lower survival rates than B-cell lymphomas at 1 year and 2 year. CD56+ T/NK lymphomas has a tendency to lower survival than CD56- T/NK lymphomas (p > 0.05). Our results of this project will serve important basic materials in diagnosing and studying lymphoma. (author). 25 refs., 4 tabs., 4 figs

  16. An Unusual Presentation of B-Cell Lymphoma as a Large Isolated Epiglottic Mass: Case Report and Literature Review

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    Changxing Liu

    2016-01-01

    Full Text Available Extranodal presentation of B-cell lymphoma is uncommon. Isolated primary epiglottic B-cell lymphoma is even rarer. To our knowledge, there has been only one description of isolated B-cell lymphoma presenting as a large epiglottic mass. We report an unusual type of B-cell lymphoma of the epiglottis, as it could not be subtyped based on routine staining and hybridization. The lymphoma presented as a large isolated globular mass pedicled to the epiglottis, occupying most of the oropharynx, but did not have any ball-valving effect or increased respiratory efforts. Initial radiographic findings were nonspecific. The diagnosis of B-cell lymphoma was determined by transoral incisional biopsy under local anesthesia. The condition was treated successfully with chemoradiation. The current standard of treatment for high grade B-cell lymphoma is concurrent chemoradiotherapy, with excellent prognosis. Although rare, B-cell lymphoma should be considered when investigating pedunculated hypopharyngeal masses.

  17. Cutaneous T-cell lymphomas and their management strategies

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    S S Pandey

    2014-01-01

    Full Text Available Cutaneous T-cell lymphomas (CTCLs comprise a heterogeneous group of lymphoproliferative disorders characterized by the proliferation of skin-homing post-thymic T-cells. It is the second most common extranodal non-Hodgekin′s lymphoma. Many variants of mycosis fungoides and CTCLs are known to date, differing in clinical, histological, and immunophenotypic characteristics. Oral involvement has also been reported rarely in CTCLs. Treatment depends on the disease stage or the type of variant. New insights into the disease and the number of emerging novel therapeutic options have made it an interesting area for dermatologists and medical oncologists.

  18. Primary cutaneous blastoid mantle cell lymphoma-case report.

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    Estrozi, Bruna; Sanches, José A; Varela, Paulo C S; Bacchi, Carlos E

    2009-06-01

    Mantle cell lymphoma (MCL) commonly involves extranodal sites, usually as a manifestation of disseminated disease. In rare cases, MCLs may arise as a primary tumor in the skin. Blastoid mantle cell lymphoma (BV-MCL) is a rare variant and has a more aggressive clinical course. The phenotype of BV-MCL is characterized as CD20+, CD5+, cyclin D1+, CD23-, and CD10-. Interphase fluorescence in situ hybridization shows a characteristic t(11;14) fusion pattern. We report a case of a BV-MCL arising in skin as primary cutaneous MCL with the characteristic immunophenotype and translocation.

  19. Dual anaplastic large cell lymphoma mimicking meningioma: A case report

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    Kim, Keun Ho; Kim, Ki Hwan; Lee, Ghi Jai; Lee, Hye Kyung; Shim, Jae Chan; Lee, Kyoung Eun; Suh, Jung Ho [Seoul Paik Hospital, Inje University College of Medicine, Seoul (Korea, Republic of); Lee, Chae Heuck [Dept. of Neurosurgery, Ilsan Paik Hospital, Inje University College of Medicine, Goyang (Korea, Republic of)

    2014-01-01

    Anaplastic large cell lymphoma (ALCL) is a rare T cell lymphoma composed of CD30-positive lymphoid cells. Most ALCLs present as nodal disease, with skin, bone, soft tissue, lung, and liver as common extranodal sites. ALCL rarely occurs in the central nervous system and is even more infrequent in the dura of the brain. We report a case of dural-based ALCL secondary to systemic disease in a 17-year-old male that mimicked meningioma on magnetic resonance imaging and angiography.

  20. A virtual tissue bank for primary central nervous system lymphomas in immunocompetent individuals.

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    Ponzoni, Maurilio; Kwee, Ivo; Mazzucchelli, Luca; Ferreri, Andrés J M; Zucca, Emanuele; Doglioni, Claudio; Cavalli, Franco; Bertoni, Francesco

    2007-01-01

    Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin's lymphoma with continuously increasing incidence in both immunosuppressed and immunocompetent individuals. PCNSL is a very aggressive tumor with a poor outcome, and its clinical outcome is much worse than for nodal lymphomas. Differently from lymphomas arising in lymph nodes or in other extranodal sites, the treatment of PCNSL remains very unsatisfactory. Current biologic knowledge of PCNSL is still limited and several fundamental questions remain to be answered. This is mainly due to the paucity of PCNSL material for adequate translational research. With the aim of providing biologic material to investigators interested in PCNSL, we have implemented a virtual tissue bank (VTB) for PCNSL in immunocompetent patients. After registration, the VTB is accessible via any web browser at www.ielsg.org. Only anonymous data are centralized at the website of the International Extranodal Lymphoma Study Group, whilst the pathologic material is maintained at the local pathology institutes. (c) 2007 S. Karger AG, Basel.

  1. Development of autoimmunity in lymphoma.

    Science.gov (United States)

    Jardin, Fabrice

    2008-03-01

    Development of lymphoproliferative diseases during the course of autoimmune and chronic inflammatory conditions is well established. Conversely, development of clinical or biological signs of autoimmunity at the time of the diagnosis of lymphoma or during its course indicates that lymphoma and autoimmune manifestations may constitute two faces of the same process. The aim of this review is to describe autoimmune manifestations related to non-Hodgkin's lymphoma and Hodgkin's lymphoma, their specificity according to the lymphoma subtype and their physiopathological signification. Lymphoma-related autoimmune manifestations include mainly skin diseases, hematological manifestations, rheumatic diseases and renal lesions. Despite the lack of studies providing a systematic prospective assessment, autoimmune manifestations are observed in all lymphoma subtypes and seem particularly prevalent in marginal-zone lymphoma and T-cell lymphoma. Autoimmune manifestation's physiopathology may implicate production of autoantibodies by CD5-positive autoreactive B cells, a loss of immune tolerance, an alteration of the Fas/Fas-ligand pathway and/or a chronic antigenic stimulation. Monoclonal antibodies (including rituximab, Campath-1H or epratuzumab) constitute the most promising approach to treat lymphoma-related immune disorders.

  2. An overview of cutaneous T cell lymphomas [version 1; referees: 2 approved

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    Nooshin Bagherani

    2016-07-01

    Full Text Available Cutaneous T cell lymphomas (CTCLs are a heterogeneous group of extranodal non-Hodgkin’s lymphomas that are characterized by a cutaneous infiltration of malignant monoclonal T lymphocytes. They typically afflict adults with a median age of 55 to 60 years, and the annual incidence is about 0.5 per 100,000. Mycosis fungoides, Sézary syndrome, and primary cutaneous peripheral T cell lymphomas not otherwise specified are the most important subtypes of CTCL. CTCL is a complicated concept in terms of etiopathogenesis, diagnosis, therapy, and prognosis. Herein, we summarize advances which have been achieved in these fields.

  3. Bortezomib, Rituximab, and Dexamethasone With or Without Temsirolimus in Treating Patients With Untreated or Relapsed Waldenstrom Macroglobulinemia or Relapsed or Refractory Mantle Cell or Follicular Lymphoma

    Science.gov (United States)

    2017-01-31

    Cognitive Side Effects of Cancer Therapy; Fatigue; Neurotoxicity Syndrome; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Therapy-Related Toxicity; Waldenstrom Macroglobulinemia

  4. Non-Hodgkin lymphoma

    Science.gov (United States)

    Lymphoma - non-Hodgkin; Lymphocytic lymphoma; Histiocytic lymphoma; Lymphoblastic lymphoma; Cancer - non-Hodgkin lymphoma ... National Cancer Institute: PDQ adult non-Hodgkin lymphoma treatment. Bethesda, MD: National Cancer Institute. Updated ... . Accessed ...

  5. Non-Hodgkin lymphoma in the developing world: review of 4539 cases from the International Non-Hodgkin Lymphoma Classification Project.

    Science.gov (United States)

    Perry, Anamarija M; Diebold, Jacques; Nathwani, Bharat N; MacLennan, Kenneth A; Müller-Hermelink, Hans K; Bast, Martin; Boilesen, Eugene; Armitage, James O; Weisenburger, Dennis D

    2016-10-01

    The distribution of non-Hodgkin lymphoma subtypes varies around the world, but a large systematic comparative study has never been done. In this study, we evaluated the clinical features and relative frequencies of non-Hodgkin lymphoma subtypes in five developing regions of the world and compared the findings to the developed world. Five expert hematopathologists classified 4848 consecutive cases of lymphoma from 26 centers in 24 countries using the World Health Organization classification, and 4539 (93.6%) were confirmed to be non-Hodgkin lymphoma, with a significantly greater number of males than females in the developing regions compared to the developed world (Pworld (90.7% and 9.3%, respectively). Also, the developing regions had significantly more cases of high-grade B-cell lymphoma (59.6%) and fewer cases of low-grade B-cell lymphoma (22.7%) compared to the developed world (39.2% and 32.7%, respectively). Among the B-cell lymphomas, diffuse large B-cell lymphoma was the most common subtype (42.5%) in the developing regions. Burkitt lymphoma (2.2%), precursor B- and T-lymphoblastic leukemia/lymphoma (1.1% and 2.9%, respectively) and extranodal natural killer/T-cell lymphoma (2.2%) were also significantly increased in the developing regions. These findings suggest that differences in etiologic and host risk factors are likely responsible, and more detailed epidemiological studies are needed to better understand these differences.

  6. Clinico-pathological signiifcance of extra-nodal spread in special types of breast cancer

    Institute of Scientific and Technical Information of China (English)

    Ecmel Isik Kaygusuz; Handan Cetiner; Hulya Yavuz

    2014-01-01

    Objective: To investigate the signiifcance of extra-nodal spread in special histological sub-types of breast cancer and the relationship of such spread with prognostic parameters. Methods: A total of 303 breast cancer cases were classiifed according to tumor type, and each tumor group was subdivided according to age, tumor diameter, lymph node metastasis, extra-nodal spread, vein invasion in the adjacent soft tissue, distant metastasis, and immunohistochemical characteristics [estrogen receptor (ER), progesterone receptor (PR) existence, p53, c-erbB-2, and proliferative rate (Ki-67)]. hTe 122 cases with extra-nodal spread were clinically followed up. Results: An extra-nodal spread was observed in 40% (122 cases) of the 303 breast cancer cases. hTe spread most frequently presented in micro papillary carcinoma histological sub-type (40 cases, 75%), but least frequently presents in mucinous carcinoma (2 cases, 8%). Patients with extra-nodal spread had a high average number of metastatic lymph nodes (8.3) and a high distant metastasis rate (38 cases, 31%) compared with patients without extra-nodal spread. Conclusion: hTe existence of extra-nodal spread in the examined breast cancer sub-types has predictive value in forecasting the number of metastatic lymph nodes and the disease prognosis.

  7. Leukemic phase of anaplastic lymphoma kinase positive, anaplastic large cell lymphoma

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    Vijaya S Gadage

    2011-01-01

    Full Text Available Anaplastic large cell lymphoma (ALCL is a distinct type of CD30+ T/null-cell non-Hodgkin′s lymphoma that frequently involves nodal and extranodal sites. The presence of leukemic phase in ALCL is extremely rare and occurs exclusively with ALK1-positive ALCL. We describe two patients with ALK1-positive ALCL who developed a leukemic phase with rapid progression of the disease. Immunophenotypic pattern assessed on peripheral blood by flow cytometry revealed CD45, CD30, and CD25 positivity in both cases but NPM-ALK1 was expressed in only one case. Both patients developed leukemic phase as a terminal event of the disease and we share the immunophenotypic features of both cases.

  8. [Lymphomas of the gastrointestinal tract: a neoplastic disease of underdevelopment].

    Science.gov (United States)

    Solidoro, A; Casanova, L; Otero, J; Santillana, S; Carracedo, C; Misad, O; Ruiz, E

    1993-01-01

    458/3,495 malignant lymphomas seen at the Instituto de Enfermedades Neoplásicas between 1965-1992, had primary extranodal disease in the GI tract. This is one of the largest institutional series reported, which would suggest that this is a relatively frequent malignancy in Peruvian population. Fifty per cent of cases had a primary in the small bowel and 38.9 per cent in the stomach. The age at presentation, the clinical picture and the location at the intestine show similarities with the so called Mediterranean lymphoma. Cases were classified according to the TNM system, and patients in stages I-II were surgically resected; 80 per cent of them were alive and free of disease at 5 years. Gastric lymphomas with inoperable disease were treated with chemotherapy with a 5-year survival of 50 per cent.

  9. Hodgkin Lymphoma (For Teens)

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    ... Can I Help Someone Who's Being Bullied? Volunteering Hodgkin Lymphoma KidsHealth > For Teens > Hodgkin Lymphoma Print A ... to check for disease, including lymphoma. What Is Hodgkin Lymphoma? Hodgkin lymphoma is a type of cancer ...

  10. [Coincidence of colonic lymphoma and gallstone ileus - case report].

    Science.gov (United States)

    Černá, M; Opatrný, V; Nosek, J; Geiger, J; Třeška, V; Boudová, L; Buriánek, V

    Primary colonic lymphoma is a very rare malignant disease of the gastrointestinal tract, accounting for 14% of all malignant diseases in this location. It is classified in the group of extranodal lymphomas; its long-term asymptomatic progression makes it different from common colorectal carcinomas making its diagnosis very difficult, more often accidental. Gallstone ileus is quite an uncommon complication of cholecystolithiasis diagnosed with difficulty. Up to 50% of cases are diagnosed during surgery. The obturated location depends on the size of the stone, location of the conjunction between the biliary and gastrointestinal tracts, and also on any preexisting stenosis due to another unknown pathology. We present a case of an 86-year-old man treated for acute diverticulitis with typical clinical symptoms. Following further examination (colonoscopy, computed tomography) revealed a tumour-like infiltration in the sigmoid colon wall and a voluminous polyp was suspected according to the colonoscopy. Computed tomography described an obstruction by a biliary stone tumbling through the cholecystocolonic fistula. Subsequent biopsy supported the suspected malignant etiology. The patient underwent resection of the sigmoid colon sec. Hartmann; an infiltration was found in the subhepatic space, which corresponded to the described fistulisation between the biliary tract and the colon. A large 40 mm gallstone was found in the resected sigmoid colon over the stenosis and the bowel wall showed diffuse thickening with several polyps; final histopathological assessment confirmed malignant lymphoma of the plasmocytoma type. No serious complications occurred in the postoperative period; after healing, the patient was transferred to hematooncology care. The article describes the presence of two rare diseases - colonic lymphoma and gallstone ileus. Clearly, without the biliary stone obstruction in the preexisting tumorous stenosis in the sigmoid colon, the malignant hematooncology

  11. Non-Hodgkin lymphoma in the developing world: review of 4539 cases from the International Non-Hodgkin Lymphoma Classification Project

    Science.gov (United States)

    Perry, Anamarija M.; Diebold, Jacques; Nathwani, Bharat N.; MacLennan, Kenneth A.; Müller-Hermelink, Hans K.; Bast, Martin; Boilesen, Eugene; Armitage, James O.; Weisenburger, Dennis D.

    2016-01-01

    The distribution of non-Hodgkin lymphoma subtypes varies around the world, but a large systematic comparative study has never been done. In this study, we evaluated the clinical features and relative frequencies of non-Hodgkin lymphoma subtypes in five developing regions of the world and compared the findings to the developed world. Five expert hematopathologists classified 4848 consecutive cases of lymphoma from 26 centers in 24 countries using the World Health Organization classification, and 4539 (93.6%) were confirmed to be non-Hodgkin lymphoma, with a significantly greater number of males than females in the developing regions compared to the developed world (P<0.05). The median age at diagnosis was significantly lower for both low- and high-grade B-cell lymphoma in the developing regions. The developing regions had a significantly lower frequency of B-cell lymphoma (86.6%) and a higher frequency of T- and natural killer-cell lymphoma (13.4%) compared to the developed world (90.7% and 9.3%, respectively). Also, the developing regions had significantly more cases of high-grade B-cell lymphoma (59.6%) and fewer cases of low-grade B-cell lymphoma (22.7%) compared to the developed world (39.2% and 32.7%, respectively). Among the B-cell lymphomas, diffuse large B-cell lymphoma was the most common subtype (42.5%) in the developing regions. Burkitt lymphoma (2.2%), precursor B- and T-lymphoblastic leukemia/lymphoma (1.1% and 2.9%, respectively) and extranodal natural killer/T-cell lymphoma (2.2%) were also significantly increased in the developing regions. These findings suggest that differences in etiologic and host risk factors are likely responsible, and more detailed epidemiological studies are needed to better understand these differences. PMID:27354024

  12. Central nervous system involvement in T-cell lymphoma: A single center experience.

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    Gurion, Ronit; Mehta, Neha; Migliacci, Jocelyn C; Zelenetz, Andrew; Moskowitz, Alison; Lunning, Matthew; Moskowitz, Craig; Hamlin, Paul; Horwitz, Steven

    2016-05-01

    Background We characterized the incidence of central nervous system (CNS) involvement, risk factors and outcome in a large single institution dataset of peripheral T-cell lymphoma (PTCL). Methods Retrospective review of the PTCL database at Memorial Sloan Kettering Cancer Center. We identified 231 patients with any subtype of PTCL between 1994-2011 with a minimum six months of follow-up or an event defined as relapse or death. Results Histologies included peripheral T-cell lymphoma-not otherwise specified (PTCL-NOS) (31.6%), angioimmunoblastic (16.9%), anaplastic large cell lymphoma (ALCL), ALK- (12.1%), ALCL, ALK + (6.1%), extranodal NK/T-cell lymphoma (7.4%), adult T-cell leukemia/lymphoma (ATLL) (7.4%), and transformed mycosis fungoides (8.7%). Seventeen patients had CNS disease (7%). Fifteen had CNS involvement with PTCL and two had diffuse large B-cell lymphoma and glioblastoma. Median time to CNS involvement was 3.44 months (0.16-103.1). CNS prophylaxis was given to 24 patients (primarily intrathecal methotrexate). Rates of CNS involvement were not different in patients who received prophylaxis. Univariate analysis identified stage III-IV, bone marrow involvement, >1 extranodal site and ATLL as risk factors for CNS disease. On multivariate analysis, >1 extranodal site and international prognostic index (IPI) ≥ 3 were predictive for CNS involvement. The median survival of patients with CNS involvement was 2.63 months (0.10-75). Conclusions Despite high relapse rates, PTCL, except ATLL, carries a low risk of CNS involvement. Prognosis with CNS involvement is poor and risk factors include: >1 extra nodal site and IPI ≥3.

  13. Clinical impact of mesorectal extranodal cancer tissue in rectal cancer: detailed pathological assessment using whole-mount sections.

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    Shimada, Yoshifumi; Takii, Yasumasa

    2010-05-01

    Mesorectal cancer deposits showing no histological evidence of lymph node structure (extranodal cancer tissue) are a common feature in rectal cancer. However, optimal categorization of extranodal cancer tissue using TNM grading is not yet established. We reviewed extranodal cancer tissue in detail using whole-mount sections to clarify its clinical impact. This retrospective study involved 214 consecutive patients with stage I-III rectal cancer. After fixation, the whole tumor mass including the mesorectum was sliced into longitudinal sections and stained. Mesorectal involvement was classified as direct tumor infiltration, lymph node involvement, or extranodal cancer tissue. Extranodal cancer tissue was classified morphologically, and its maximum size and distance from the primary tumor were measured. The clinical impact of extranodal cancer tissue was evaluated by univariate and multivariate analyses. : A total of 498 extranodal cancer deposits were detected in 88 patients (41.1%). Multivariate Cox proportional hazards model analysis indicated that the presence of extranodal cancer tissue was an independent prognostic factor for relapse-free (P cancer tissue was higher than for nodal involvement, irrespective of morphological classification. The clinical impact differed significantly with the number of histological types of extranodal cancer tissue, the number of deposits, their maximum size, and their distance from the primary tumor. In the present study, we have shown that extranodal cancer tissue detected by whole-mount sections has a clinical impact that is more severe than nodal involvement.

  14. From array-based hybridization of Helicobacter pylori isolates to the complete genome sequence of an isolate associated with MALT lymphoma

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    Mégraud Francis

    2010-06-01

    Full Text Available Abstract Background elicobacter pylori infection is associated with several gastro-duodenal inflammatory diseases of various levels of severity. To determine whether certain combinations of genetic markers can be used to predict the clinical source of the infection, we analyzed well documented and geographically homogenous clinical isolates using a comparative genomics approach. Results A set of 254 H. pylori genes was used to perform array-based comparative genomic hybridization among 120 French H. pylori strains associated with chronic gastritis (n = 33, duodenal ulcers (n = 27, intestinal metaplasia (n = 17 or gastric extra-nodal marginal zone B-cell MALT lymphoma (n = 43. Hierarchical cluster analyses of the DNA hybridization values allowed us to identify a homogeneous subpopulation of strains that clustered exclusively with cagPAI minus MALT lymphoma isolates. The genome sequence of B38, a representative of this MALT lymphoma strain-cluster, was completed, fully annotated, and compared with the six previously released H. pylori genomes (i.e. J99, 26695, HPAG1, P12, G27 and Shi470. B38 has the smallest H. pylori genome described thus far (1,576,758 base pairs containing 1,528 CDSs; it contains the vacAs2m2 allele and lacks the genes encoding the major virulence factors (absence of cagPAI, babB, babC, sabB, and homB. Comparative genomics led to the identification of very few sequences that are unique to the B38 strain (9 intact CDSs and 7 pseudogenes. Pair-wise genomic synteny comparisons between B38 and the 6 H. pylori sequenced genomes revealed an almost complete co-linearity, never seen before between the genomes of strain Shi470 (a Peruvian isolate and B38. Conclusion These isolates are deprived of the main H. pylori virulence factors characterized previously, but are nonetheless associated with gastric neoplasia.

  15. Extranodal manifestation of Rosai-Dorfman disease with bilateral ocular involvement

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    Chayanika Kala

    2011-01-01

    Full Text Available Rosai-Dorfman disease, that is, sinus histiocytosis with massive lymphadenopathy is a benign systemic proliferative disorder of histiocytes. The typical clinical presentation of the disease includes bilateral painless massive lymphadenopathy, fever and polyclonal hypergammaglobulinemia. Extranodal involvement is present in only a few cases and skin lesions are the most common form of extranodal disease. However, purely cutaneous Rosai-Dorfman disease is uncommon. In this study, we describe a 10-year-old child presenting with bilateral ocular involvement.

  16. Plasmablastic Lymphoma: A Report of 2 Cases with Review of Literature.

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    Geetha Vasudevan

    2016-09-01

    Full Text Available Plasmablastic lymphoma (PBL is an aggressive subtype of Non-Hodgkin Lymphoma (NHL initially described in extra-nodal sites in an immunocompromised and later in immune-competent patients. PBL remains a diagnostic challenge due to morphological overlap with various other entities and also due to similarities on immunohistochemistry with plasma cell myeloma. In-spite of therapeutic advances, PBL remains an aggressive disease with high fatality rate. We describe 2 cases of this uncommon neoplasm; both in immunocompromised patients. [J Interdiscipl Histopathol 2016; 4(3.000: 74-78

  17. Flow cytometric detection and quantification of CD56 (neural cell adhesion molecule, NCAM) expression in diffuse large B cell lymphomas and review of the literature.

    Science.gov (United States)

    Stacchini, Alessandra; Barreca, Antonella; Demurtas, Anna; Aliberti, Sabrina; di Celle, Paola Francia; Novero, Domenico

    2012-02-01

    To report unusual CD56 (neural cell adhesion molecule, NCAM) expression on diffuse large B cell lymphoma (DLBCL). CD56 expression was first detected and quantified on tissues obtained from five cases of DLBCL by flow cytometry (FC), then confirmed by immunohistochemistry. The CD56 expression pattern was heterogeneous among the cases [the molecular equivalent of soluble fluorochrome (MESF) level ranged from 2214 to 133 466]. All were CD10 and Bcl-6 positive, suggesting their germinal centre origin; one was also CD5 positive. An extranodal presentation occurred in three of five cases. CD56 expression in B cell lymphoma is a rare occurrence. FC is able to identify aberrant immunophenotypes that can be useful in the identification and monitoring of B cell lymphoma subtypes. The presence of CD56 reported by the literature on certain DLBCL with extranodal presentation might be related to mechanisms involved in growth and expansion. © 2012 Blackwell Publishing Limited.

  18. Monoclonal Antibody Therapy Before Stem Cell Transplant in Treating Patients With Relapsed or Refractory Lymphoid Malignancies

    Science.gov (United States)

    2015-12-07

    Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; T-cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenström Macroglobulinemia

  19. t(11 ; 18) (q21 ; q21) in gastric extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue%胃黏膜相关淋巴组织结外边缘区淋巴瘤t(11;18)(q21;q21)染色体易位分析

    Institute of Scientific and Technical Information of China (English)

    黄榕芳; 陈刚; 陈宝珍; 朱伟峰; 何诚

    2014-01-01

    目的 探讨胃黏膜相关淋巴组织(MALT)结外边缘区淋巴瘤t(11;18)(q21;q21)染色体易位的发生情况及相关临床意义.方法 采用间期荧光原位杂交(FISH)方法对18例胃MALT淋巴瘤进行检测.结果 3例检测到t(11;18)(q21;q21),发生率16.7%(3/18).1例黏膜相关淋巴组织淋巴瘤易位基因1(MALT1)拷贝数增加,发生率5.6%(1/18).结论 t(11;18)(q21;q21)是胃MALT淋巴瘤具有特异性的诊断指标,而间期FISH是检测t(11;18)(q21;q21)有效而可靠的方法.评估MALT1基因数目可能有利于预测胃MALT淋巴瘤患者的临床过程.

  20. Chlamydia psittaci in ocular adnexa MALT lymphoma: a possible role in lymphomagenesis and a different geographical distribution

    Directory of Open Access Journals (Sweden)

    Collina Francesca

    2012-04-01

    Full Text Available Abstract Ocular adnexa MALT-lymphomas represent approximatively 5-15% of all extranodal lymphomas. Almost 75% of OAMLs are localized in orbital fat, while 25% of cases involves conjunctive. MALT-lymphomas often recognize specific environmental factors responsible of lymphoma development and progression. In particular as Helicobacter pylori in gastric MALT lymphomas, other bacterial infections have been recognized related to MALT lymphomas in specific site. Recently Chlamydia psittaci has been identified in Ocular Adnexa MALT lymphomas, with variable frequence dependently from geographic areas. Thus bacterial infection is responsible of clonal selection on induced MALT with subsequent lymphoma development. Moreover Chlamydia psittaci could promote chromosomal aberration either through genetic instability as a consequence of induced proliferation and probably through DNA oxidative damage. The most common translocation described in MALT lymphomas affects NF-kB pathway with a substantial antiapoptotic effect. Several therapeutic approaches are now available, but the use of antibiotic-therapy in specific cases, although with conflicting results, could improve the treatment of ocular adnexa MALT lymphomas. In this review we analyse the most relevant features of Ocular adnexa MALT lymphomas, underlining specific biological characteristics mainly related to the potential role of Chlamydia psittaci in lymphomagenesis.

  1. Alemtuzumab, Fludarabine Phosphate, and Total-Body Irradiation Followed by Cyclosporine and Mycophenolate Mofetil in Treating Patients Who Are Undergoing Donor Stem Cell Transplant for Hematologic Cancer

    Science.gov (United States)

    2016-06-13

    Acute Undifferentiated Leukemia; Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Myeloid Leukemia in Remission; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative; Childhood Acute Lymphoblastic Leukemia in Remission; Childhood Acute Myeloid Leukemia in Remission; Childhood Burkitt Lymphoma; Childhood Chronic Myelogenous Leukemia; Childhood Diffuse Large Cell Lymphoma; Childhood Immunoblastic Large Cell Lymphoma; Childhood Myelodysplastic Syndromes; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Chronic Myelomonocytic Leukemia; Chronic Phase Chronic Myelogenous Leukemia; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Juvenile Myelomonocytic Leukemia; Mast Cell Leukemia; Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Childhood Anaplastic Large Cell Lymphoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Cutaneous T

  2. Fludarabine Phosphate, Low-Dose Total-Body Irradiation, and Donor Stem Cell Transplant Followed by Cyclosporine, Mycophenolate Mofetil, Donor Lymphocyte Infusion in Treating Patients With Hematopoietic Cancer

    Science.gov (United States)

    2016-08-01

    Acute Undifferentiated Leukemia; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Childhood Burkitt Lymphoma; Childhood Diffuse Large Cell Lymphoma; Childhood Grade III Lymphomatoid Granulomatosis; Childhood Immunoblastic Large Cell Lymphoma; Childhood Myelodysplastic Syndromes; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Chronic Myelomonocytic Leukemia; Cutaneous B-cell Non-Hodgkin Lymphoma; de Novo Myelodysplastic Syndromes; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Juvenile Myelomonocytic Leukemia; Mast Cell Leukemia; Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable; Myeloid/NK-cell Acute Leukemia; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Post-transplant Lymphoproliferative Disorder; Previously Treated Myelodysplastic Syndromes; Primary Systemic Amyloidosis; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Childhood Anaplastic Large Cell Lymphoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma

  3. Gastric lymphoma

    Directory of Open Access Journals (Sweden)

    Sravani Padala

    2016-06-01

    Full Text Available Gastrointestinal lymphomas represent 5-20% of extra nodal lymphomas and mainly occur in the stomach and small intestine. Clinical findings are not specific, thus often determining a delay in the diagnosis. Imaging features at conventional and cross-sectional imaging must be known by the radiologist since he/she plays a pivotal role in the diagnosis and disease assessment, thus assisting in the choice of the optimal treatment to patients. This review focuses on the wide variety of imaging presentation of esophageal, gastric, and small and large bowel lymphoma presenting their main imaging appearances at conventional and cross-sectional imaging, mainly focusing on computed tomography and magnetic resonance, helping in the choice of the best imaging technique for the disease characterization and assessment and the recognition of potential complications. Gastrointestinal tract is the most common extra nodal site involved by lymphoma. Although lymphoma can involve any part of the gastrointestinal tract .The most frequent sites in order of its occurrence are the stomach followed by small intestine and ileocecal region. Gastrointestinal tract lymphoma is usually secondary to the widespread nodal diseases and primary gastrointestinal tract lymphoma is relatively rare. [Int J Res Med Sci 2016; 4(6.000: 2481-2486

  4. A rare cytological diagnosis of primary non-Hodgkin lymphoma of the parotid gland

    Directory of Open Access Journals (Sweden)

    Biswajit Dey

    2016-01-01

    Full Text Available Primary lymphoma of the parotid gland is relatively rare and constitutes about 4-5% of extranodal lymphomas. The majority of them is non-Hodgkin lymphoma (NHL and is B cell in nature. We report a case of primary diffuse large B-cell lymphoma (DLBCL of the parotid gland in an elderly male. The case was diagnosed on fine needle aspiration cytology (FNAC of the right parotid gland as high grade B-cell NHL and confirmed on histopathology as DLBCL. In correlation with the clinicoradiological findings, the case was diagnosed as primary parotid DLBCL. The case highlights the role of FNAC as a timely and useful diagnostic tool.

  5. Primary NK/T cell lymphoma nasal type of the colon

    Directory of Open Access Journals (Sweden)

    Ana María Chirife

    2013-02-01

    Full Text Available Since nasal NK/T-cell lymphoma and NK/T-cell lymphoma nasal type are rare diseases, colonic involvement has seldom been seen. We report a case of a patient with a primary NK/T-cell lymphoma nasal type of the colon. The patient had no history of malignant diseases and was diagnosed after exhaustive study in the context of fever of unknown origin. The first therapeutic approach followed the DAEPOCH-protocol: etoposide, prednisone, doxor-rubicin, vincristine and cyclophosphamide. The persistence of constitutional symptoms after the first treatment course motivated the switch to a second line following the SMILE-protocol: dexamethasone, metotrexate, ifosfamide, E.coli L-asparaginase, and etoposide. Despite intensive chemotherapy, the patient died 2 months after the diagnose of an extranodal NK/T-cell lymphoma of the colon and 4 months after the first symptomatic appearance of disease.

  6. Endoscopic ultrasound-guided fine needle aspiration cytology and biopsy in the evaluation of lymphoma

    Science.gov (United States)

    Gimeno-García, Antonio Z.; Elwassief, Ahmed; Paquin, Sarto C.; Sahai, Anand V.

    2012-01-01

    Accurate diagnosis and subtyping of lymphoma have important prognostic implications and are generally required for treatment planning. Histological assessment, immunophenotyping, and genetic studies are usually necessary. Endoscopic ultrasound guided-fine needle aspiration cytology (EUS-FNAC) is a minimally invasive technique widely used for the evaluation of deep-seated benign and malignant lesions. However, the value of cytological samples in lymphoma diagnosis is still a matter of debate. Endoscopic ultrasound guided-fine needle biopsy (EUS-FNAB) can provide tissue core samples that may help overcome the limitations of cytology. The aim of this review is to summarize the available literature regarding EUS-FNAC and EUS-FNAB for the diagnosis and subtyping of lymphoma. In addition, we discuss its usefulness in the management of primary extra-nodal lymphomas, as well as technical issues that may influence sample quality. PMID:24949331

  7. Non Hodgkin's lymphoma with cutaneous involvement in AIDS patients: report of five cases and review of the literature

    Directory of Open Access Journals (Sweden)

    Marcelo Corti

    2010-02-01

    Full Text Available Cutaneous B cell lymphoma (CBCL is a lymphoproliferative disorder of neoplastic B cell of the skin with a wide range of clinical manifestations. Commonly, the clinical features of CBCL are plaques, nodules, or ulcerative lesions. Skin is one of the common sites for extra-nodal lymphomas in patients with AIDS and B cell type is less common than T cell type. Only recently, the existence of B cell lymphomas presenting clinically in the skin without evidence of extra-cutaneous involvement has been accepted as primary CBCL. Here, we are presenting 5 patients with cutaneous involvement in the setting of HIV/AIDS disease. Two of them were primary cutaneous non-Hodgkin lymphomas. All were CBCL; 3 were immunoblastic, 1 was plasmablastic, and the other was a Burkitt lymphoma. We analyzed the epidemiological, clinical, virological, and immunological characteristics of this group of patients.

  8. [Diffuse large B-cell lymphoma with concomitant c-MYC and BCL6 gene rearrangements with primary skin involvement: A case report and a review of literature].

    Science.gov (United States)

    Gabeeva, N G; Koroleva, D A; Belyaeva, A V; Chernova, N G; Kuzmina, L A; Sudarikov, A B; Obukhova, T N; Kovrigina, A M; Zvonkov, E E; Savchenko, V G

    2017-01-01

    Double-hit lymphoma (DHL) is a rare aggressive B-cell lymphoma with concomitant c-MYC, BCL2 or BCL6 gene rearrangements, which is characterized by the high frequency of extranodal lesions and by resistance to chemotherapy. The median survival does not exceed 18 months in patients with this disease. The majority of DHL is represented by с-MYC/BCL2 cases. The combination of c-MYC/BCL6 occurs rarely (5-8%). The paper describes a case of DHL with concomitant c-MYC and BCL6 gene rearrangements, which mimics diffuse large B-cell lymphoma, leg-type.

  9. Hodgkin lymphoma - children

    Science.gov (United States)

    Lymphoma - Hodgkin - children; Hodgkin disease - children; Cancer - Hodgkin lymphoma - children; Childhood Hodgkin lymphoma ... In children, Hodgkin lymphoma is more likely to occur between ages 15 to 19 years. The cause of this type of ...

  10. Hodgkin Lymphoma (For Kids)

    Science.gov (United States)

    ... Too Tall or Too Short All About Puberty Hodgkin Lymphoma KidsHealth > For Kids > Hodgkin Lymphoma Print A ... of the cool things he's missed. What Is Hodgkin Lymphoma? Lymphoma (say: lim-FOH-mah) is cancer ...

  11. Cutaneous presentation of Double Hit Lymphoma

    Directory of Open Access Journals (Sweden)

    Yousef Khelfa MD, FACP

    2016-04-01

    Full Text Available Diffuse large B-cell lymphoma (DLBCL is the most common type of non-Hodgkin lymphoma (NHL, representing approximately 25% of diagnosed NHL. DLBCL is heterogeneous disease both clinically and genetically. The 3 most common chromosomal translocations in DLBCL involve the oncogenes BCL2, BCL6, and MYC. Double hit (DH DLBCL is an aggressive form in which MYC rearrangement is associated with either BCL2 or BCL6 rearrangement. Patients typically present with a rapidly growing mass, often with B symptoms. Extranodal disease is often present. Though there is a paucity of prospective trials in this subtype, double hit lymphoma (DHL has been linked to very poor outcomes when patients are treated with standard R-CHOP. There is, therefore, a lack of consensus regarding the standard treatment for DHL. Several retrospective analyses have been conducted to help guide treatment of this disease. These suggest that DA EPOCH-R may be the most promising regimen and that achievement of complete resolution predicts better long-term outcomes.

  12. Expression of MALT1 oncogene in hematopoietic stem/progenitor cells recapitulates the pathogenesis of human lymphoma in mice

    OpenAIRE

    Vicente-Dueñas, Carolina; Campos-Sánchez, Elena; González, Marcos; Cobaleda, César; Abollo-Jiménez, Fernando; Martínez-Climent, José Ángel

    2012-01-01

    Chromosomal translocations involving the MALT1 gene are hallmarks of mucosa-associated lymphoid tissue (MALT) lymphoma. To date, targeting these translocations to mouse B cells has failed to reproduce human disease. Here, we induced MALT1 expression in mouse Sca1(+)Lin(-) hematopoietic stem/progenitor cells, which showed NF-κB activation and early lymphoid priming, being selectively skewed toward B-cell differentiation. These cells accumulated in extranodal tissues and gave rise to clonal tum...

  13. Breast lymphoma

    African Journals Online (AJOL)

    Expression of oestrogen receptor protein as determined by ... lymphomas. While this classification has been fairly widely accepted, a ... minimum a full history and physical examination, chest radiographs ... and hepatic function. A number ...

  14. Hodgkin's Lymphoma

    Science.gov (United States)

    ... for information in your local library and on the Internet. Start your information search with the National Cancer ... www.mayoclinic.org/diseases-conditions/hodgkins-lymphoma/basics/definition/CON-20030667 . Mayo Clinic Footer Legal Conditions and ...

  15. Primary lymphoma of the brain

    Science.gov (United States)

    Brain lymphoma; Cerebral lymphoma; Primary lymphoma of the central nervous system; Lymphoma - brain ... The cause of primary brain lymphoma is not known. People with a weakened immune system are at high risk for primary lymphoma of the brain. ...

  16. T-Cell Lymphoma

    Science.gov (United States)

    Getting the Facts T-Cell Lymphoma Overview Lymphoma is the most common blood cancer. The two main forms of lymphoma are Hodgkin lymphoma ... develop into lymphomas: B-lymphocytes (B-cells) and T-lymphocytes (T-cells). T-cell lymphomas account for ...

  17. Non-Hodgkin′s lymphoma of the hard palate

    Directory of Open Access Journals (Sweden)

    Jayakrishnan R

    2008-01-01

    Full Text Available Background: Non-Hodgkin′s lymphoma usually involves lymph nodes but can also involve extranodal sites. Oral lymphomas are relatively rare and often difficult to diagnose in a clinical setting. We present a case of a patient of non-Hodgkin′s lymphoma of the hard palate who had undergone external beam radiation therapy and was found to be well 1 year following treatment. Case presentation: A 43-year-old male attended the Community Oncology Division of Regional Cancer Centre with complaints of painless swelling on the right side of face of three months′ duration. A computed tomography (CT scan confirmed irregular destruction of the hard palate towards the right side of the midline with adjacent soft tissue component. Histopathology report confirmed the diagnosis of follicular lymphoma. He was given radical external beam radiation therapy (40 Gy in 20 fractions over 4 weeks. Clinical examination at the end of radiation revealed complete disappearance of the palatal swelling. Conclusion: Oral lymphomas are relatively rare and often difficult to diagnose as the clinical features mimic other pathological entities such as periodontal disease, osteomyelitis, and other malignancies. A careful clinical evaluation supported by radiologic and histopathologic investigations will help in identifying the disease at an early stage, which will result in better prognosis.

  18. ALK-positive anaplastic large cell lymphoma with soft tissue involvement in a young woman

    Directory of Open Access Journals (Sweden)

    Gao KH

    2016-07-01

    Full Text Available Kehai Gao, Hongtao Li, Caihong Huang, Huazhuang Li, Jun Fang, Chen Tian Department of Orthopaedics, Yidu Central Hospital, Shandong, People’s Republic of China Introduction: Anaplastic large cell lymphoma (ALCL is a type of non-Hodgkin lymphoma that has strong expression of CD30. ALCL can sometimes involve the bone marrow, and in advanced stages, it can produce destructive extranodal lesions. But anaplastic large cell lymphoma kinase (ALK+ ALCL with soft tissue involvement is very rare.Case report: A 35-year-old woman presented with waist pain for over 1 month. The biopsy of soft tissue lesions showed that these cells were positive for ALK-1, CD30, TIA-1, GranzymeB, CD4, CD8, and Ki67 (90%+ and negative for CD3, CD5, CD20, CD10, cytokeratin (CK, TdT, HMB-45, epithelial membrane antigen (EMA, and pan-CK, which identified ALCL. After six cycles of Hyper-CVAD/MA regimen, she achieved partial remission. Three months later, she died due to disease progression.Conclusion: This case illustrates the unusual presentation of ALCL in soft tissue with a bad response to chemotherapy. Because of the tendency for rapid progression, ALCL in young adults with extranodal lesions are often treated with high-grade chemotherapy, such as Hyper-CVAD/MA. Keywords: anaplastic large cell lymphoma, ALK+, soft tissue involvement, Hyper-CVAD/MA

  19. AIDS related thoracic lymphoma: evaluation by computed tomography; Linfoma toracico na sindrome da imunodeficiencia adquirida: avaliacao por tomografia computadorizada

    Energy Technology Data Exchange (ETDEWEB)

    Siciliano, Antonio Alexandre de Oliveira [Hospital Universitario Clementino Fraga Filho, Rio de Janeiro, RJ (Brazil). Servico de Radiodiagnostico; Marchiori, Edson [Universidade Federal, Rio de Janeiro, RJ (Brazil). Curso de Pos-graduacao em Radiologia

    2000-02-01

    The authors reviewed five cases of patients with acquired immunodeficiency syndrome (AIDS) related lymphoma to describe the thoracic findings on computed tomography. The patients were followed at Hospital Universitario Clementino Fraga Filho, Hospital Universitario Antonio Pedro and Hospital da Lagoa, from November, 1989 to March 1998. Epidemiological, clinical and pathological data from these patients were quiet variable and pulmonary nodules and masses, hilar and mediastinal lymphadenopathy, and thoracic wall masses were observed. AIDS related lymphomas involving the chest are pleomorphic and most commonly extranodal. (author)

  20. Combination therapy with brentuximab vedotin and cisplatin/cytarabine in a patient with primarily refractory anaplastic lymphoma kinase positive anaplastic large cell lymphoma.

    Science.gov (United States)

    Heidegger, Simon; Beer, Ambros J; Geissinger, Eva; Rosenwald, Andreas; Peschel, Christian; Ringshausen, Ingo; Keller, Ulrich

    2014-01-01

    Anaplastic large cell lymphoma (ALCL) is a common subtype of the heterogeneous group of peripheral T-cell lymphomas, which is characterized by large pleomorphic cells with strong expression of CD30. Translocations involving ALK, the anaplastic lymphoma kinase gene, are associated with a favorable clinical outcome. Such ALK-positive ALCLs are usually responsive to a multidrug chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone). However, there is no general consensus on the optimal therapy for relapsed or refractory ALCL. We report the case of a 24-year-old male suffering from ALK-positive ALCL with an uncommon manifestation of only extranodal disease in the gastric cardia region that showed primary refractoriness to standard CHOP chemotherapy. A combination therapy consisting of the anti-CD30 drug conjugate, brentuximab vedotin, and classical lymphoma salvage regimen DHAP (cisplatin, high-dose cytarabine and dexamethasone) was administered. Following two treatment cycles in 21-day intervals, the lymphoma showed considerable regression based on imaging diagnostics and no evidence of vital lymphoma in a subsequent biopsy. We did not observe any increase in toxicity; in particular, polyneuropathy and febrile neutropenia were not observed. In summary, we report that the antibody-drug conjugate brentuximab vedotin and a classical regimen used for aggressive lymphoma, DHAP, could be combined as salvage therapy in a case of refractory ALK-positive ALCL. Phase I/II studies will be required for safety and efficacy analysis.

  1. Disseminated non-Hodgkin's lymphoma presenting as bilateral salivary gland enlargement: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Revanappa, Manjunatha M. [Dept. of Oral Medicine and Radiology, College of Dental Sciences, Davangere (India); Sattur, Atul P.; Naikmasur, Venkatesh G. [Dept. of Oral Medicine and Radiology, SDM College of Dental Sciences and Hospital, Dharwad (India); Thakur, Arpita Rai [Dept. of Oral Medicine and Radiology, Jamia Milia Islamia University, New Delhi (India)

    2013-03-15

    Non-Hodgkin's lymphoma (NHL) constitutes a group of malignancies those arises from cellular components of lymphoid or extranodal tissues. The head and neck is the most common area for the presentation of these lymphoproliferative disorders. Primary involvement of salivary glands is uncommon. This report described a case of a 73-year-old female patient who presented with involvement of both nodal and extranodal sites, with predominant involvement of salivary glands. The tumor staging worked up along with imaging, histopathological, and immunohistochemical findings were discussed. Computed tomographic images showed the involvement of Waldeyer's ring, larynx, orbit, and spleen. This report described imaging and prognostic tumor markers in diagnosing, treatment planning, and prognosis.

  2. Cardiac Tamponade Associated with the Presentation of Anaplastic Large Cell Lymphoma in a 2-Year-Old Child

    Directory of Open Access Journals (Sweden)

    Gema Mira-Perceval Juan

    2015-01-01

    Full Text Available The anaplastic large cell lymphoma is a rare entity in pediatric patients. We present an unusual case of pericardial involvement, quite uncommon as extranodal presentation of this type of disorder, that provoked a life-risk situation requiring an urgent pericardiocentesis. To our knowledge, this is the first report on a child with pericardial involvement without an associated cardiac mass secondary to anaplastic large cell lymphoma in pediatric age. We report the case of a 21-month-old Caucasian male infant with cardiac tamponade associated with the presentation of anaplastic large cell lymphoma. Initially, the child presented with 24-day prolonged fever syndrome, cutaneous lesions associated with hepatomegaly, inguinal adenopathies, and pneumonia. After a 21-day asymptomatic period, polypnea and tachycardia were detected in a clinical check-up. Chest X-ray revealed a remarkable increase of the cardiothoracic index. The anaplastic large cell lymphoma has a high incidence of extranodal involvement but myocardial or pericardial involvements are rare. For this reason, we recommend a close monitoring of patients with a differential diagnosis of anaplastic large cell lymphoma.

  3. Lymphoma cytogenetics.

    Science.gov (United States)

    Dave, Bhavana J; Nelson, Marilu; Sanger, Warren G

    2011-12-01

    Lymphomas are a heterogeneous group of neoplasms with distinct morphologic, immunologic, and cytogenetic characteristics. Overlapping morphologic and immunophenotypic features often makes accurate diagnosis difficult. Cytogenetics helps simplify the diagnostic complexities presented in transforming and progressive lymphoid malignancies. Genetic studies using technical advances such as fluorescence in situ hybridization and the newer approaches of array comparative genomic hybridization and gene expression profiling play a critical and often defining role in the diagnosis, progression, prognosis, and therapeutic stratification. This article reviews characteristic cytogenetic abnormalities in specific subtypes of lymphomas at diagnosis, disease progression, and prognosis.

  4. A Case Report of Primary Nasal Natural Killer (NK)/T-Cell Lymphoma in an African American Patient Presenting with Hemophagocytic Syndrome

    Science.gov (United States)

    Tan, Bowei; Abdelmalek, Cherif; O’Donnell, James E.; Toltaku, Thomas; Chaudhry, Rashid; Wang, Jen C.; Gotlieb, Vladimir

    2017-01-01

    Patient: Male, 55 Final Diagnosis: Primary NK-T cell lymphoma • nasal type Symptoms: Fever • nasal bleeding • nasal mass • weight loss Medication: — Clinical Procedure: Chemotherapy×2 cycles • radiation therap Specialty: Oncology Objective: Rare disease Background: Extranodal natural killer/T-cell lymphoma, nasal type (ENKTCL) is generally an aggressive and rare non-Hodgkin lymphoma. It is most common in East Asians, Native Americans, and South Americans, but is rarely reported in blacks. Case Report: A 55-year-old African American male born in Grenada presented with a left nostril mass with facial swelling and biopsy subsequently confirmed a diagnosis of extranodal NK/T-cell lymphoma, nasal type (ENKTCL). Immunochemistry was positive for CD2, cytoplasmic CD3, CD7, CD 43, CD 56, granzyme B, and TIA-1. In situ hybridization was positive for Epstein-Barr virus encoded ribonucleic acid (EBERs). Bone marrow aspiration did not show lymphoma involvement. The patient had progressive neutropenia upon presentation, with further investigations showing hepatomegaly, hyperferritinemia, and hemophagocytosis in the bone marrow. We reached a diagnosis of hemophagocytic syndrome. He was treated with a high-dose combination chemotherapy and radiation therapy; the neutropenia improved significantly with steroids as treatment for immune activation in the setting of hemophagocytic syndrome. Conclusions: To the best of our knowledge, this is the only second report of extranodal NK/T-cell lymphoma, nasal type in a black patient, and it raises the awareness of early recognition of rare manifestations of NK/T-cell lymphoma such as hemophagocytic syndrome. PMID:28193996

  5. 443例恶性淋巴瘤临床病理特征分析%Clinical and pathological analysis of 443 cases with malignant lymphoma

    Institute of Scientific and Technical Information of China (English)

    吴永芳; 许春伟; 韩鸿雁; 王海艳; 邵云; 张博; 李晓兵; 苏航; 张伟京

    2015-01-01

    malignant lymphoma, Hodgkin’s lymphoma was 11.06%(49/443)and non-Hodgkin’s lymphoma was 88.94%(394/443). Among non-Hodgkin’s lymphoma, B cell lymphoma was 66.37%(294/443) and T cell lymphoma was 22.35%(99/443). The commonest subtypes were diffuse large B-cell lymphoma 41.53%(184/443), follicular lymphoma 11.06%(49/443), peripheral T-cell lymphoma 5.65%(25/443), T leukaemia/lymphoblastic lymphoma 5.19%(23/443), extranodal marginal zone B-cell lymphoma of MALT type 4.74%(21/443). Nodular sclerosis classical Hodgkin’s lymphoma was the commonest pathological type of Hodgkin′s lymphoma (4.51%, 20/443). hTe ratio of male to female was 1.58:1. hTe mean age at presentation was 57 years (range, 7~94 years). hTe lymph nodes of the cervical region were most commonly involved. hTe lymph nodes of the cervical region were most commonly involved. Conclusion:Of 443 cases of malignant lymphoma, diffuse large B-cell lymphoma is the commonest subtype of non-Hodgkin’s lymphoma, and Nodular sclerosis classical Hodgkin’s lymphoma is the commonest subtype of Hodgkin’s lymphoma. Most subtypes show a male predominance with most commonly affected in cervical lymphnodes.

  6. CD3-CD4+ lymphoid variant of hypereosinophilic syndrome: nodal and extranodal histopathological and immunophenotypic features of a peripheral indolent clonal T-cell lymphoproliferative disorder.

    Science.gov (United States)

    Lefèvre, Guillaume; Copin, Marie-Christine; Roumier, Christophe; Aubert, Hélène; Avenel-Audran, Martine; Grardel, Nathalie; Poulain, Stéphanie; Staumont-Sallé, Delphine; Seneschal, Julien; Salles, Gilles; Ghomari, Kamel; Terriou, Louis; Leclech, Christian; Morati-Hafsaoui, Chafika; Morschhauser, Franck; Lambotte, Olivier; Ackerman, Félix; Trauet, Jacques; Geffroy, Sandrine; Dumezy, Florent; Capron, Monique; Roche-Lestienne, Catherine; Taieb, Alain; Hatron, Pierre-Yves; Dubucquoi, Sylvain; Hachulla, Eric; Prin, Lionel; Labalette, Myriam; Launay, David; Preudhomme, Claude; Kahn, Jean-Emmanuel

    2015-08-01

    The CD3(-)CD4(+) lymphoid variant of hypereosinophilic syndrome is characterized by hypereosinophilia and clonal circulating CD3(-)CD4(+) T cells. Peripheral T-cell lymphoma has been described during this disease course, and we observed in our cohort of 23 patients 2 cases of angio-immunoblastic T-cell lymphoma. We focus here on histopathological (n=12 patients) and immunophenotypic (n=15) characteristics of CD3(-)CD4(+) lymphoid variant of hypereosinophilic syndrome. Atypical CD4(+) T cells lymphoid infiltrates were found in 10 of 12 CD3(-)CD4(+) L-HES patients, in lymph nodes (n=4 of 4 patients), in skin (n=9 of 9) and other extra-nodal tissues (gut, lacrymal gland, synovium). Lymph nodes displayed infiltrates limited to the interfollicular areas or even an effacement of nodal architecture, associated with proliferation of arborizing high endothelial venules and increased follicular dendritic cell meshwork. Analysis of 2 fresh skin samples confirmed the presence of CD3(-)CD4(+) T cells. Clonal T cells were detected in at least one tissue in 8 patients, including lymph nodes (n=4 of 4): the same clonal T cells were detected in blood and in at least one biopsy, with a maximum delay of 23 years between samples. In the majority of cases, circulating CD3(-)CD4(+) T cells were CD2(hi) (n=9 of 14), CD5(hi) (n=12 of 14), and CD7(-)(n=4 of 14) or CD7(low) (n=10 of 14). Angio-immunoblastic T-cell lymphoma can also present with CD3(-)CD4(+) T cells; despite other common histopathological and immunophenotypic features, CD10 expression and follicular helper T-cell markers were not detected in lymphoid variant of hypereosinophilic syndrome patients, except in both patients who developed angio-immunoblastic T-cell lymphoma, and only at T-cell lymphoma diagnosis. Taken together, persistence of tissular clonal T cells and histopathological features define CD3(-)CD4(+) lymphoid variant of hypereosinophilic syndrome as a peripheral indolent clonal T-cell lymphoproliferative

  7. HEPATITIS C VIRUS INFECTION AND LYMPHOMA

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    Emmanuel Bachy

    2010-03-01

    Full Text Available Apart from its well known role as an etiological agent for non-A and non-B viral hepatitis, there is growing evidence that hepatitis C virus is associated to B-cell non-Hodgkin lymphoma. The association between HCV and lymphoproliferative disorders has been recently postulated based on epidemiological data, biological studies and clinical observations. Although various subtypes of lymphomas appear to be associated to HCV, diffuse large B-cell lymphoma, small lymphocytic lymphoma/chronic lymphocytic leukemia and marginal zone lymphoma appeared to be particularly represented among HCV-positive patients.  The causative role of HCV in those disorders has been further supported by the response to anti-viral therapy. Despite a better understanding of pathophysiological processes at stake leading from HCV infection to overt lymphoma, many issues still need to be further elucidated. Although HCV has been demonstrated to directly infect peripheral blood mononuclear cells both in vitro and, in some cases, in vivo, a strong body of evidence rather supports the hypothesis of an indirect transformation mechanism by which sustained antigenic stimulation leads from oligoclonal to monoclonal expansion and sometimes to lymphoma, probably through secondary oncogenic events. Here, we review epidemiological and biological studies, as well as clinical data on antiviral therapy, linking HCV-infection to B-cell non-Hodgkin lymphoma.

  8. Vorinostat and Bortezomib in Treating Young Patients With Refractory or Recurrent Solid Tumors, Including Central Nervous System Tumors and Lymphoma

    Science.gov (United States)

    2013-07-01

    Childhood Burkitt Lymphoma; Childhood Central Nervous System Choriocarcinoma; Childhood Central Nervous System Germ Cell Tumor; Childhood Central Nervous System Germinoma; Childhood Central Nervous System Mixed Germ Cell Tumor; Childhood Central Nervous System Teratoma; Childhood Central Nervous System Yolk Sac Tumor; Childhood Choroid Plexus Tumor; Childhood Craniopharyngioma; Childhood Diffuse Large Cell Lymphoma; Childhood Immunoblastic Large Cell Lymphoma; Childhood Medulloepithelioma; Childhood Meningioma; Childhood Mixed Glioma; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Childhood Oligodendroglioma; Recurrent Childhood Anaplastic Large Cell Lymphoma; Recurrent Childhood Brain Stem Glioma; Recurrent Childhood Central Nervous System Embryonal Tumor; Recurrent Childhood Cerebellar Astrocytoma; Recurrent Childhood Cerebral Astrocytoma; Recurrent Childhood Ependymoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Malignant Germ Cell Tumor; Recurrent Childhood Medulloblastoma; Recurrent Childhood Pineoblastoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Childhood Subependymal Giant Cell Astrocytoma; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor; Recurrent Childhood Visual Pathway and Hypothalamic Glioma; Recurrent Childhood Visual Pathway Glioma; Recurrent/Refractory Childhood Hodgkin Lymphoma; Unspecified Childhood Solid Tumor, Protocol Specific

  9. A case of non-Hodgkin's lymphoma primary arising in both adrenal glands associated with adrenal failure.

    Science.gov (United States)

    Nishiuchi, Takamasa; Imachi, Hitomi; Fujiwara, Mako; Murao, Koji; Onishi, Hiroaki; Kiguchi, Tohru; Takimoto, Hidetaka; Kushida, Yoshio; Haba, Reiji; Ishida, Toshihiko

    2009-02-01

    It is known that adrenal insufficiency is one of the complications in primary adrenal lymphoma, especially those with bilateral adrenal involvement. A 73-year-old man was referred for general fatigue and high fever to the nearest hospital. The patient was transferred to our hospital for evaluation of bilateral adrenal tumors and hyponatremia. He was diagnosed as having non-Hodgkin's lymphoma (NHL) with primaries arising in both adrenal glands. Primary adrenal lymphoma (PAL) is a rare extra-nodal NHL. Although an appropriate treatment of this disease has not been established, our case has demonstrated that the combination of rituximab and THP-COP chemotherapy could be administered, and that it improved clinical manifestations. This case raises the suggestion that malignant lymphoma should be suspected in patients with bilateral adrenal tumors that present with progressive adrenal insufficiency.

  10. Clinical outcomes and prognostic factors in patients with breast diffuse large B cell lymphoma; Consortium for Improving Survival of Lymphoma (CISL study

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    Lee Je-Jung

    2010-06-01

    Full Text Available Abstract Background The breast is a rare extranodal site of non-Hodgkin lymphoma, and primary breast lymphoma (PBL has been arbitrarily defined as disease localized to one or both breasts with or without regional lymph nodes involvement. The aim of this study was to evaluate the clinical outcomes in patients with diffuse large B cell lymphoma (DLBCL and breast involvement, and to find the criteria of PBL reflecting the outcome and prognosis. Methods We retrospectively analyzed data from 68 patients, newly diagnosed with DLBCL and breast involvement at 16 Korean institutions between January 1994 and June 2009. Results Median age at diagnosis was 48 years (range, 20-83 years. Forty-three (63.2% patients were PBL according to previous arbitrary criteria, sixteen (23.5% patients were high-intermediate to high risk of international prognostic index. The patients with one extranodal disease in the breast (OED with or without nodal disease were 49 (72.1%, and those with multiple extranodal disease (MED were 19 (27.9%. During median follow-up of 41.5 months (range, 2.4-186.0 months, estimated 5-year progression-free survival (PFS was 53.7 ± 7.6%, and overall survival (OS was 60.3 ± 7.2%. The 5-year PFS and OS was significantly higher for patients with the OED group than those with the MED group (5-year PFS, 64.9 ± 8.9% vs. 27.5 ± 11.4%, p = 0.001; 5-year OS, 74.3 ± 7.6% vs. 24.5 ± 13.0%, p Conclusions Our results show that the patients included in OED group, reflecting different treatment outcome, prognosis and pattern of progression, should be considered as PBL in the future trial. Further studies are warranted to validate our suggested criteria.

  11. Various Neurological Symptoms by Neurolymphomatosis as the Initial Presentation of Primary Testicular Lymphoma

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    Yoshitaka Sunami

    2015-04-01

    Full Text Available Neurological symptoms induced by the infiltration of malignant lymphoma into the nervous systems are subsumed under the term neurolymphomatosis (NL. Here, we report the case of a 30-year-old Japanese man with primary testicular lymphoma complicated, as seen in various neurological findings, by secondary NL prior to testicular swelling. Painless right scrotal enlargement was noticed more than 1 month after the appearance of neurological complications such as right upper extremity numbness, dysarthria, facial palsy, and diplopia. Proactive investigation and biopsies of extranodal sites at high risk of central nervous system infiltration of malignant lymphoma, such as the testes, should be considered when secondary NL is suspected based on imaging findings.

  12. Hepatitis C virus and non-Hodgkin’s lymphomas: A minireview

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    Hussein Khaled

    2017-03-01

    Full Text Available B-cell NHL is strongly associated with HCV that was proved in the last 2 decades. The most common HCV infection related B-NHL subtypes include MZL and DLBCL lymphomas. HCV-positive NHL patients usually present with older age at diagnosis, higher LDH, and more extranodal disease. The standard chemo-immunotherapy tolerance is generally good. Antiviral treatment achieves virological and hematological remission in HCV associated indolent lymphoma. More aggressive lymphoma requires combination of antiviral treatment and chemotherapy. New generation of HCV antiviral drugs is safe and is highly efficacious. Regimens including DAAs appear promising options as they can reduce the HCV-associated NHL incidence by dramatically lowering the HCV chronic carriers.

  13. Isolated cutaneous involvement in a child with nodal anaplastic large cell lymphoma

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    Vibhu Mendiratta

    2016-01-01

    Full Text Available Non-Hodgkin lymphoma is a common childhood T-cell and B-cell neoplasm that originates primarily from lymphoid tissue. Cutaneous involvement can be in the form of a primary extranodal lymphoma, or secondary to metastasis from a non-cutaneous location. The latter is uncommon, and isolated cutaneous involvement is rarely reported. We report a case of isolated secondary cutaneous involvement from nodal anaplastic large cell lymphoma (CD30 + and ALK + in a 7-year-old boy who was on chemotherapy. This case is reported for its unusual clinical presentation as an acute febrile, generalized papulonodular eruption that mimicked deep fungal infection, with the absence of other foci of systemic metastasis.

  14. Primary Diffuse Large B-Cell Lymphoma of the Liver in a Patient with Sjogren Syndrome

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    Vadim Gorodetskiy

    2016-01-01

    Full Text Available Sjögren’s syndrome (SS has the highest incidence of malignant lymphoproliferative disorders transformation among autoimmune diseases. We present a case of extranodal high grade lymphoma of the liver in a 52-year-old patient with long history of SS. Lymphoma manifested with sharp significant pain in the right hypochondrium, weakness, and profuse night sweats. Contrast-enhanced computed tomography scan (CT-scan of the abdomen revealed multiple low density foci with homogeneous structure and clear contours in both lobes of the liver. Histologically, proliferation of medium sized lymphoma cells with round-oval and slightly irregular nuclei with fine chromatin was shown. Immunohistochemical and molecular features of the tumors allowed diagnosis of diffuse large B-cell lymphoma (DLBCL. To exclude secondary liver lesion by non-Hodgkin lymphoma, chest and small pelvis CT-scan, endoscopy of upper and lower gastrointestinal tract and study of bone marrow were performed. After 8 cycles of R-CHOP chemotherapy (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone, the complete remission was achieved, which persists after 45 months of follow-up. Primary hepatic lymphomas are extremely rare, and previously only low-grade hepatic lymphomas have been described in SS. To our knowledge, the patient described here represents the first reported case of DLBCL with primary liver involvement in SS.

  15. Plasmoblastic lymphoma associated with human immunodeficiency virus.

    Science.gov (United States)

    Horváth, Emoke; Krenács, L; Bagdi, Eniko; Pávai, Z; Macarie, I; Nagy, Elod-Erno; Demian, Smaranda

    2008-01-01

    Plasmoblastic lymphoma (PBL) is a subtype of the diffuse large B-cell lymphoma, typically present as extranodal disease associated with human immune deficiency virus (HIV) infection. PBLs are often the initial manifestation of AIDS. Here we present a case of PBL concerning the oral cavity. A 34-year-old woman presented a tumor in the oral cavity that involved the maxilla and gingiva (confirmed by CT-scan). The gingival biopsy showed a massive infiltration by large lymphoid cells with round, vesicular nuclei, prominent nucleoli, fine chromatin and an significant amount of basophilic cytoplasm which express CD79a, CD138, cytoplasmic lambda light chain and LCA, without staining for CD20, CD38, CD3 and CTK. Serological analysis confirmed HIV positivity. PBLs lack most B-lineage markers, but many express CD79a in at least some of the cells, therefore generate difficulties in differential diagnosis. Overall assessment and correlation of the histopathological and immunohistochemical features with the clinical findings and serology investigation are the most helpful diagnostic tools and can lead to the final diagnosis.

  16. Nodal marginal zone B-cell lymphoma with monocional IgM:2 cases report and review of literatures%结内边缘带B细胞淋巴瘤伴有单克隆IgM增高两例并文献复习

    Institute of Scientific and Technical Information of China (English)

    王欢; 周小鸽; 熊梅; 王昭

    2009-01-01

    Objective To elucidate the clinical and pathological features, diagnosis, treatment and prognosis of nodal marginal zone B-cell lymphoma (NMZL). Methods 2 patients, with NMZL who has extremely elevated monoclonal IgM were analyzed and review were included. Results The common pathological diagnosis caused by the association of monoclonal gammopathy with indolent B-cell non-Hodgkin lymphomas was marginal zone lymphoma or B small cell lymphocytic lymphoma/B-cell chronic lymphocytic leukemia. According to our cases and literatures, the main distribution of involved sites in NMZL were lymph nodes, spleen and bone marrow. Sixty percent of the NMZL patients presented with advanced stage with, high risk of IPI. The diagnosis of NMZL was based on pathology of the lymph node. The proliferation and distribution of the differentiation monocytoid cell had a specified significance. Prognosis of early stage ( I or Ⅱ) NMZL were much better than that of advanced stage NMZL even the patients had received chemotherapy.Conclusion NMZL mainly involved lymph nodes, bone marrow and spleen, sporadically associated with the elevated monoclonal IgM. Most of the NMZL patients presented with advanced stage.The prognoses were poor.%目的 分析结内边缘带B细胞淋巴瘤(NMZL)伴有单克隆lgM增高病例的临床及病理特点、诊断、治疗及预后.方法 对2例NMZL病例进行分析并进行文献综述.结果 惰性B细胞淋巴瘤伴有单克隆免疫球蛋白升高,最常见的两种病理类型为NMZL和B-SLL/CLL.NMZL主要累及淋巴结、脾脏、骨髓,60%发病时为进展期,国际预后指数(IPI)评分为高危,诊断主要依据淋巴结病理,单核样B细胞增生及分布有重要的参考价值.Ⅰ和(或)Ⅱ期化疗疗效好,进展期疗效差.结论 NMZL主要累积淋巴结、脾脏、骨髓,町伴有单克隆IgM增多,发病时多处于疾病进展期,预后不佳.

  17. Infectious agents as causes of non-Hodgkin lymphoma.

    Science.gov (United States)

    Engels, Eric A

    2007-03-01

    Among exposures presently viewed as possible etiologic factors in non-Hodgkin lymphoma (NHL), infections are close to being regarded as established causes. Infectious agents causing NHL can be classified, according to mechanism, into three broad groups. First, some viruses can directly transform lymphocytes. Lymphocyte-transforming viruses include Epstein Barr virus (linked to Burkitt's lymphoma, NHLs in immunosuppressed individuals, and extranodal natural killer/T-cell NHL), human herpesvirus 8 (primary effusion lymphoma), and human T lymphotropic virus type I (adult T-cell leukemia/lymphoma). Second, human immunodeficiency virus is unique in causing profound depletion of CD4+ T lymphocytes, leading to acquired immunodeficiency syndrome and an associated high risk for some NHL subtypes. Third, recent evidence suggests that some infections increase NHL risk through chronic immune stimulation. These infections include hepatitis C virus as well as certain bacteria that cause chronic site-specific inflammation and seem to increase risk for localized mucosa-associated lymphoid tissue NHLs. Establishing that an infectious agent causes NHL depends on showing that the agent is present in persons with NHL as well as laboratory experiments elucidating the mechanisms involved. Only epidemiologic studies can provide evidence that infection is actually a risk factor by showing that infection is more frequent in NHL cases than in controls. Given the range of mechanisms by which infections could plausibly cause NHL and our growing molecular understanding of this malignancy, this field of research deserves continued attention.

  18. NON-HODGKIN'S LYMPHOMAS OF FEMALE REPRODUCTIVE SYSTEM

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    A. V. Babkina

    2008-01-01

    Full Text Available Non-Hodgkin's lymphomas are extremely rare among all tumors of female reproductive system. Diagnostic mistakes and inadequate therapeu- tic tactics in these diseases are results of usual absence of alertness of gynecologists. The aims are to analyze reasons of diagnostic mistakes in patients with non-Hodgkin's lymphomas of female reproductive system and to discover definitive clinical and morphological characteristics of female reproductive system lymphoid tumors. During the period between 1989 and 2006, 305 cases of primary extranodal non-Hodgkin's lym- phomas were detected; female reproductive system was affected in 7% of patients (totally 40 patients, which were included in investigated group. In the whole analyzed group of women (n=40, median age 43 yrs, range 17-84 yrs, patients with primary lesion of female reproductive system had median age of 40 yrs and with secondary involvement - 46 yrs. Most of patients were fertile (60%, n=24. Such tumors was localized in breast in 40% of cases (n=16, in ovaries - 20% (n=8, in uterine corpus - 12,5% (n=5, in uterine cervix - 15% (n=6, and in vagina - remaining 12,5% (n=5. Average time from diagnosis to beginning of the treatment was 7,5 months. As a result, the onset of specific therapy was delayed in 65% cases (n=26 and 50% (n=20 underwent unneeded surgery. Diagnostic mistakes lead to inadequate treatment. Extranodal non-Hodgkin’s lymphomas of female reproductive system, both primary and secondary, are rare pathology. Primary lesion is more typical for older women, sec- ondary is mainly affecting younger women (in reproductive period. Chemotherapy response and prognosis are better in primary cases.

  19. Clinical analysis of voriconazole in treatment of invasive fungal pneumonia in marginal zone B-cell lymphoma%伏立康唑治疗边缘带B细胞淋巴瘤合并侵袭性真菌性肺炎的临床分析

    Institute of Scientific and Technical Information of China (English)

    刘景华; 周凡; 张晓琳; 李敏燕; 刘彦琴; 王吉刚

    2011-01-01

    目的 探讨伏立康唑在边缘带B细胞淋巴瘤应用氟达拉滨治疗过程中并发侵袭性真菌性肺炎的疗效.方法 回顾性分析边缘带B细胞淋巴瘤患者在应用氟达拉滨治疗过程中并发侵袭性真菌性肺炎,比较应用伏立康唑抗真菌治疗前后患者症状、血气分析、肺部影像学、真菌表面抗原等的改变.结果 氟达拉滨为基础方案化疗两个疗程后,出现侵袭性真菌性肺炎,其病程呈延缓性,在发病1个月后开始给予伏立康唑抗真菌治疗,治疗3d后临床症状改善,1周后血气分析改善,约2周真菌表面抗原滴度降低,约5周肺部炎性病变大部吸收.结论 伏立康唑在边缘带B细胞淋巴瘤应用氟达拉滨治疗过程中并发侵袭性真菌性肺炎中的疗效是确切的.%OBJECTIVE To investigate the clinical effect of voriconazole in treatment invasive fungal pneumonia patients with in marginal zone B-cell lymphoma. METHODS Retrospective analysis the change of symptoms, blood gas analysis, lung imaging and fungal surface antigens titer before and after voriconazole treatment invasive fungal pneumonia in marginal zone B-cell lymphoma. RESULTS Invasive fungal pneumonia appeared after two circles of fludarabine-based chemotherapy. Disease progress of invasive fungal pneumonia was slowly, voriconazole treatment invasive fungal pneumonia was started 1 month after onset. Clinical symptoms was improved after 3 days, blood gas analysis was improved after 1 week, fungal surface antigen titer was reduced after about 2 weeks, and lung imaging was obviously improved after about 5 weeks voriconazole treatment invasive fungal pneumonia. CONCLUSION The clinical effect of voriconazole treatment invasive fungal pneumonia in marginal zone B-cell lymphoma is exact.

  20. Radiotherapy in the treatment of localised non-Hogkin's lymphoma (report no. 16)

    Energy Technology Data Exchange (ETDEWEB)

    Phillips, D.L. (Middlesex Hospital, London (UK))

    1981-09-01

    This report describes the experience of the British National Lymphoma Investigation (BNLI) in the treatment of localised (Stages I, II) non-Hodgkin's lymphoma (NHL). About a third of all patients with NHL presenting to collaborators in the BNLI were considered after investigation to have localised disease. This relatively large number suggests that some cases may have been understaged. Patients with Grade I disease were treated with either local irradiation or with local irradiation followed by chlorambucil. Survival rates in the two groups were essentially similar and it appeared that radiotherapy alone provided equally effective treatment. Patients with Grade 2 lymphomas were treated either with local irradiation or with local irradiation followed by COP. Primary nodal disease was not obviously affected by the addition of chemotherapy. Extranodal disease arising in the gut below the diaphragm appeared to be adversely affected by the irradiation which was of necessity spread over several weeks, delaying the use of chemotherapy. Extranodal disease arising in the head and neck region is controlled in about 80% of patients and the routine use of prophylactic combination chemotherapy confers no benefit to the patient.

  1. [Plasmablastic lymphoma].

    Science.gov (United States)

    Fernández-Álvarez, Rubén; Sancho, Juan-Manuel; Ribera, Josep-María

    2016-11-04

    Plasmablastic lymphoma (PBL) is a rare and aggressive subtype of non-Hodgkin lymphoma that commonly occurs in human immunodeficiency virus (HIV)-positive individuals, and affects oral sites. Occasionally, it has been described in HIV-negative patients and involving non-oral sites. Pathologically, PBL is a high-grade B-cell lymphoma that displays the immunophenotype of a terminally differentiated B-lymphocyte with loss of B-cell markers (CD20) and expression of plasma-cell antigens. Epstein-Barr virus infection and MYC rearrangements are frequently observed. Treatment of PBL is challenging because of the lack of established treatment and poor outcomes, with median survival times shorter than one year. In this review, we discuss the clinical and epidemiologic spectrum of PBL as well as its distinct pathological features. Finally, we summarize the currently available approaches for the treatment of patients with PBL. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

  2. Combination therapy with brentuximab vedotin and cisplatin/cytarabine in a patient with primarily refractory anaplastic lymphoma kinase positive anaplastic large cell lymphoma

    Directory of Open Access Journals (Sweden)

    Heidegger S

    2014-06-01

    Full Text Available Simon Heidegger,1 Ambros Beer,2 Eva Geissinger,3 Andreas Rosenwald,3 Christian Peschel,1 Ingo Ringshausen,1 Ulrich Keller11III Medical Department, 2Nuclear Medicine Department, Technische Universität München, Munich, Germany; 3Institute of Pathology, University of Würzburg, Würzburg, GermanyAbstract: Anaplastic large cell lymphoma (ALCL is a common subtype of the heterogeneous group of peripheral T-cell lymphomas, which is characterized by large pleomorphic cells with strong expression of CD30. Translocations involving ALK, the anaplastic lymphoma kinase gene, are associated with a favorable clinical outcome. Such ALK-positive ALCLs are usually responsive to a multidrug chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone. However, there is no general consensus on the optimal therapy for relapsed or refractory ALCL. We report the case of a 24-year-old male suffering from ALK-positive ALCL with an uncommon manifestation of only extranodal disease in the gastric cardia region that showed primary refractoriness to standard CHOP chemotherapy. A combination therapy consisting of the anti-CD30 drug conjugate, brentuximab vedotin, and classical lymphoma salvage regimen DHAP (cisplatin, high-dose cytarabine and dexamethasone was administered. Following two treatment cycles in 21-day intervals, the lymphoma showed considerable regression based on imaging diagnostics and no evidence of vital lymphoma in a subsequent biopsy. We did not observe any increase in toxicity; in particular, polyneuropathy and febrile neutropenia were not observed. In summary, we report that the antibody-drug conjugate brentuximab vedotin and a classical regimen used for aggressive lymphoma, DHAP, could be combined as salvage therapy in a case of refractory ALK-positive ALCL. Phase I/II studies will be required for safety and efficacy analysis.Keywords: anaplastic large cell lymphoma (ALCL, refractory/relapsed lymphoma, anti-CD30 drug conjugate, DHAP

  3. Expression of MALT1 oncogene in hematopoietic stem/progenitor cells recapitulates the pathogenesis of human lymphoma in mice.

    Science.gov (United States)

    Vicente-Dueñas, Carolina; Fontán, Lorena; Gonzalez-Herrero, Ines; Romero-Camarero, Isabel; Segura, Victor; Aznar, M Angela; Alonso-Escudero, Esther; Campos-Sanchez, Elena; Ruiz-Roca, Lucía; Barajas-Diego, Marcos; Sagardoy, Ainara; Martinez-Ferrandis, Jose I; Abollo-Jimenez, Fernando; Bertolo, Cristina; Peñuelas, Ivan; Garcia-Criado, Francisco J; García-Cenador, María B; Tousseyn, Thomas; Agirre, Xabier; Prosper, Felipe; Garcia-Bragado, Federico; McPhail, Ellen D; Lossos, Izidore S; Du, Ming-Qing; Flores, Teresa; Hernandez-Rivas, Jesus M; Gonzalez, Marcos; Salar, Antonio; Bellosillo, Beatriz; Conde, Eulogio; Siebert, Reiner; Sagaert, Xavier; Cobaleda, Cesar; Sanchez-Garcia, Isidro; Martinez-Climent, Jose A

    2012-06-26

    Chromosomal translocations involving the MALT1 gene are hallmarks of mucosa-associated lymphoid tissue (MALT) lymphoma. To date, targeting these translocations to mouse B cells has failed to reproduce human disease. Here, we induced MALT1 expression in mouse Sca1(+)Lin(-) hematopoietic stem/progenitor cells, which showed NF-κB activation and early lymphoid priming, being selectively skewed toward B-cell differentiation. These cells accumulated in extranodal tissues and gave rise to clonal tumors recapitulating the principal clinical, biological, and molecular genetic features of MALT lymphoma. Deletion of p53 gene accelerated tumor onset and induced transformation of MALT lymphoma to activated B-cell diffuse large-cell lymphoma (ABC-DLBCL). Treatment of MALT1-induced lymphomas with a specific inhibitor of MALT1 proteolytic activity decreased cell viability, indicating that endogenous Malt1 signaling was required for tumor cell survival. Our study shows that human-like lymphomas can be modeled in mice by targeting MALT1 expression to hematopoietic stem/progenitor cells, demonstrating the oncogenic role of MALT1 in lymphomagenesis. Furthermore, this work establishes a molecular link between MALT lymphoma and ABC-DLBCL, and provides mouse models to test MALT1 inhibitors. Finally, our results suggest that hematopoietic stem/progenitor cells may be involved in the pathogenesis of human mature B-cell lymphomas.

  4. Prolonged or Standard Infusion of Cefepime Hydrochloride in Treating Patients With Febrile Neutropenia

    Science.gov (United States)

    2013-07-10

    Adult Acute Lymphoblastic Leukemia; Adult Acute Myeloid Leukemia; Adult Burkitt Lymphoma; Adult Diffuse Large Cell Lymphoma; Adult Diffuse Mixed Cell Lymphoma; Adult Diffuse Small Cleaved Cell Lymphoma; Adult Hodgkin Lymphoma; Adult Immunoblastic Large Cell Lymphoma; Adult Lymphoblastic Lymphoma; Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative; Breast Cancer; Chronic Eosinophilic Leukemia; Chronic Lymphocytic Leukemia; Chronic Myelogenous Leukemia; Chronic Myelomonocytic Leukemia; Chronic Neutrophilic Leukemia; Cutaneous T-cell Non-Hodgkin Lymphoma; Disseminated Neuroblastoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Grade 1 Follicular Lymphoma; Grade 2 Follicular Lymphoma; Grade 3 Follicular Lymphoma; Malignant Testicular Germ Cell Tumor; Mantle Cell Lymphoma; Marginal Zone Lymphoma; Multiple Myeloma; Mycosis Fungoides/Sezary Syndrome; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Neutropenia; Nodal Marginal Zone B-cell Lymphoma; Ovarian Epithelial Cancer; Ovarian Germ Cell Tumor; Plasma Cell Neoplasm; Poor Prognosis Metastatic Gestational Trophoblastic Tumor; Primary Myelofibrosis; Prolymphocytic Leukemia; Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma

  5. Report on recent advances on marginal zone lymphoma in the 54th ASH annual meeting%边缘区淋巴瘤的多面世界和研究进展:从2012年美国血液学会年会报道说起

    Institute of Scientific and Technical Information of China (English)

    李增军; 邱录贵

    2013-01-01

    边缘区淋巴瘤分为三类,它们在常见累及部位、病因、发病机制、分子细胞遗传学特点及预后等多方面存在差异.以2012年美国血液学会(ASH)年会关于边缘区淋巴瘤的报道为契机,参考会议摘要和近年的相关研究,介绍目前对该病三种亚型的认识和在病因、发病机制及诊断、鉴别诊断方面的研究进展.%Marginal zone lymphomas include three subgroups of lymphoma,with differences on etiology,pathogenesis,molecular cytogenetics,prognosis,as well as involved sites.Based on the reports from the latest ASH annual meeting,the present article introduces the uptodate understanding on the three subtypes of MZL.The recent advances on etioloty,pathogenesis,diagnosis and differential diagnosis were reviewed.

  6. Testicular lymphoma

    DEFF Research Database (Denmark)

    Møller, Michael Boe; d'Amore, F; Christensen, Bjarne Egelund

    1994-01-01

    In a Danish population-based non-Hodgkin's lymphoma registry, 2687 newly diagnosed patients were registered from 1983 to 1992. 39 had testicular involvement (TL) (incidence 0.26/10(5)/year). Median age was 71 years. 24 cases had localised and 15 had disseminated disease. Histologically, all cases...... were diffuse (65% diffuse centroblastic type). Of the 27 tested, 11% were of T- and 89% of B-immunophenotype. In localised cases, where surgery was supplemented by combination chemotherapy (CCT), the relapse rate was 15.4%. The relapse rate for cases with localised disease treated with other regimens...

  7. Chimeric antigen receptor T-cell therapies for lymphoma.

    Science.gov (United States)

    Brudno, Jennifer N; Kochenderfer, James N

    2017-08-31

    New therapies are needed for patients with Hodgkin or non-Hodgkin lymphomas that are resistant to standard therapies. Indeed, unresponsiveness to standard chemotherapy and relapse after autologous stem-cell transplantation are indicators of an especially poor prognosis. Chimeric antigen receptor (CAR) T cells are emerging as a novel treatment modality for these patients. Clinical trial data have demonstrated the potent activity of anti-CD19 CAR T cells against multiple subtypes of B-cell lymphoma, including diffuse large-B-cell lymphoma (DLBCL), follicular lymphoma, mantle-cell lymphoma, and marginal-zone lymphoma. Importantly, anti-CD19 CAR T cells have impressive activity against chemotherapy-refractory lymphoma, inducing durable complete remissions lasting >2 years in some patients with refractory DLBCL. CAR-T-cell therapies are, however, associated with potentially fatal toxicities, including cytokine-release syndrome and neurological toxicities. CAR T cells with novel target antigens, including CD20, CD22, and κ-light chain for B-cell lymphomas, and CD30 for Hodgkin and T-cell lymphomas, are currently being investigated in clinical trials. Centrally manufactured CAR T cells are also being tested in industry-sponsored multicentre clinical trials, and will probably soon become a standard therapy. Herein, we review the clinical efficacy and toxicity of CAR-T-cell therapies for lymphoma, and discuss their limitations and future directions with regard to toxicity management, CAR designs and CAR-T-cell phenotypes, conditioning regimens, and combination therapies.

  8. [Research progress on the etiology and pathogenesis of MALT lymphoma].

    Science.gov (United States)

    Wang, Xiao-Can; Ke, Xiao-Yan

    2012-12-01

    Mucosa-associated lymphoid tissue (MALT) lymphoma originated outside the lymph nodes is low grade malignant B cell lymphoma. It is the most frequent type of marginal zone non-Hodgkin's lymphoma, that usually occurs in the stomach, salivary gland, thyroid gland and orbital adnexa. Gastric MALT lymphoma accounts for 50% of MALT lymphoma. Gastric MALT lymphoma has been confirmed to relate with Helicobacter pylori (HP) infection, its main pathogenesis is immune reaction, but some patients with chromosome translocation have no response to HP eradication, suggesting presence of other unknown pathogenesis. The chromosome translocations in MALT lymphoma are t(11;18)(q21;q21), t(1;14)(p22;q32), t(14;18)(q32;q21), t(3;14)(p14.1;q32). Recent studies show some new chromosomal abnormalities such as 6q23.3/A20 and so on, which have some effects on clinical course and prognosis. MALT lymphoma with chromosome abnormalities usually activate common NF-κB molecular pathway, and persistent active NF-κB pathway drives tumor cell proliferative and active, resulting in lymphoma incidence. In this article, the advances in the etiology and pathogenesis of MALT lymphoma were reviewed.

  9. [Meta-analysis of relationship between extranodal tumor deposits and prognosis in patients with colorectal cancer].

    Science.gov (United States)

    Zhang, Xianxiang; Shao, Shihong; Gao, Yuan; Zhang, Maoshen; Lu, Yun

    2016-03-01

    To investigate the relationship between extranodal tumor deposits and prognosis in patients with colorectal cancer. The literatures on extranodal tumor deposits and postoperative survival rate in patients with colorectal cancer published at home and abroad from 1990 to 2014 were retrieved in 15 English literature databases such as MEDLINE/PubMed, Web of Science, Directory of Open Access Journals(DOAJ), SpringerLink and Chinese literature databases such as Chinese Biomedical Literature Database CD-ROM, China National Knowledge Infrastructure (CNKI) Database with the internet platform of Yonsei University Library. After screening for inclusion, data extraction and quality assessment, meta-analysis was conducted by the Review Manager 5.3 software. There were 10 studies meeting the inclusion criteria for meta-analysis. The total sample size of the studies was 4 068 cases with ENTD(+) 727 cases, while ENTD(-) 3 341 cases. Meta analysis showed that 5-year overall survival rate and 5-year relapse-free survival rate were significantly lower in ENTD(+) group than those in ENTD(-) group (OR 0.27, 0.23; 95% CI:0.18 to 0.43, 0.16 to 0.34 respectively, both P=0.000); the 5-year overall survival rates were both significantly lower in ENTD(+) group as compared to ENTD(-) group for patients with N0 and N(+) colorectal cancer (both P<0.05). Extranodal tumor deposits is a poor prognostic factor of patients with colorectal cancer.

  10. Color Doppler sonography and angioscintigraphy in hepatic Hodgkin's lymphoma

    Institute of Scientific and Technical Information of China (English)

    Mirjana V Stojkovi(c); Vera M Artiko; Irena B Radoman; Slavko J Kne(z)evi(c); Snezana M Luki(c); Mirko D Kerkez; Nebojsa S Leki(c); Andrija A Anti(c); Marinko M (Z)vela; Vitomir I Rankovi(c); Milorad N Petrovi(c); Dragana P (S)obi(c); Vladimir B Obradovi(c)

    2009-01-01

    AIM: To estimate the characteristics of Color Doppler findings and the results of hepatic radionuclide angiography (HRA) in secondary Hodgkin's hepatic lymphoma.METHODS: The research included patients with a diagnosis of Hodgkin's lymphoma with metastatic focal lesions in the liver and controls. Morphologic characteristics of focal liver lesions and hemodynamic parameters were examined by pulsed and Color Doppler in the portal, hepatic and splenic veins were examined. Hepatic perfusion index (HPI) estimated by HRA was calculated.RESULTS: In the majority of patients, hepatomegaly was observed. Lesions were mostly hypoechoic and mixed, solitary or multiple. Some of the patients presented with dilated splenic veins and hepatofugal blood flow. A pulse wave was registered in the centre and at the margins of lymphoma. The average velocity of the pulse wave was higher at the margins ( P >0.05). A continuous venous wave was found only at the margins of lymphoma. There was no linear correlation between lymphoma size and velocity of pulse and continuous wave ( r = 390, P < 0.01). HPI was significantly lower in patients with lymphomas than in controls ( P < 0.05), pointing out increased arterial perfusion in comparison to portal perfusion.CONCLUSION: Color Doppler ultrasonography is a sensitive method for the detection of neovascularization in Hodgkin's hepatic lymphoma and estimation of its intensity. Hepatic radionuclide angiography can additionally help in the assesment of vascularisation of liver lesions.

  11. Stages of Adult Hodgkin Lymphoma

    Science.gov (United States)

    ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ...

  12. Stages of Childhood Hodgkin Lymphoma

    Science.gov (United States)

    ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ...

  13. Infected primary non-Hodgkin lymphoma of spine

    Directory of Open Access Journals (Sweden)

    Che-Wei Liu

    2012-01-01

    Full Text Available Primary bone lymphoma (PBL comprises less than 5% of all malignant bone tumors and almost 7% of all extranodal lymphomas. Only 1.7% of all PBLs have been reported to involve the vertebrae. In our case, osteomyelitis was accidentally found during surgery, which might have resulted in the rapid collapse of vertebral body. This is the first report on primary lymphoma of the vertebrae with superimposed osteomyelitis in the English literature to the best of our knowledge. The patient reported here received anterior vertebrectomy and posterior interbody fusion with instrumentation for spinal instability. Tumor mass and the necrotic debris were removed. After the procedure, the patient received treatment with antibiotics and six cycles of chemotherapy. This case reminds us the possibility of hematologic seeding of bacteria in the tissue, especially with tumor necrosis. We suggest percutaneous needle aspiration for pathology and culture before making a decision whether or not to proceed with surgical decompression for fear of missing the occult bacterial infection.

  14. Double-hit primary unilateral adrenal lymphoma with good outcome

    Directory of Open Access Journals (Sweden)

    Marković Olivera

    2014-01-01

    Full Text Available Introduction. Primary adrenal non-Hodgkin’s lymphoma (NHL is a rare neoplasm with poor prognosis. On the other side, double-hit lymphomas with BCL2 and MYC translocation are characterized by advanced disease stage, extranodal and central nervous system involvements at presentation or disease progression. Case report. We reported a 73-year-old male patient with double-hit primary adrenal lymphoma and preserved adrenal function, showing a favorable clinical course. Computed tomography of abdomen showed a 9 7 cm mass of the left adrenal gland. Laparatomy with left adrenalectomy was done and histological examination revealed diagnosis of a diffuse large B-cell NHL (DLBCL, non-GCB subtype. The patient was treated with 6 cycles of R-CHOP chemotherapy with reduced doses of doxorubicin because of the decreased left verticle ejection fraction. The patient was followed up regularly for 20 months with no evidence of tumor recurrence despite the inherently poor prognostic profile and double-hit phenotype of the disease. Conclusion. R-CHOP chemotherapy in combination with adrenalectomy can be an effective first-line regimen for primary adrenal DLBCL, despite the inherently poor prognostic profile (non-GCB subtype, bulky disease, elevated lactate dehydrogenase and double-hit phenotype of the disease.

  15. A case of Primary Bone Anaplastic Large Cell Lymphoma

    Science.gov (United States)

    Kim, Kyung Hyun; Jung, Yun Hwa; Han, Chi Wha; Woo, In Sook; Son, Jong ho

    2016-01-01

    Patient: Female, 52 Final Diagnosis: Primary bone anaplastic large cell lymphoma Symptoms: Bone pain Medication: — Clinical Procedure: — Specialty: Oncology Objective: Unusual clinical course Background: Anaplastic large cell lymphoma (ALCL) is a relatively rare subtype of non-Hodgkin’s lymphoma (NHL). Like other types of NHL, ALCL primarily involves the nodal area, and sometimes it can involve several extra-nodal sites such as skin, soft tissue, and lungs. However, extensive bone involvement in cases of ALCL is very rare whether it is primary or secondary. Without nodular involvement, ALCL can be misdiagnosed as bone tumor or metastatic carcinoma such as lung, breast, or prostate cancer, which frequently spread to bone. Case Report: A 52-year-old woman with generalized pain and 2 months of fever of unknown origin presented to our institution. After extensive evaluation, only multiple osteolytic bone lesions with periosteal soft tissue reaction were identified. Repeated core needle biopsy revealed only inflammatory cells with histiocytic reactions. After pathologic and chromosomal analysis of sufficient tissue, which was acquired from incisional biopsy, primary bone ALCL was confirmed. Conclusions: Clinicians should keep in mind that ALCL can present with extensive bone involvement without nodal involvement. PMID:27729639

  16. The pleural sandwich sign in two cases of primary pleural lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Yoo Kyung; Lee, Myung Jae; Ryu, Yon Ju; Cho, Min Sun [School of Medicine, Ewha Womans University, Seoul (Korea, Republic of)

    2015-02-15

    The sandwich sign is used to describe mesenteric lymphoma in which mesenteric vessels and fat are enveloped by enlarged mesenteric lymph nodes. We present two cases of primary pleural lymphoma demonstrating the 'pleural sandwich sign'. Contrast-enhanced computed tomography showed conglomerated parietal pleural and extrapleural masses encasing the intercostal arteries. Histopathological examinations confirmed low grade marginal zone B-cell lymphoma in an 80-year-old man and diffuse large B-cell lymphoma in a 68-year-old man. The pleural sandwich sign may suggest the diagnosis of primary pleural lymphoma.

  17. Primary parotid gland lymphoma: a case report

    Directory of Open Access Journals (Sweden)

    Paraskevas Katsaronis

    2011-08-01

    Full Text Available Abstract Introduction Mucosa associated lymphoid tissue lymphomas are the most common lymphomas of the salivary glands. The benign lymphoepithelial lesion is also a lymphoproliferative disease that develops in the parotid gland. In the present case report, we describe one case of benign lymphoepithelial lesion with a subsequent low transformation to grade mucosa associated lymphoid tissue lymphoma appearing as a cystic mass in the parotid gland. Case presentation A 78-year-old Caucasian female smoker was referred to our clinic with a non-tender left facial swelling that had been present for approximately three years. The patient underwent resection of the left parotid gland with preservation of the left facial nerve through a preauricular incision. The pathology report was consistent with a low-grade marginal-zone B-cell non-Hodgkin lymphoma (mucosa associated lymphoid tissue lymphoma following benign lymphoepithelial lesion of the gland. Conclusions Salivary gland mucosa associated lymphoid tissue lymphoma should be considered in the differential diagnosis of cystic or bilateral salivary gland lesions. Parotidectomy is recommended in order to treat the tumor and to ensure histological diagnosis for further follow-up planning. Radiotherapy and chemotherapy should be considered in association with surgery in disseminated forms or after removal.

  18. Treatment Options for Non-Hodgkin Lymphoma

    Science.gov (United States)

    ... Lymphoma Treatment AIDS-Related Lymphoma Treatment Chronic Lymphocytic Leukemia Treatment (small lymphocytic lymphoma) Mycosis Fungoides (Including Sézary Syndrome) Treatment (cutaneous T-cell lymphoma) Primary CNS Lymphoma Treatment Non-Hodgkin lymphoma ...

  19. Stages of Adult Non-Hodgkin Lymphoma

    Science.gov (United States)

    ... Lymphoma Treatment AIDS-Related Lymphoma Treatment Chronic Lymphocytic Leukemia Treatment (small lymphocytic lymphoma) Mycosis Fungoides (Including Sézary Syndrome) Treatment (cutaneous T-cell lymphoma) Primary CNS Lymphoma Treatment Non-Hodgkin lymphoma ...

  20. Peripheral T-Cell Lymphoma

    Science.gov (United States)

    Getting the Facts Peripheral T-Cell Lymphoma Overview Lymphoma is the most common blood cancer. The two main forms of lymphoma are Hodgkin lymphoma and ... develop into lymphomas: B-lymphocytes (B-cells) and T-lymphocytes (T-cells). Peripheral T-cell lymphoma (PTCL) ...

  1. Mantle Cell Lymphoma of Intestine Presenting as Multiple Lymphomatous Polyposis with Intussusception

    Directory of Open Access Journals (Sweden)

    Meena N. Jadhav

    2015-01-01

    Full Text Available Mantle Cell Lymphoma (MCL is a distinct clinicopathological subtype of B-cell non-Hodgkin's lymphoma (NHL accounting for 2-10% of all NHL cases. Gastrointestinal tract (GIT is the predominant site of extranodal MCL which commonly presents as Multiple Lymphomatous Polyposis (MLP. A 60 year old male presented with pain abdomen, diarrhea and weight loss of two months duration. On colonoscopy multiple polyps were found in the entire colon and rectum. Computed tomography revealed ileo-colic intussusception with nodularity in the lead point. Histopathology suggested features of MCL. On immunohistochemistry, the tumor cells were positive for CD20, CD5, Cyclin D1, negative for CD3, CD10, CD23, and CD45 RO

  2. AN UNUSUAL CASE OF PRIMARY BILATERAL RENAL LYMPHOMA: A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Mitul

    2014-08-01

    Full Text Available Primary renal lymphoma (PRL is rare. They are variants of extranodal non-Hodgkins lymphoma. Lymphomatous involvement of the kidney is often seen as a part of disseminated disease. PRL can mimic other renal lesions such as renal cell carcinoma, renal abscess, and metastasis and may have aggressive clinical course. Pathological data are scanty and prognosis is usually poor with median survival less than a year. We present a rare case of a 22 year-old male who presented with acute febrile illness, respiratory problems and acute kidney injury. The clinical diagnosis was missed partly due to absence of obvious renal symptoms like renal mass lesion, gross hematuria or abdominal pain. However, radiological investigations combined with renal histology helped in establishing the diagnosis in the present case

  3. Prognostic Assessment in Patients with Indolent B-Cell Lymphomas

    Directory of Open Access Journals (Sweden)

    Luca Arcaini

    2012-01-01

    Full Text Available Follicular lymphoma (FL is an indolent lymphoma with long median survival. Many studies have been performed to build up prognostic scores potentially useful to identify patients with poorer outcome. In 2004, an international consortium coordinated by the International Follicular Lymphoma Prognostic Factor project was established and a new prognostic study was launched (FLIPI2 using progression-free survival (PFS as main endpoint and integrating all the modern parameters prospectively collected. Low-grade non-Hodgkin lymphomas were once considered as a heterogenous group of lymphomas characterized by an indolent clinical course. Each entity is characterized by unique clinicobiologic features. Some studies have been focused on prognostic factors in single lymphoma subtypes, with the development of specific-entity scores based on retrospective series, for instance splenic marginal zone lymphoma (SMZL. A widely accepted prognostic tool for clinical usage for indolent non-follicular B-cell lymphomas is largely awaited. In this paper we summarized the current evidence regarding prognostic assessment of indolent follicular and non-follicular lymphomas.

  4. Radiographic enlargement of mandibular canal as first feature of non-Hodgkin's lymphoma.

    Science.gov (United States)

    Buric, N; Jovanovic, G; Radovanovic, Z; Buric, M; Tijanic, M

    2010-09-01

    Non-Hodgkin's lymphoma has the propensity to affect non-lymphoid tissue including oral tissue. Primary non-Hodgkin's lymphoma of the mandible mistreated as chronic periodontitis with diffuse enlargement of the mandibular canal and ice-cold numbness is very rarely described in English medical literature. A 57-year-old patient presented with a painful swelling on the left side of the mandible with a clinically chronic periodontitis associated with ice-cold numbness. A panoramic radiograph showed a diffuse uniform enlargement of the mandibular canal. Histological examination showed that the lesion was a primary intraosseous non-Hodgkin's lymphoma of the mandible. Immunohistochemical examination showed a positive reaction for CD20+, Ki-67+. Seven months after chemotherapy the patient was observed for possible life-threatening propagation of the disease. In conclusion, primary (extra-nodal) non-Hodgkin's lymphoma of the mandible usually clinically presents with bone swelling, teeth mobility and neurological disturbance. Radiographic features presenting as diffuse enlargement of the mandibular canal could be considered as non-Hodgkin's lymphoma.

  5. Radiation therapy for early stage unfavorable Hodgkin lymphoma: is dose reduction feasible?

    Science.gov (United States)

    Laskar, Siddhartha; Kumar, Deepak P; Khanna, Nehal; Menon, Hari; Sengar, Manju; Arora, Brijesh; Gujral, Sumeet; Shet, Tanuja; Sridhar, Epari; Rangarajan, Venkatesh; Muckaden, Mary Ann; Nair, Reena; Banavali, Shripad

    2014-10-01

    One hundred and fifty-one patients aged between 3 and 70 years with early stage unfavorable Hodgkin lymphoma were included. Patients received 4-6 cycles of ABVD (doxorubicin, bleomycin, vinblastine and dacarbazine) chemotherapy and involved field radiation therapy (IFRT). The most common histology was mixed cellularity (43%). The majority had stage IIAX disease. IFRT doses were 25.2 Gy/14 fractions and 34.2 Gy/19 fractions for adults with a complete response (CR) and partial response (PR), respectively, while the doses were 19.8 Gy/11 fractions and 30.6 Gy/17 fractions, respectively, for children. After 60 months (median), the 10-year progression-free survival (PFS) and overall survival (OS) were 88.4% and 93.2%, respectively. On univariate analysis, prognostic factors with significant impact on PFS were age ≥ 18 years, nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) histology, extranodal disease and response to treatment. Extranodal disease had a significant impact on OS. On multivariate analysis, NLPHL histology (p = 0.001) and response at 3 months (p = 0.000) had a significant impact on PFS. There were no in-field relapses in patients with bulky disease receiving RT doses > 25.2 Gy. Chemotherapy related acute pulmonary toxicity was documented in 21.4% and 4.8% of patients after six and four cycles of ABVD chemotherapy (p = 0.041). Four cycles of ABVD and reduced dose IFRT resulted in optimal outcomes.

  6. A Case of Primary Central Nervous System Lymphoma Located at Brain Stem in a Child

    Science.gov (United States)

    Kim, Jinho

    2016-01-01

    Primary central nervous system lymphoma (PCNSL) is an extranodal Non-Hodgkin's lymphoma that is confined to the brain, eyes, and/or leptomeninges without evidence of a systemic primary tumor. Although the tumor can affect all age groups, it is rare in childhood; thus, its incidence and prognosis in children have not been well defined and the best treatment strategy remains unclear. A nine-year old presented at our department with complaints of diplopia, dizziness, dysarthria, and right side hemiparesis. Magnetic resonance image suggested a diffuse brain stem glioma with infiltration into the right cerebellar peduncle. The patient was surgically treated by craniotomy and frameless stereotactic-guided biopsy, and unexpectedly, the histopathology of the mass was consistent with diffuse large B cell lymphoma, and immunohistochemical staining revealed positivity for CD20 and CD79a. Accordingly, we performed a staging work-up for systemic lymphoma, but no evidence of lymphoma elsewhere in the body was obtained. In addition, she had a negative serologic finding for human immunodeficient virus, which confirmed the histopathological diagnosis of PCNSL. She was treated by radiosurgery at 12 Gy and subsequent adjuvant combination chemotherapy based on high dose methotrexate. Unfortunately, 10 months after the tissue-based diagnosis, she succumbed due to an acute hydrocephalic crisis. PMID:27867930

  7. Complete remission of gastric Burkitt's lymphoma after eradication of Helicobacter pylori

    Institute of Scientific and Technical Information of China (English)

    Isabelle Baumgaertner; Christiane Copie-Bergman; Michael Levy; Corinne Haioun; Antoine Charachon; Maryse Baia; Iradj Sobhani; Jean-Charles Delchier

    2009-01-01

    Burkitt's lymphoma is a highly aggressive non- Hodgkin lymphoma, often presenting in extra-nodal sites. It generally has a poor spontaneous outcome and needs aggressive treatment with systemic and intrathecal chemotherapy. Occurrence at the gastric site is rare. We report the case of a 39-year old woman who presented with a prominent ulcerated lesion of the antrum corresponding histologically to a Burkitt's lymphoma associated with Helicobacter pylori ( H pylori) infection. Interphase fluorescence in situ hybridization (FISH) demonstrated c-MYC gene rearrangement in tumour cells without BCL2 or BCL6 gene translocations.Ulcer healing and tumour regression with a complete histological response were obtained 8 wk after H pylor ieradication. In spite of this complete remission, taking into account the high risk of recurrence, the patient received systemic and intrathecal chemotherapy. Two years later, the patient remained in complete remission.This is the first report of a gastric Burkitt's lymphoma responding to H pylori eradication. These findings raise the question of the potential role of H pylori in the pathogenesis of some gastric Burkitt's lymphomas, and show the importance of searching for and eradicating the bacteria in combination with conventional chemotherapy regimens.

  8. Secondary breast lymphoma diagnosed by vacuum-assisted breast biopsy: a case report

    Directory of Open Access Journals (Sweden)

    Zagouri Flora

    2007-10-01

    Full Text Available Abstract Introduction Breast lymphoma, either as a manifestation of primary extranodal disease or as secondary involvement, is a rare malignancy, and its diagnosis, prognosis, and treatment have not been clearly defined. On the other hand, Vacuum-assisted breast biopsy (VABB is a minimally invasive technique with ever-growing use for the diagnosis of mammographically detected, non-palpable breast lesions. Case presentation A symptom-free, 56-year-old woman presented with a non-palpable BI-RADS 4B lesion without microcalcifications. She had a positive family history for breast cancer and a history of atypical ductal hyperplasia in the ipsilateral breast four years ago. She reported having been treated for non-Hodgkin lymphoma 12 years ago. With the suspicion of breast cancer, mammographically guided VABB with 11-gauge probe (on the stereotactic Fisher's table was performed. VABB made the diagnosis of a non-Hodgkin, grade II, B-cell germinal-center lymphoma. VABB yielded enough tissue for immunohistochemistry/WHO classification. Conclusion This is the first case in the literature demonstrating the successful diagnosis of breast lymphoma by VABB, irrespectively of the level of clinical suspicion. It should be stressed that VABB was able to yield enough tissue for WHO classification. In general, lymphoma should never be omitted in the differential diagnosis, since no pathognomonic radiologic findings exist for its diagnosis.

  9. International Lymphoma Epidemiology Consortium

    Science.gov (United States)

    The InterLymph Consortium, or formally the International Consortium of Investigators Working on Non-Hodgkin's Lymphoma Epidemiologic Studies, is an open scientific forum for epidemiologic research in non-Hodgkin's lymphoma.

  10. Non-Hodgkin's Lymphoma

    Science.gov (United States)

    ... These include the lymphatic vessels, tonsils, adenoids, spleen, thymus and bone marrow. Occasionally, non-Hodgkin's lymphoma involves ... understand the possible link between pesticides and the development of non-Hodgkin's lymphoma. Older age. Non-Hodgkin's ...

  11. Primary pancreatic lymphoma: Report of six cases

    Institute of Scientific and Technical Information of China (English)

    Hai Lin; Shu-De Li; Xian-Gui Hu; Zhao-Shen Li

    2006-01-01

    patient is still alive 21 mo, after diagnosis and treatment.CONCLUSION: PPL is a rare form of extranodal lymphoma originating from the pancreatic parenchyma. Clinical and imaging findings are otherwise not specific in the differentiation of pancreatic lymphoma and pancreatic cancer, which deserves attention. EUS-guided fine-needle aspiration (EUS-FNA) of the pancreas requires experienced cytopathologists as well as advanced immunohistochemical assays to obtain a final diagnosis on a small amount of tissue. Surgery and adjuvant chemotherapy or radiotherapy can produce fairly good outcomes.

  12. Ocular Adnexal Follicular Lymphoma

    DEFF Research Database (Denmark)

    Rasmussen, Peter K; Coupland, Sarah E; Finger, Paul T

    2014-01-01

    , and 31 (45%) had stage IIE lymphoma. Patients with disseminated lymphoma had stage IIIE (9 of 19 [47%]) and stage IV (10 of 19 [53%]) disease, whereas patients with a relapse of systemic lymphoma presented with stage IE (8 of 10 [80%]), stage IIE (1 of 10 [10%]), and stage IIIE (1 of 10 [10%]) disease...

  13. Unusual case of Hodgkin lymphoma presenting as soft tissue mass with intracranial metastasis and review of literature

    Directory of Open Access Journals (Sweden)

    Vandana Singh Kushwaha

    2015-01-01

    Full Text Available Hodgkin lymphoma (HL primarily presents as nodal disease and may involve extranodal sites during the progression of the disease. Extra nodal involvement of lung, gastrointestinal tract, testis and thyroid etc., is well-recognized in non-HL but clinically detectable soft tissue involvement is rare and quite exceptional with HL. We report here an unusual case of a young adult male, who presented with a frontal soft tissue mass associated with cervical lymph node. Histopathology supplemented with immunohistochemistry revealed classical HL. Patient did not respond to treatment adequately, and he developed intracranial metastasis during the course of treatment.

  14. Plasmablastic lymphoma

    Science.gov (United States)

    Han, Xiao; Duan, Minghui; Hu, Lixing; Zhou, Daobin; Zhang, Wei

    2017-01-01

    Abstract Background: Plasmablastic lymphoma (PBL) is a B-cell malignancy associated with human immunodeficiency virus (HIV). PBL could also influence the HIV-negative patients. The study aimed to identify prognostic factors for survival among Chinese PBL patients. Materials and methods: Eligible patients from literature and Peking Union Medical College Hospital (PUMCH) were included in this study. Clinical characteristics and immunophenotypic data were extracted. Kaplan–Meier curve was used to describe the survival status. Cox regression was used for multivariate analysis. Results: A total of 60 Chinese PBL patients were included, including 54 patients from 36 published articles and 6 new patients that have not been reported. The median overall survival was 7 months (95% confidence interval 3.853–10.147 months). An overwhelming majority (79.31%) of the included cases were Ann Arbor stage IV patients. All the Chinese PBL patients were HIV-negative; 46.81% were Epstein-Barr virus-positive. CD38, CD138, or MUM1 was positively expressed in more than 80% of patients; CD20 expression was also found in 22.03% of cases. Kaplan–Meier curve revealed obvious differences in patient survival between patients in primary stages and advanced stages, as well as between patients with kidney involvement and those without kidney involvement. Cox regression analysis indicated that stage and age were 2 prognostic factors for patient survival. Conclusions: Advanced stage might be associated with poor prognosis among PBL HIV-negative patients in Chinese. PMID:28248855

  15. Extranodal lymphoma originating in the gluteal muscle with adjacent bone involvement and mimicking a soft tissue sarcoma

    Directory of Open Access Journals (Sweden)

    Morihiro Katsura

    2015-01-01

    Conclusion: A rapid growth pattern and destructive masses that invade adjacent structures on CT are key findings of DLBCL, and 18F-FDG PET/CT is a useful imaging modality to accurately determine the disease stage and disease aggressiveness of NHL.

  16. Genome-wide DNA methylation profiles according to Chlamydophila psittaci infection and the response to doxycycline treatment in ocular adnexal lymphoma.

    Science.gov (United States)

    Lee, Min Joung; Min, Byung-Joo; Choung, Ho-Kyung; Kim, Namju; Kim, Young A; Khwarg, Sang In

    2014-01-01

    To compare genome-wide DNA methylation profiles according to Chlamydophila psittaci (Cp) infection status and the response to doxycycline treatment in Korean patients with ocular adnexal extranodal marginal zone B-cell lymphoma (EMZL). Twelve ocular adnexal EMZL cases were classified into two groups (six Cp-positive cases and six Cp-negative cases). Among the 12 cases, eight were treated with doxycycline as first-line therapy, and they were divided into two groups according to their response to the treatment (four doxy-responders and four doxy-nonresponders). The differences in the DNA methylation states of 27,578 methylation sites in 14,000 genes were evaluated using Illumina bead assay technology. We also validated the top-ranking differentially methylated genes (DMGs) with bisulfite direct sequencing or pyrosequencing. The Infinium methylation chip assay revealed 180 DMGs in the Cp-positive group (74 hypermethylated genes and 106 hypomethylated genes) compared to the Cp-negative group. Among the 180 DMGs, DUSP22, which had two significantly hypomethylated loci, was validated, and the correlation was significant for one CpG site (Spearman coefficient=0.6478, p=0.0262). Regarding the response to doxycycline treatment, a total of 778 DMGs were revealed (389 hypermethylated genes and 336 hypomethylated genes in the doxy-responder group). In a subsequent replication study for representative hypomethylated (IRAK1) and hypermethylated (CXCL6) genes, the correlation between the bead chip analysis and pyrosequencing was significant (Spearman coefficient=0.8961 and 0.7619, respectively, p<0.05). Ocular adnexal EMZL showed distinct methylation patterns according to Cp infection and the response to doxycycline treatment in this genome-wide methylation study. Among the candidate genes, DUSP22 has a methylation status that was likely attributable to Cp infection. Our data also suggest that the methylation statuses of IRAK1 and CXCL6 may reflect the response to doxycycline

  17. Clinicopathological features and prognosis assessment of extranodal follicular dendritic cell sarcoma

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    AIM: To establish a model for prognosis assessment of extranodal follicular dendritic cell (FDC) sarcoma.METHODS: Nine lesions were examined by routine and molecular approaches.Clinicopathological factors from the new cases and 97 reported cases were analyzed for their prognostic values.RESULTS: The current lesions were found in f ive male and four female patients,located mainly in the head and neck area and averaging 7.2 cm in size.Six patients had recurrence or metastasis and three remained free of diseas...

  18. Diffuse large B-cell lymphoma of the parotid gland: Cytological, histopathological, and immunohistochemical features: A rare case report

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    Sainath K Andola

    2016-01-01

    Full Text Available Primary malignant lymphomas of the salivary glands are rare, accounting for 2-5% of salivary gland tumors and 5% of extranodal lymphomas, frequently seen in the parotid gland. There are single case reports mentioned in the literature. Clinical presentation is not characteristic and the disease is often overlooked with delay in diagnosis and treatment. We are reporting a case of bilateral parotid gland lymphoma in a 55-year-old male, presented with bilateral enlarged parotids. Magnetic resonance imaging (MRI showed bilateral enlarged parotid glands with multiple well-defined intraparotid lesions. Fine Needle Aspiration Cytology (FNAC of both showed mixed population of lymphoid cells with large monocytoid cells with scant cytoplasm, anisonucleosis with prominent nucleoli, and numerous mitoses suggestive of non-Hodgkin's lymphoma (NHL. Histopathology showed sheets of large lymphoma cells destructing the salivary acini and infiltrating the periparotid fat. Immunohistochemistry (IHC showed diffuse CD20 positivity, B-cell lymphoma 6 protein (Bcl-6 was focally positive and negative for cluster of differentiation (CD 3, CD5, CD10, and Multiple myeloma oncogene-1 (MUM1 which led to the diagnosis of NHL-Diffuse large B cell type.

  19. Primary pulmonary AIDS-related lymphoma Linfoma primario de pulmón en un paciente con sida

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    Marcelo Corti

    2005-08-01

    Full Text Available Extranodal involvement is common in lymphomas associated with human immunodeficiency virus infection (HIV and acquired immunodeficiency syndrome (AIDS. However, primary pulmonary AIDS-related non-Hodgkin's lymphoma is very rare and only few reports were published in the medical literature. Clinical presentation is nonspecific, with "B" and respiratory symptoms. Also, patients were with advanced immunodeficiency at the time of diagnosis. Generally, chest radiography showed peripheral nodules or cavitary masses. Primary pulmonary lymphoma associated with AIDS is generally a high-grade B-cell non-Hodgkin lymphoma and Epstein-Barr virus is strongly associated with the pathogenesis of these tumors. We report a patient with AIDS and primary pulmonary lymphoma which clinical presentation was a total atelectasis of the left lung.El compromiso extranodal es frecuente en los linfomas asociados con la enfermedad debida al virus de la inmunodeficiencia humana y su consecuencia, el síndrome de inmunodeficiencia adquirida. Sin embargo, el linfoma pulmonar primario es muy raro y solo existen pocos casos publicados en la literatura. La presentación clínica de esta complicación es inespecífica, con síntomas "B" y manifestaciones respiratorias. Generalmente, la radiografía de tórax muestra nódulos periféricos o masas que pueden cavitarse y los pacientes presentan inmunodeficiencia severa al momento del diagnóstico. El linfoma pulmonar primario asociado con el sida es un tumor de alto grado, de células B y asociado en su patogenia con el virus de Epstein-Barr. Se relata un caso de linfoma primario de pulmón que se presentó bajo la forma radiológica de una atelectasia global del pulmón izquierdo.

  20. Expression of BCL-10 protein and the relationship with API2-MALT1 fusion gene in extranodal marginal zone B-cell lymphoma of mucosa associated lymphoid tissue%黏膜相关淋巴组织结外边缘区B细胞淋巴瘤BCL-10蛋白表达及其与API2-MALT1融合基因的关系

    Institute of Scientific and Technical Information of China (English)

    李雷; 李甘地; 江炜; 杨文秀; 刘卫平; 张文燕; 李吉满; 唐源

    2006-01-01

    目的 探讨MALT淋巴瘤中BCL-10蛋白核表达与API2-MALT1融合基因的关系.方法 对86例MALT淋巴瘤进行回顾性研究,采用SP法检测BCL-10的表达,RT-PCR检测API2-MALT1融合基因.结果 10例桥本甲状腺炎均表达BCL-10于胞浆,86例MALT淋巴瘤患者中,42例(48.8%)同时表达BCL-10于胞核和胞浆,35例(40.7%)检测出API2-MALT1融合基因.BCL-10核表达的检出与API2-MALT1融合基因具有相关性(r=0.374,P=0.000).结论 MALT淋巴瘤中BCL-10蛋白的核表达与API2-MALT1融合基因相关.

  1. PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMA: CLINICOPATHOLOGICAL AND IMMUNOHISTOCHEMICAL PROFILE

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    Kanwardeep Singh

    2016-03-01

    Full Text Available BACKGROUND Primary central nervous system lymphoma (PCNSL is a rare form of extranodal non-Hodgkin lymphoma (NHL confined to the brain, spinal cord and/or eye, occurring in immunocompetent individuals. Histologically, they are diffuse large B-cell lymphomas. Over the last few decades there has been a gradual increase in their incidence. AIM To study the clinical, histopathological and immunohistochemical profile of primary central nervous system lymphoma. SETTING AND DESIGN Retrospective audit of seven cases of PCNSL diagnosed over a period of five years in a tertiary referral hospital of North India. MATERIAL AND METHODS The clinical, radiological and laboratory findings were retrieved from the hospital records. Histopathology slides were reviewed, studied in detail and a panel of immunohistochemical markers comprising of CD3, CD5, CD20, CD10, BCL6, BCL2, MUM1, CD30, EBV (LMP1, Ki-67 and p53 was done on all cases. RESULTS The male to female ratio was 3:4 with a median age of 60 years. The most common form of presentation was neurological deficits and altered sensorium. Imaging showed contrast enhancing, single or multiple, deep seated lesions within the cerebral hemispheres. Histologically, all were high-grade diffuse large B-cell lymphomas showing typical angiocentricity and a median Ki-67 proliferative index of 80%. Based on immunohistochemistry (Hans classifier three cases had germinal centre B-cell (GCB and four had non-germinal centre B-cell (non-GCB phenotype. p53 was expressed in all cases with strong expression in four of them. Four patients died before treatment could be initiated, one received palliative chemo-radiotherapy and two did not follow up after diagnosis. CONCLUSIONS Primary CNS lymphomas are high-grade diffuse large B-cell lymphomas which show high Ki-67 proliferative indices and frequent overexpression of p53. Irrespective of histological subtype, GCB or non-GCB, outcome is uniformly poor. Early and prompt diagnosis is

  2. 以冷凝集素综合征为首发表现的脾边缘区淋巴瘤一例%Splenic Marginal Zone Lymphoma With Cold-agglutination Syndrome as the First Manifestation:A Case Report

    Institute of Scientific and Technical Information of China (English)

    赵丽云

    2015-01-01

    Cold-agglutination syndrome is chronic hemolytic anemia induced by autoreactive agglutination of red cells and cold-induced factors.The disease can be primary and secondary , and it is always secondary to malignant proliferation of B -lymphocyte.This article made a case report of a patient with splenic marginal zone lymphoma ( SMZL) and cold-agglutination syndrome as the first manifestation .Abnormal dysplasia in lymph was noted in the myelography of the patient , with mainly mature and small lymphocytes , and short down was observed .The condition improved after three courses of treatment by RCHOP -21 regimen.%冷凝集素综合征是由于自身反应性红细胞凝集及冷诱导因素导致的慢性溶血性贫血性疾病,可分为原发性和继发性,后者常继发于恶性B淋巴细胞增生性疾病等。本文报道以冷凝集素综合征为首发表现的1例脾边缘区淋巴瘤( SMZL)患者,骨髓像见淋巴系异常增生,以成熟小淋巴细胞为主,可见短绒毛,经3个疗程RCHOP-21方案化疗后病情好转。

  3. Concomitant sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman Disease and diffuse large B-cell lymphoma: a case report

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    Moore James C

    2008-03-01

    Full Text Available Abstract Introduction Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman Disease, is a rare and benign source of lymphadenopathy first described in 1969, which mimics neoplastic processes. This disease commonly presents in children and young adults with supra-diaphragmatic lymphadenopathy or extranodal lesions consisting of tissue infiltrates composed of a polyclonal population of histiocytes. Since its description greater than 400 cases have been described, sometimes in patients with a variety of treated and untreated neoplastic diseases. However, the literature contains reports of only 19 cases of Rosai-Dorfman Disease in association with lymphomas, Hodgkin's or non-Hodgkin's. The majority of these cases have the two diagnoses, malignant lymphoma and Rosai-Dorfman Disease, separated in time. Interestingly, infradiaphragmatic lymphadenopathy was a feature in the majority of previously reported cases of Rosai-Dorfman Disease and non-Hodgkin's lymphoma. Case presentation This report provides details of a case with co-existing sinus histiocytosis with massive lymphadenopathy and diffuse large B cell non-Hodgkin's lymphoma. This case is the fifth described case of simultaneous Rosai-Dorfman Disease and concurrent non-Hodgkin's lymphoma. Unfortunately, the diagnosis of a clinically aggressive diffuse large B cell lymphoma was made at autopsy. The aggressive biological behavior of the diffuse large B cell lymphoma in this patient may have been related to the underlying immune dysregulation believed to be part of the pathophysiology of Rosai-Dorfman Disease. Conclusion Taken together this report and the preceding reports of Rosai-Dorfman Disease and non-Hodgkin's lymphoma suggests that in cases with a diagnosis of Rosai-Dorfman Disease in the setting of prominent infradiaphragmatic lymphadenopathy, clinicians should maintain a high index of suspicion for the presence of occult non-Hodgkin's lymphoma especially if the

  4. Direct Liver Invasion from a Gastric Adenocarcinoma as an Initial Presentation of Extranodal Tumor Spread

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    Mitanshu Shah

    2012-01-01

    Full Text Available Gastric cancer often carries a poor prognosis, with an estimated 740,000 deaths from the malignancy occurring yearly worldwide (Dicken et al., 2005. The mortality of disease is largely dependent on the extent of tumor spread, as gastric cancer has a predilection to metastasize to other visceral secondaries via hematogenous and lymphatic dissemination. Direct invasion of a gastric adenocarcinoma to adjacent organs secondary to gastric wall perforation does occur; however, it is often present in the setting of advanced disease. Rarely does direct tumor invasion to adjacent organs from a gastric adenocarcinoma present as the initial manifestation of extranodal tumor spread. We present a case of a 40-year-old male with direct tumor extension to the liver as an initial presentation of extranodal tumor spread from a gastric adenocarcinoma. Clinicians should be aware of such an occurrence, as treatment modalities in direct liver extension from a gastric adenocarcinoma vary and may be directed towards palliation rather than curative intent.

  5. Lenalidomide After Donor Bone Marrow Transplant in Treating Patients With High-Risk Hematologic Cancers

    Science.gov (United States)

    2016-12-26

    Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With Inv(16)(p13.1q22); CBFB-MYH11; Adult Acute Myeloid Leukemia With t(16;16)(p13.1;q22); CBFB-MYH11; Adult Acute Myeloid Leukemia With t(8;21); (q22; q22.1); RUNX1-RUNX1T1; Adult Acute Myeloid Leukemia With t(9;11)(p22.3;q23.3); MLLT3-KMT2A; Adult Acute Promyelocytic Leukemia With PML-RARA; Adult Grade III Lymphomatoid Granulomatosis; Adult Nasal Type Extranodal NK/T-Cell Lymphoma; Alkylating Agent-Related Acute Myeloid Leukemia; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-Cell Lymphoma; Cutaneous B-Cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Hepatosplenic T-Cell Lymphoma; Intraocular Lymphoma; Lymphomatous Involvement of Non-Cutaneous Extranodal Site; Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma; Nodal Marginal Zone Lymphoma; Post-Transplant Lymphoproliferative Disorder; Prolymphocytic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-Cell Leukemia/Lymphoma; Recurrent Cutaneous T-Cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides and Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Richter Syndrome; Small Intestinal Lymphoma; Splenic Marginal Zone Lymphoma; T-Cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenstrom Macroglobulinemia

  6. Lymphoma in acquired generalized lipodystrophy.

    Science.gov (United States)

    Brown, Rebecca J; Chan, Jean L; Jaffe, Elaine S; Cochran, Elaine; DePaoli, Alex M; Gautier, Jean-Francois; Goujard, Cecile; Vigouroux, Corinne; Gorden, Phillip

    2016-01-01

    Acquired generalized lipodystrophy (AGL) is a rare disease thought to result from autoimmune destruction of adipose tissue. Peripheral T-cell lymphoma (PTCL) has been reported in two AGL patients. We report five additional cases of lymphoma in AGL, and analyze the role of underlying autoimmunity and recombinant human leptin (metreleptin) replacement in lymphoma development. Three patients developed lymphoma during metreleptin treatment (two PTCL and one ALK-positive anaplastic large cell lymphoma), and two developed lymphomas (mycosis fungoides and Burkitt lymphoma) without metreleptin. AGL is associated with high risk for lymphoma, especially PTCL. Autoimmunity likely contributes to this risk. Lymphoma developed with or without metreleptin, suggesting metreleptin does not directly cause lymphoma development; a theoretical role of metreleptin in lymphoma progression remains possible. For most patients with AGL and severe metabolic complications, the proven benefits of metreleptin on metabolic disease will likely outweigh theoretical risks of metreleptin in lymphoma development or progression.

  7. Primary Dural Spinal Lymphoma Presentation of a Rare Spinal Tumor Case

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    Dilber Ayçiçek Çeçen

    2015-01-01

    Full Text Available Background. Primary spinal dural lymphomas (PSDL are tumors with characteristic histopathology of a lymphoma, which are completely in the spinal epidural space without any other systemic involvement. Extranodal primary lymphoma involving nervous system prefers thalamus/basal ganglia, periventricular region, cerebellum, eyes, meninges/dura, and cranial nerves or spinal cord. Rare spinal localization with acute spinal cord compression is worth attention. Case Presentation. A 48-year-old male presented with a several-month-long history of upper back pain. Lately, he had numbness and weakness at both lower extremities and was unable to walk for one week. A spinal MRI showed a thoracic lesion with cord compression at T2–T4 levels. The patient underwent surgical decompression, with his final histopathology showing diffuse large B-cell lymphoma. Systemic work-up was negative for nodal disease. Following surgery, he received radiotherapy combined with chemotherapy. He experienced a good outcome after four years. Conclusion. The upper thoracic cord is a rare location for primary spinal lesions/metastases, both of which prefer the lower thoracic and upper lumbar regions. In cases of progressive paraparesis, there should be immediate surgical intervention in the case of denovo disease, followed by combined radiotherapy and chemotherapy procedures.

  8. Targetable genetic features of primary testicular and primary central nervous system lymphomas.

    Science.gov (United States)

    Chapuy, Bjoern; Roemer, Margaretha G M; Stewart, Chip; Tan, Yuxiang; Abo, Ryan P; Zhang, Liye; Dunford, Andrew J; Meredith, David M; Thorner, Aaron R; Jordanova, Ekaterina S; Liu, Gang; Feuerhake, Friedrich; Ducar, Matthew D; Illerhaus, Gerald; Gusenleitner, Daniel; Linden, Erica A; Sun, Heather H; Homer, Heather; Aono, Miyuki; Pinkus, Geraldine S; Ligon, Azra H; Ligon, Keith L; Ferry, Judith A; Freeman, Gordon J; van Hummelen, Paul; Golub, Todd R; Getz, Gad; Rodig, Scott J; de Jong, Daphne; Monti, Stefano; Shipp, Margaret A

    2016-02-18

    Primary central nervous system lymphomas (PCNSLs) and primary testicular lymphomas (PTLs) are extranodal large B-cell lymphomas (LBCLs) with inferior responses to current empiric treatment regimens. To identify targetable genetic features of PCNSL and PTL, we characterized their recurrent somatic mutations, chromosomal rearrangements, copy number alterations (CNAs), and associated driver genes, and compared these comprehensive genetic signatures to those of diffuse LBCL and primary mediastinal large B-cell lymphoma (PMBL). These studies identify unique combinations of genetic alterations in discrete LBCL subtypes and subtype-selective bases for targeted therapy. PCNSLs and PTLs frequently exhibit genomic instability, and near-uniform, often biallelic, CDKN2A loss with rare TP53 mutations. PCNSLs and PTLs also use multiple genetic mechanisms to target key genes and pathways and exhibit near-uniform oncogenic Toll-like receptor signaling as a result of MYD88 mutation and/or NFKBIZ amplification, frequent concurrent B-cell receptor pathway activation, and deregulation of BCL6. Of great interest, PCNSLs and PTLs also have frequent 9p24.1/PD-L1/PD-L2 CNAs and additional translocations of these loci, structural bases of immune evasion that are shared with PMBL. © 2016 by The American Society of Hematology.

  9. Radiotherapy in the treatment of primary central nervous system lymphoma (PCNSL).

    Science.gov (United States)

    Nelson, D F

    1999-07-01

    The use of radiotherapy alone to treat primary central nervous system lymphoma (PCNSL) does not produce the high local control and survival rates that it does in limited extranodal non-Hodgkin's lymphoma outside the central nervous system (CNS). Even with doses of whole brain radiation therapy (WBRT) to 40+20 Gy boost, the Radiation Therapy Oncology Group (RTOG) reported a local control rate of 39%. Seventy-nine percent of recurrences were in the 60 Gy region. The median survival was 11.6 months. This response to local radiotherapy is quite different from the response of non-CNS Diffuse Large Cell Lymphoma where doses of 30-40 and >40 Gy have a 75-90% local control rate. Neither systemic lymphoma nor PCNSL have a classic radiotherapy dose response. For PCNSL there appears to be a threshold dose that ranges in the literature between 30 and > 50 Gy with a median of 40 Gy. Therefore, when radiotherapy is combined with chemotherapy that crosses the BBB, WBRT and/or boost doses may be able to be decreased, especially in patients achieving a complete response. Promising data from the Centre Leon Berard suggest that this is possible. When such chemotherapy was combined with intrathecal chemotherapy and 20 Gy WBRT, they obtained a 56% actuarial 5 year survival rate. Confirmation of single institution reports of favorable results such as these are needed. Cooperative group and intergroup trials are needed to define optimal therapy.

  10. Modern Radiation Therapy for Hodgkin Lymphoma: Field and Dose Guidelines From the International Lymphoma Radiation Oncology Group (ILROG)

    Energy Technology Data Exchange (ETDEWEB)

    Specht, Lena, E-mail: lena.specht@regionh.dk [Department of Oncology and Hematology, Rigshospitalet, University of Copenhagen (Denmark); Yahalom, Joachim [Department of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, New York (United States); Illidge, Tim [Institute of Cancer Sciences, University of Manchester, Manchester Academic Health Sciences Centre, Christie Hospital NHS Trust, Manchester (United Kingdom); Berthelsen, Anne Kiil [Department of Radiation Oncology and PET Centre, Rigshospitalet, University of Copenhagen (Denmark); Constine, Louis S. [Department of Radiation Oncology and Pediatrics, James P. Wilmot Cancer Center, University of Rochester Medical Center, Rochester, New York (United States); Eich, Hans Theodor [Department of Radiation Oncology, University of Münster (Germany); Girinsky, Theodore [Department of Radiation Oncology, Institut Gustave-Roussy, Villejuif (France); Hoppe, Richard T. [Department of Radiation Oncology, Stanford University, Stanford, California (United States); Mauch, Peter [Department of Radiation Oncology, Brigham and Women' s Hospital and Dana-Farber Cancer Institute, Harvard University, Boston, Massachusetts (United States); Mikhaeel, N. George [Department of Clinical Oncology and Radiotherapy, Guy' s and St Thomas' NHS Foundation Trust, London (United Kingdom); Ng, Andrea [Department of Radiation Oncology, Brigham and Women' s Hospital and Dana-Farber Cancer Institute, Harvard University, Boston, Massachusetts (United States)

    2014-07-15

    Radiation therapy (RT) is the most effective single modality for local control of Hodgkin lymphoma (HL) and an important component of therapy for many patients. These guidelines have been developed to address the use of RT in HL in the modern era of combined modality treatment. The role of reduced volumes and doses is addressed, integrating modern imaging with 3-dimensional (3D) planning and advanced techniques of treatment delivery. The previously applied extended field (EF) and original involved field (IF) techniques, which treated larger volumes based on nodal stations, have now been replaced by the use of limited volumes, based solely on detectable nodal (and extranodal extension) involvement at presentation, using contrast-enhanced computed tomography, positron emission tomography/computed tomography, magnetic resonance imaging, or a combination of these techniques. The International Commission on Radiation Units and Measurements concepts of gross tumor volume, clinical target volume, internal target volume, and planning target volume are used for defining the targeted volumes. Newer treatment techniques, including intensity modulated radiation therapy, breath-hold, image guided radiation therapy, and 4-dimensional imaging, should be implemented when their use is expected to decrease significantly the risk for normal tissue damage while still achieving the primary goal of local tumor control. The highly conformal involved node radiation therapy (INRT), recently introduced for patients for whom optimal imaging is available, is explained. A new concept, involved site radiation therapy (ISRT), is introduced as the standard conformal therapy for the scenario, commonly encountered, wherein optimal imaging is not available. There is increasing evidence that RT doses used in the past are higher than necessary for disease control in this era of combined modality therapy. The use of INRT and of lower doses in early-stage HL is supported by available data. Although the

  11. Pediatric lymphomas in Brazil

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    Gabriela Gualco

    2010-01-01

    Full Text Available OBJECTIVE: This study provides the clinical pathological characteristics of 1301 cases of pediatric/adolescent lymphomas in patients from different geographic regions of Brazil. METHODS: A retrospective analyses of diagnosed pediatric lymphoma cases in a 10-year period was performed. We believe that it represents the largest series of pediatric lymphomas presented from Brazil. RESULTS: Non-Hodgkin lymphomas represented 68% of the cases, including those of precursor (36% and mature (64% cell origin. Mature cell lymphomas comprised 81% of the B-cell phenotype and 19% of the T-cell phenotype. Hodgkin lymphomas represented 32% of all cases, including 87% of the classical type and 13% of nodular lymphocyte predominant type. The geographic distribution showed 38.4% of the cases in the Southeast region, 28.7% in the Northeast, 16.1% in the South, 8.8% in the North, and 8% in the Central-west region. The distribution by age groups was 15-18 years old, 33%; 11-14 years old, 26%; 6-10 years old, 24%; and 6 years old or younger, 17%. Among mature B-cell lymphomas, most of the cases were Burkitt lymphomas (65%, followed by diffuse large B-cell lymphomas (24%. In the mature T-cell group, anaplastic large cell lymphoma, ALK-positive was the most prevalent (57%, followed by peripheral T-cell lymphoma, then not otherwise specified (25%. In the group of classic Hodgkin lymphomas, the main histological subtype was nodular sclerosis (76%. Nodular lymphocyte predominance occurred more frequently than in other series. CONCLUSION: Some of the results found in this study may reflect the heterogeneous socioeconomical status and environmental factors of the Brazilian population in different regions.

  12. Lymphomas of large cells.

    Science.gov (United States)

    Staples, W G; Gétaz, E P

    1977-09-03

    Historial aspects of the classification of large-cell lymphomas are described. Immunological characterization of the lymphomas has been made possible by identification of T and B lymphocytes according to their cell membrane surface characteristics. The pathogenesis of lymphomas has been clarified by the germinal (follicular) centre cell concepts of Lennert and Lukes and Collins. The various classifications are presented and compared. Whether these subdivisions will have any relevance in the clinical context remains to be seen.

  13. 肺原发性非霍奇金淋巴瘤28例临床病理分析%Clinicopathological analysis of primary non-Hodgkin lymphoma of lung---28 cases report

    Institute of Scientific and Technical Information of China (English)

    张锦; 黄幸; 陆珍凤; 周晓军; 印洪林

    2015-01-01

    Purpose To explore the clinicopathological characteristics of primary non-Hodgkin lymphoma ( PNHL) of lung. Methods The clinical features, morphology and immunohistopathological phenotypes were retrospectively studied in 28 cases of PNHL and re-viewed of the literature. Results The composition of this group of cases is 18 cases of male, 10 cases female. The a median age of pa-tients was 57 years old. According to the WHO classification, all of the 28 cases of in our PNHL series were diagnosed as non-Hodgkin lymphoma ( NHL) , including 17 mucosa-associated lymphoid tissue extranodal marginal zone lymphomas ( MALT) , 5 diffuse large B cell lymphomas ( DLBLC) , 2 NK/T cell lymphomas and 2 anaplastic large cell lymphomas, 1 mantle cell lymphoma ( MCL) and 1 pe-ripheral T cell lymphomas, unspecified, respectively. One-third PNHL patients presented with specific clinical symptoms such as cough, chest pain, dyspnea, and fatigue, Imaging examination showed unilateral or bilateral pulmonary infiltrate, single lesions or multiple nodules, and the lesions always involved with the trachea, bronchus and lung. Follow-up was completed in 16 patients ( range, 3 to 38 months) . 3 cases were conducted with pneumonectomy, among which 2 cases were given postoperative adjuvant chem-otherapy. Seven patients were given chemotherapy alone, and 5 patients did not give any treatment following initial diagnosis. At the time of last follow-up, 13 patients were alive with disease, 2 patients were died. The tumor metastasis in the left inguinal lymph node was found in one patient after 2 years by surgery. Conclusions It was shown that there was no specific clinical manifestations and fea-tures of pulmonary PNHL. Among of them, the MALT is the most common diseases, and the highly aggressive lymphomas many be oc-cur, such as the DLBLC, NK/T cell lymphoma. The diagnosis of PNHL depends on pathological examination. The immunohistochemi-cal staining and molecular pathological technology may helpful

  14. Diagnosis of primary pulmonary lymphoma with 18 F-FDG PET/CT%18F-FDG PET/CT诊断原发性肺淋巴瘤

    Institute of Scientific and Technical Information of China (English)

    冉鹏程; 田嘉禾; 王瑞民; 刘长滨; 关志伟; 富丽萍

    2011-01-01

    目的 探讨原发性肺淋巴瘤(PPL)的18F-FDGPET/CT表现.方法 回顾性分析11例经病理证实的PPL的1s F-FDG PET/CT影像表现,并测量病灶的最大标准化摄取值(SUVmax).结果 11例患者中,结节硬化型霍奇金病2例,非霍奇金病9例,包括支气管黏膜相关淋巴组织结外边缘区B细胞淋巴瘤(MALT)7例、弥漫性大B细胞淋巴瘤1例、血管内大B细胞淋巴瘤1例;其中3例MALT淋巴瘤合并淋巴细胞性间质性肺炎(LIP).6例病变累及双肺,3例累及右肺,2例累及左肺;共有7例累及右肺中叶.病灶形态表现为单发肿块(结节)4例,肺炎样实变3例,多发结节3例,混合型1例.6例实变或较大肿块病灶中可见充气支气管征,其中的3例可见跨叶分布征.合并LIP的3例患者可见双肺广泛分布的磨玻璃影和多发含气空腔影.大多数病灶表现为放射性浓聚影.结论 18F-FDGPET/CT可以准确地显示PPL病灶的分布、形态和肿瘤活性,其影像表现具有一定的特征性,能够为PPL的诊断提供帮助.%Objective To explore the manifestations of primary pulmonary lymphoma (PPL) on 18F-FDG PET/CT images. Methods The manifestations of 11 patients with PPL confirmed by pathology were analyzed retrospectively, and the maximal standardized uptake value (SUVmax) were measured. Results There were 2 cases of nodular sclerosis Hodgkin disease and 9 cases of non-Hodgkin lymphoma (NHL). NHL included extranodal marginal zone B-cell lymphoma of muco-sa-associated lymphoid tissue (MALT, n=7) , diffuse large B-cell lymphoma and intravascular large B-cell lymphoma (each n=l). Three patients of MALT complicated with lymphocytic interstitial pneumonia (LIP). Six of the 11 cases involved double lungs, 3 involved right lung and 2 involved the left lung. The right middle lobe was involved in 7 cases. The lesion was a single mass (node) in 4 cases, similar to the consolidation caused by pneumonia in 3 cases, disseminated nodes in 3 cases and mixed type

  15. Lymphoma Microenvironment and Immunotherapy.

    Science.gov (United States)

    Xu, Mina L; Fedoriw, Yuri

    2016-03-01

    Understanding of the lymphoma tumor microenvironment is poised to expand in the era of next-generation sequencing studies of the tumor cells themselves. Successful therapies of the future will rely on deeper appreciation of the interactions between elements of the microenvironment. Although the phenotypic, cytogenetic, and molecular characterization of tumor cells in lymphomas has progressed faster than most other solid organ tumors, concrete advancements in understanding the lymphoma microenvironment have been fewer. This article explores the composition of the lymphoma tumor microenvironment; its role in immune surveillance, evasion, and drug resistance; and its potential role in the development of targeted therapies. Copyright © 2016 Elsevier Inc. All rights reserved.

  16. Bilateral Primary Intraocular Lymphoma

    Directory of Open Access Journals (Sweden)

    Mehrdad Karimi

    2011-01-01

    Full Text Available Purpose: To report a case of bilateral primary intraocular lymphoma. Case report: A 33-year-old man presented with bilateral blurred vision since two years ago. Examination revealed large keratic precipitates, anterior chamber reaction, posterior subcapsular cataracts, and vitreous infiltration. After a short trial of topical and periocular steroids, diagnostic 25-gauge pars plana vitrectomy was performed and cytologic evaluation of the aspirate confirmed a diagnosis of intraocular lymphoma. The patient was subsequently managed with intravitreal methotrexate in both eyes and responded favorably. Central nervous system workup for lymphoma was negative. Conclusion: Primary intraocular lymphoma should be considered in young adults suffering from chronic recalcitrant panuveitis.

  17. Presence of a high-grade component in gastric mucosa-associated lymphoid tissue (MALT) lymphoma is not associated with an adverse prognosis.

    Science.gov (United States)

    Ang, Mei-Kim; Hee, Siew Wan; Quek, Richard; Yap, Swee Peng; Loong, Susan; Tan, Leonard; Tao, Miriam; Lim, Soon Thye

    2009-05-01

    Gastric mucosa-associated lymphoid tissue (MALT) lymphoma and diffuse large B cell lymphoma (DLBCL) show a spectrum of disease characterized by varying proportions of low-grade and high-grade components. While the natural history and optimum treatment for low-grade gastric MALT lymphoma and DLBCL is well established, the prognosis and optimal treatment of patients with both low- and high-grade components is not well established. The purpose of our study was to evaluate the clinical characteristics, survival outcomes, and prognostic factors of patients with gastric MALT lymphoma and gastric DLBCL. A retrospective review of patients with gastric MALT lymphoma, gastric DLBCL, or MALT lymphoma with a high-grade component treated at our centers from 1994 to 2006 was performed. Patients were divided into three categories: "pure MALT lymphoma," "MALT lymphoma with high-grade component" (mixed), and "pure DLBCL." Seventy-six patients were included in our study-26 with pure MALT, 22 with MALT with high-grade component ("mixed"), and 28 with pure DLBCL. Pure MALT lymphoma and mixed lymphoma patients had similar clinical characteristics, whereas pure DLBCL patients had less favorable disease characteristics with significantly poorer performance status, higher number of extranodal sites of disease, higher stage, and larger proportion of bone marrow involvement and international prognostic index (IPI) scores compared with mixed lymphoma. The majority of mixed lymphoma (72.7%) and DLBCL patients (71.4%) were treated with chemotherapy. Of patients receiving chemotherapy, a higher proportion of mixed lymphoma and DLBCL patients received anthracycline-based combination chemotherapy regimens compared with MALT lymphoma (73% vs 71% vs 8%) whereas the proportion of mixed lymphoma and DLBCL patients was similar (p = 0.919). At a median follow-up of 37 months, the 5-year overall survival was 66.9%. The 5-year overall survival was 78% for MALT lymphoma, 84% for mixed lymphoma, and 45

  18. A clinically based prognostic index for diffuse large B-cell lymphoma with a cut-off at 70 years of age significantly improves prognostic stratification: population-based analysis from the Danish Lymphoma Registry.

    Science.gov (United States)

    Gang, Anne O; Pedersen, Michael; d'Amore, Francesco; Pedersen, Lars M; Jensen, Bo A; Jensen, Paw; Møller, Michael B; Mourits-Andersen, Hans T; Pedersen, Robert S; Klausen, Tobias W; de N Brown, Peter

    2015-01-01

    The introduction of rituximab and generally improved health among elderly patients have increased the survival of patients with diffuse large B-cell lymphoma (DLBCL). The International Prognostic Index (IPI) from 1992 is based on pre-rituximab data from clinical trials including several lymphoma subtypes. We applied IPI factors to a population-based rituximab-treated cohort of 1990 patients diagnosed 2000-2010 and explored new factors and the optimal prognostic age cut-off for DLBCL. Multivariate-analyses (MVA) confirmed the prognostic value of all IPI factors except the presence of > 1 extranodal lesion. The optimal age cut-off was 70 years. In a MVA of albumin, lymphocyte count, sex, immunoglobulin G, bulky disease, hemoglobin and B-symptoms, only albumin was prognostic. We propose: (1) a modified DLBCL prognostic index (DLBCL-PI) including: age (70 years), performance status (PS), lactate dehydrogenase (LDH), stage and albumin level, and (2) a separate age-adjusted DLBCL-PI for patients ≤ 70 years including PS, LDH, albumin level and > 1 extranodal lesion, however excluding stage.

  19. Margin requirements, margin loans, and margin rates: practice and principles

    OpenAIRE

    Peter Fortune

    2000-01-01

    The Board of Governors of the Federal Reserve System establishes initial margin requirements under Regulations T, U, and X. Recent margin loan increases, both in aggregate value and relative to market capitalization, have rekindled the debate about using margin requirements as an instrument to affect the prices of common stocks. Proponents of a more active margin requirement policy see the regulations as instruments for affecting the level and volatility of stock prices by influencing investo...

  20. Whole-body MRI for initial staging of paediatric lymphoma: prospective comparison to an FDG-PET/CT-based reference standard

    Energy Technology Data Exchange (ETDEWEB)

    Littooij, Annemieke S. [University Medical Centre Utrecht/Wilhelmina Children' s Hospital, Department of Radiology and Nuclear Medicine, Utrecht (Netherlands); KK Women' s and Children' s Hospital, Department of Diagnostic and Interventional Imaging, Singapore (Singapore); Kwee, Thomas C.; Vermoolen, Malou A.; Keizer, Bart de; Beek, Frederik J.A.; Hobbelink, Monique G.; Nievelstein, Rutger A.J. [University Medical Centre Utrecht/Wilhelmina Children' s Hospital, Department of Radiology and Nuclear Medicine, Utrecht (Netherlands); Barber, Ignasi; Enriquez, Goya [Hospital Materno-Infantil Vall d' Hebron, Department of Paediatric Radiology, Barcelona (Spain); Granata, Claudio [IRCCS Giannina Gaslini Hospital, Department of Radiology, Genoa (Italy); Zsiros, Jozsef [University of Amsterdam, Department of Paediatric Oncology, Emma Children' s Hospital, Academic Medical Centre, Amsterdam (Netherlands); Soh, Shui Yen [KK Women' s and Children' s Hospital, Haematology and Oncology service, Department of Paediatric Subspecialities, Singapore (Singapore); Bierings, Marc B. [University Medical Centre Utrecht/Wilhelmina Children' s Hospital, Department of Paediatric Haematology-Oncology, Utrecht (Netherlands); Stoker, Jaap [University of Amsterdam, Department of Radiology, Academic Medical Centre, Amsterdam (Netherlands)

    2014-05-15

    To compare whole-body MRI, including diffusion-weighted imaging (whole-body MRI-DWI), with FDG-PET/CT for staging newly diagnosed paediatric lymphoma. A total of 36 children with newly diagnosed lymphoma prospectively underwent both whole-body MRI-DWI and FDG-PET/CT. Whole-body MRI-DWI was successfully performed in 33 patients (mean age 13.9 years). Whole-body MRI-DWI was independently evaluated by two blinded observers. After consensus reading, an unblinded expert panel evaluated the discrepant findings between whole-body MRI-DWI and FDG-PET/CT and used bone marrow biopsy, other imaging data and clinical information to derive an FDG-PET/CT-based reference standard. Interobserver agreement of whole-body MRI-DWI was good [all nodal sites together (κ = 0.79); all extranodal sites together (κ = 0.69)]. There was very good agreement between the consensus whole-body MRI-DWI- and FDG-PET/CT-based reference standard for nodal (κ = 0.91) and extranodal (κ = 0.94) staging. The sensitivity and specificity of consensus whole-body MRI-DWI were 93 % and 98 % for nodal staging and 89 % and 100 % for extranodal staging, respectively. Following removal of MRI reader errors, the disease stage according to whole-body MRI-DWI agreed with the reference standard in 28 of 33 patients. Our results indicate that whole-body MRI-DWI is feasible for staging paediatric lymphoma and could potentially serve as a good radiation-free alternative to FDG-PET/CT. (orig.)

  1. Alemtuzumab, Fludarabine Phosphate, and Low-Dose Total Body Irradiation Before Donor Stem Cell Transplantation in Treating Patients With Hematological Malignancies

    Science.gov (United States)

    2016-01-05

    Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Childhood Burkitt Lymphoma; Childhood Chronic Myelogenous Leukemia; Childhood Diffuse Large Cell Lymphoma; Childhood Immunoblastic Large Cell Lymphoma; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Chronic Phase Chronic Myelogenous Leukemia; Contiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Contiguous Stage II Grade 1 Follicular Lymphoma; Contiguous Stage II Grade 2 Follicular Lymphoma; Contiguous Stage II Marginal Zone Lymphoma; Contiguous Stage II Small Lymphocytic Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable; Nodal Marginal Zone B-cell Lymphoma; Noncontiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Noncontiguous Stage II Grade 1 Follicular Lymphoma; Noncontiguous Stage II Grade 2 Follicular Lymphoma; Noncontiguous Stage II Marginal Zone Lymphoma; Noncontiguous Stage II Small Lymphocytic Lymphoma; Peripheral T-cell Lymphoma; Previously Treated Myelodysplastic Syndromes; Progressive Hairy Cell Leukemia, Initial Treatment; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Childhood Acute

  2. Usefulness of Random Skin Biopsy as a Diagnostic Tool of Intravascular Lymphoma Presenting With Fever of Unknown Origin.

    Science.gov (United States)

    Pongpudpunth, Marinya; Rattanakaemakorn, Ploysyne; Fleischer, Alan B

    2015-09-01

    Intravascular B-cell lymphoma (IVBCL) is a rare type of extranodal lymphoma in which the lymphoma proliferates within vascular lumen. One of the most common presenting symptoms is prolonged fever. Although examination of a random skin biopsy from healthy-appearing skin in patients with suspected intravascular lymphoma has been reported to be useful, the sensitivity of this method for diagnosis is still unknown. To evaluate the usefulness of a random skin biopsy in the diagnosis of intravascular lymphoma in the patient presenting with fever of unknown origin. All cases that we performed random skin biopsy were retrospectively reviewed from March 2007 to June 2012 in Ramathibodi Hospital. The incisional biopsy technique was performed in 3 separate locations including 2 specimens from both upper thighs and 1 specimen from the abdomen. Of the 24 cases that were consulted to dermatologist for random skin biopsy, 3 cases (13%) were diagnosed of intravascular lymphoma and all presented with prolong fever, weight loss, and remarkable deterioration in Eastern Cooperative Oncology Group (ECOG) performance status (ECOG score) > 2. Interestingly, 2 cases (8%) were diagnosed with cytophagic histiocytic panniculitis from normal-appearing skin and subsequently investigated, which leads to the diagnosed of T-cell lymphoma. In an elderly patient with fever of unknown origin and/or weight loss with no evident of skin lesion, markedly elevated lactate dehydrogenase, remarkable deterioration in ECOG performance status who suspected IVBCL should be consider to perform incisional random skin biopsy at least 3 specimens from both thighs and abdomen to achieve a high sensitivity. We propose that this technique may serve as an important diagnostic tool to obtain the diagnosis of IVBCL. In summary, 5 of 24 (21%) positive results from random skin biopsy helped diagnoses of lymphoma.

  3. Clinicopathologic significance of tumor microenvironment CD11c, and FOXP3 expression in diffuse large B-cell lymphoma patients receiving rituximab, cyclophosphamide, anthracycline, vincristine, and prednisone (R-CHOP) combination chemotherapy.

    Science.gov (United States)

    Lee, Seul; Kim, Dong Hyun; Oh, Sung Yong; Kim, So Yeon; Koh, Myeong Seok; Lee, Ji Hyun; Lee, Suee; Kim, Sung-Hyun; Kwak, Jong-Young; Pak, Min Gyoung; Ju, Mi Ha; Kim, Hyo-Jin; Jeong, Jin Sook

    2017-03-01

    CD11c is a dendritic cell marker in humans, which potentially induces a cytotoxic effect on lymphoma cells. Forkhead boxP3 (FOXP3) is a regulator of T lymphocyte in the microenvironment of the lymphoma. The principal objective of this study was to determine whether the tumors' microenvironment expressions of CD11c and FOXP3 are predictive of clinical outcomes in diffuse large B-cell lymphoma (DLBCL) patients receiving treatment with rituximab, cyclophosphamide, anthracycline, vincristine, and prednisone (R-CHOP) combination chemotherapy. The study population consisted of 100 patients with DLBCL. The CD11c and FOXP3 expression in primary tumors' microenvironment were evaluated using an immunohistochemistry (IHC). CD11c and FOXP3 expression positivity in microenvironment were 25% and 35%, respectively. Each one counted for 1 point. In CD11c and FOXP3 stain, positive was counted as 0 and negative was 1. The points were separated into low risk (0 to 1) and high risk (2) groups. Only the extranodal DLBCL patient group analysis conveyed significant differences of progression-free survival (p = 0.019) and overall survival (p = 0.039) between the two groups. We can achieve possible clinical significance of lymphoma tumor microenvironments through CD11c and FOXP3 IHC stains in extranodal DLBCL patients receiving R-CHOP therapy.

  4. Extranodal Rosai-Dorfman Disease Involving the Left Atrium: Cardiac MRI, CT, and PET Scan Findings

    Directory of Open Access Journals (Sweden)

    Vistasp J. Daruwalla

    2015-01-01

    Full Text Available Rosai-Dorfman disease (RDD is a rare entity that usually involves the lymph nodes but extranodal involvements have been seen in numerous cases, although RDD with cardiovascular involvement is extremely rare. We describe a case of a young male who presented with intermittent palpitations and was found to have a left atrium mass. Our case not only emphasizes the rarity of the above lesion but also highlights the importance of modern-day imaging like computed tomography, Cardiac Magnetic Resonance Imaging (CMRI, and PET scan in characterizing such nonspecific lesions and directing appropriate line of treatment. RDD should be considered as one of the differentials even for isolated cardiac lesions.

  5. [Molecular abnormalities in lymphomas].

    Science.gov (United States)

    Delsol, G

    2010-11-01

    Numerous molecular abnormalities have been described in lymphomas. They are of diagnostic and prognostic value and are taken into account for the WHO classification of these tumors. They also shed some light on the underlying molecular mechanisms involved in lymphomas. Overall, four types of molecular abnormalities are involved: mutations, translocations, amplifications and deletions of tumor suppressor genes. Several techniques are available to detect these molecular anomalies: conventional cytogenetic analysis, multicolor FISH, CGH array or gene expression profiling using DNA microarrays. In some lymphomas, genetic abnormalities are responsible for the expression of an abnormal protein (e.g. tyrosine-kinase, transcription factor) detectable by immunohistochemistry. In the present review, molecular abnormalities observed in the most frequent B, T or NK cell lymphomas are discussed. In the broad spectrum of diffuse large B-cell lymphomas microarray analysis shows mostly two subgroups of tumors, one with gene expression signature corresponding to germinal center B-cell-like (GCB: CD10+, BCL6 [B-Cell Lymphoma 6]+, centerine+, MUM1-) and a subgroup expressing an activated B-cell-like signature (ABC: CD10-, BCL6-, centerine-, MUM1+). Among other B-cell lymphomas with well characterized molecular abnormalies are follicular lymphoma (BCL2 deregulation), MALT lymphoma (Mucosa Associated Lymphoid Tissue) [API2-MALT1 (mucosa-associated-lymphoid-tissue-lymphoma-translocation-gene1) fusion protein or deregulation BCL10, MALT1, FOXP1. MALT1 transcription factors], mantle cell lymphoma (cycline D1 [CCND1] overexpression) and Burkitt lymphoma (c-Myc expression). Except for ALK (anaplastic lymphoma kinase)-positive anaplastic large cell lymphoma, well characterized molecular anomalies are rare in lymphomas developed from T or NK cells. Peripheral T cell lymphomas not otherwise specified are a heterogeneous group of tumors with frequent but not recurrent molecular abnormalities

  6. Sarcoidosis Occurring After Lymphoma

    Science.gov (United States)

    London, Jonathan; Grados, Aurélie; Fermé, Christophe; Charmillon, Alexandre; Maurier, François; Deau, Bénédicte; Crickx, Etienne; Brice, Pauline; Chapelon-Abric, Catherine; Haioun, Corinne; Burroni, Barbara; Alifano, Marco; Le Jeunne, Claire; Guillevin, Loïc; Costedoat-Chalumeau, Nathalie; Schleinitz, Nicolas; Mouthon, Luc; Terrier, Benjamin

    2014-01-01

    Abstract Sarcoidosis is a granulomatous disease that most frequently affects the lungs with pulmonary infiltrates and/or bilateral hilar and mediastinal lymphadenopathy. An association of sarcoidosis and lymphoproliferative disease has previously been reported as the sarcoidosis-lymphoma syndrome. Although this syndrome is characterized by sarcoidosis preceding lymphoma, very few cases of sarcoidosis following lymphoma have been reported. We describe the clinical, biological, and radiological characteristics and outcome of 39 patients presenting with sarcoidosis following lymphoproliferative disease, including 14 previously unreported cases and 25 additional patients, after performing a literature review. Hodgkin lymphoma and non-Hodgkin lymphoma were equally represented. The median delay between lymphoma and sarcoidosis was 18 months. Only 16 patients (41%) required treatment. Sarcoidosis was of mild intensity or self-healing in most cases, and overall clinical response to sarcoidosis was excellent with complete clinical response in 91% of patients. Sarcoidosis was identified after a follow-up computerized tomography scan (CT-scan) or 18fluorodeoxyglucose-positron emission tomography/computerized tomography (18FDG-PET/CT) evaluation in 18/34 patients (53%). Sarcoidosis is therefore a differential diagnosis to consider when lymphoma relapse is suspected on a CT-scan or 18FDG-PET/CT, emphasizing the necessity to rely on histological confirmation of lymphoma relapse. PMID:25380084

  7. Biomarkers for lymphoma

    Science.gov (United States)

    Zangar, Richard C.; Varnum, Susan M.

    2014-09-02

    A biomarker, method, test kit, and diagnostic system for detecting the presence of lymphoma in a person are disclosed. The lymphoma may be Hodgkin's lymphoma or non-Hodgkin's lymphoma. The person may be a high-risk subject. In one embodiment, a plasma sample from a person is obtained. The level of at least one protein listed in Table S3 in the plasma sample is measured. The level of at least one protein in the plasma sample is compared with the level in a normal or healthy subject. The lymphoma is diagnosed based upon the level of the at least one protein in the plasma sample in comparison to the normal or healthy level.

  8. [Secondary orbital lymphoma].

    Science.gov (United States)

    Basanta, I; Sevillano, C; Álvarez, M D

    2015-09-01

    A case is presented of an 85 year-old Caucasian female with lymphoma that recurred in the orbit (secondary ocular adnexal lymphoma). The orbital tumour was a diffuse large B-cell lymphoma according to the REAL classification (Revised European-American Lymphoma Classification). Orbital lymphomas are predominantly B-cell proliferations of a variety of histological types, and most are low-grade tumours. Patients are usually middle-aged or elderly, and it is slightly more common in women. A palpable mass, proptosis and blepharoptosis are the most common signs of presentation. Copyright © 2011 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.

  9. Angioimmunoblastic T-Cell Lymphoma

    Science.gov (United States)

    Angioimmunoblastic T-Cell Lymphoma Overview Lymphoma is the most common blood cancer. The two main forms of lymphoma are ... develop into lymphomas: B-lymphocytes (B-cells) and T-lymphocytes (T-cells). Cancerous lymphocytes can travel to ...

  10. Primary leptomeningeal lymphoma

    Science.gov (United States)

    Taylor, Jennie W.; Flanagan, Eoin P.; O'Neill, Brian P.; Siegal, Tali; Omuro, Antonio; DeAngelis, Lisa; Baehring, Joachim; Nishikawa, Ryo; Pinto, Fernando; Chamberlain, Marc; Hoang-Xuan, Khe; Gonzalez-Aguilar, Alberto; Batchelor, Tracy; Blay, Jean-Yves; Korfel, Agnieszka; Betensky, Rebecca A.; Lopes, Maria-Beatriz S.

    2013-01-01

    Objective: To evaluate clinical presentation, optimal diagnostic evaluation and treatment, and outcome in primary leptomeningeal lymphoma, a rare form of primary CNS lymphoma without parenchymal or systemic involvement. Methods: The International Primary CNS Lymphoma Collaborative Group, a multidisciplinary group of physicians with a particular interest in primary CNS lymphoma, retrospectively identified cases of lymphoma isolated to the leptomeninges as diagnosed by CSF cytology, flow cytometry, or biopsy, without systemic or parenchymal brain/spinal cord lymphoma or immunodeficiency. Results: Forty-eight patients were identified, with median age at diagnosis of 51 years and median Eastern Cooperative Oncology Group performance status of 2. Presenting symptoms were multifocal in 68%. Leptomeningeal enhancement was seen in 74% and CSF profile was abnormal in all cases. CSF cytology detected malignant lymphocytes in 67%. Flow cytometry identified monoclonal population in 80%, as did receptor gene rearrangement studies in 71%. Sixty-two percent had B-cell lymphoma, 19% T-cell, and 19% unclassified. Treatment varied and included fractionated radiotherapy (36%), systemic chemotherapy (78%), and intra-CSF chemotherapy (66%), with 66% receiving ≥2 modalities. Seventy-one percent had a favorable clinical response; ultimately, 44% received salvage treatment. Median overall survival was 24 months, with 11 patients still alive at 50 months follow-up. Conclusion: Primary leptomeningeal lymphoma is a rare form of primary CNS lymphoma. Patients usually present with multifocal symptoms, with evidence of leptomeningeal enhancement and diagnostic CSF analysis. Although treatment is highly variable, patients have a better prognosis than previously reported and a subset may be cured. PMID:24107866

  11. Radiotherapy for Hodgkin lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Specht, Lena [Rigshospitalet Copenhagen Univ. (Denmark). Depts. of Oncology and Haematology; Yahalom, Joachim (eds.) [Memorial Sloan-Kettering Cancer, New York, NY (United States). Dept. of Radiation Oncology

    2011-07-01

    This book deals in detail with all aspects of the best practice in modern radiotherapy for Hodgkin lymphoma. It provides the background and rationale for the inclusion of radiotherapy in today's combined-modality approach, including special clinical situations such as Hodgkin lymphoma in children, in the pregnant patient, and in the elderly. Radiotherapy planning using state-of-the-art imaging, target definition, planning software, and treatment equipment is expounded in detail. Acute and long-term side effects of radiotherapy are analyzed, and the implications for modern radiotherapy approaches in Hodgkin lymphomas are explained. (orig.)

  12. Linfoma não Hodgkin gástrico Gastric non-Hodgkin Lymphoma

    Directory of Open Access Journals (Sweden)

    Renata O. Costa

    2010-02-01

    Full Text Available Os linfomas extralinfonodais representam aproximadamente 1/3 de todos os linfomas não Hodgkin (LNH e, embora possam ter início em qualquer tecido, mais frequentemente acometem o trato gastrointestinal, sendo o estômago o órgão responsável pela grande maioria dos casos. Os linfomas primários gástricos são comumente LNH, sendo representados em mais de 95% dos casos pelo linfoma difuso de grandes células B e pelo linfoma MALT (mucosa associated lymphoid tissue. De evolução indolente, o linfoma MALT destaca-se por ser um modelo de câncer secundário à estimulação antigênica crônica exercida por uma bactéria denominada Helicobacter pylori (HP. No outro polo, situa-se o linfoma difuso de células B (LDGCB, que, de patogênese duvidosa, pode tratar-se de uma transformação de LNH MALT ou ainda se caracterizar por um linfoma "de novo". Neste estudo, revisamos a literatura, enfatizando aspectos importantes à prática clínica destes linfomas.Extranodal lymphomas account for about 30% of all non-Hodgkin lymphomas (NHL, and although they can originate in any tissue, the gastrointestinal tract is the most commonly affected structure with the stomach being the most common subtype. Diffuse Large B cell lymphoma (DLBCL and MALT (mucosa associated lymphoid tissue lymphoma account for more than 95% of the cases of gastric lymphoma. The indolent development of MALT lymphoma stands out as it is a type of cancer subject to chronic antigen stimulation by the Helicobacter pylori bacteria. Conversely, diffuse large B cell lymphomas, whose pathogenesis is uncertain, can be a transformation from MALT NHL or perhaps a new type of lymphoma. In this study we carried out a review of the literature, stressing the key aspects of these lymphomas in the clinical practice.

  13. Treatment Options for Adult Hodgkin Lymphoma

    Science.gov (United States)

    ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ...

  14. General Information about AIDS-Related Lymphoma

    Science.gov (United States)

    ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ...

  15. General Information about Adult Hodgkin Lymphoma

    Science.gov (United States)

    ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ...

  16. Treatment Options for AIDS-Related Lymphoma

    Science.gov (United States)

    ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ...

  17. Treatment Option Overview (Childhood Hodgkin Lymphoma)

    Science.gov (United States)

    ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ...

  18. Treatment Option Overview (Adult Hodgkin Lymphoma)

    Science.gov (United States)

    ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ...

  19. Stages of Childhood Non-Hodgkin Lymphoma

    Science.gov (United States)

    ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ...

  20. Treatment Options for Hodgkin Lymphoma during Pregnancy

    Science.gov (United States)

    ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ... Treatment Adult NHL Treatment AIDS-Related Lymphoma Treatment Mycosis Fungoides & Sézary Syndrome Treatment Primary CNS Lymphoma Treatment ...

  1. Non-Hodgkin Lymphoma (For Parents)

    Science.gov (United States)

    ... Kids to Be Smart About Social Media Non-Hodgkin Lymphoma KidsHealth > For Parents > Non-Hodgkin Lymphoma Print ... harmful things out of the body. About Non-Hodgkin Lymphoma No n-Hodgkin lymphoma is a disease ...

  2. Assessment of BIOMED-2 assays for detection of clonal Ig gene rearrangements in mature B-cell lymphomas%BIOMED-2聚合酶链反应Ig基因重排对成熟非霍奇金B细胞淋巴瘤诊断的价值

    Institute of Scientific and Technical Information of China (English)

    张婧; 吴迎辉; 孔海鹰; 周小鸽; 金哈斯; 吴晓明; 张丹丹; 宫丽平

    2009-01-01

    目的 探讨BIOMED-2聚合酶链反应(PCR)在成熟非霍奇金B细胞淋巴瘤(B-NHL)诊断中的价值.方法 收集成熟B-NHL组织标本72例,其中弥漫性大B细胞淋巴瘤37例,黏膜相关淋巴组织结外边缘区淋巴瘤35例为研究对象,并以反应性增生病变25例作为对照.提取以上组织的DNA,并以PCR来检测其完整性和可扩增性,选取质量合格的DNA.85.6%(83/97)的样品DNA长度>300 bp,其中60例成熟B-NHL和23例反应性增生可用于BIOMED-2 PCR检测免疫球蛋白重链(IgH)和kappa轻链(IgK)基因重排的克隆性.结果 利用BIOMED-2 PCR检测的60例成熟B-NHL中,57例存在Ig基因的克隆性重排,其检测敏感性为95%,23例反应性增生病例中未出现Ig基因的克隆性重排,其检测特异性为100%.结论 BIOMED-2 PCR适用于石蜡包埋组织.该方法具有很高的敏感性和特异性,对成熟B-NHL诊断的辅助价值很高.%Objective To evaluate the efficiency of the BIOMED-2 PCR assay and its implication in the diagnosis of mature B-cell non-Hodgkin's lymphomas. Methods Clinical, morphological and immunohistochemical features of 72 cases of non-Hodgkin's lymphomas were studied, including 25 reactive lymphoid hyperplasia, 37 diffuse large B cell lymphomas (DLBCL) and 35 extranodal marginal zone lymphomas of mucosa associated lymphoid tissues (MALT lymphoma and in addition, 25 cases of reactive lymphoid hyperplasia were used as the controls). DNA was exacted from the paraffin embedded formalin fixed tissue blocks and the quality of DNA was assessed using the BIOMED-2 specimen control reaction. Adequate samples were then analyzed by BIOMED-2 for immunoglobulin heavy and kappa light chain rearrangements. Results Adequate DNA was obtained in 83 of 97 samples, including 60 mature B cell lymphomas and 23 reactive lymphoid hyperplasia. Clonal B-cell gene rearrangements were detected in 57 of 60(95%) lymphomas. In contrast, clonal Ig gene rearrangements were not detected in any of the 23

  3. An update on the management of peripheral T-cell lymphoma and emerging treatment options

    Directory of Open Access Journals (Sweden)

    Phillips AA

    2011-09-01

    Full Text Available Adrienne A Phillips1, Colette Owens2, Sangmin Lee1, Govind Bhagat31Division of Medical Oncology, Department of Medicine, 2Division of General Medicine, Department of Medicine, 3Department of Pathology and Cell Biology, Columbia University Medical Center and New York Presbyterian Hospital, Columbia University, New York, NY, USAAbstract: Peripheral T-cell lymphomas (PTCLs comprise a rare and heterogeneous subset of non-Hodgkin’s lymphomas (NHLs that arise from post-thymic T-cells or natural killer (NK-cells at nodal or extranodal sites. Worldwide, PTCLs represent approximately 12% of all NHLs and the 2008 World Health Organization (WHO classification includes over 20 biologically and clinically distinct T/NK-cell neoplasms that differ significantly in presentation, pathology, and response to therapy. Because of the rarity and heterogeneity of these diseases, large clinical trials have not been conducted and optimal therapy is not well defined. Most subtypes are treated with similar combination chemotherapy regimens as used for aggressive B-cell NHL, but with poorer outcomes. New treatment combinations and novel agents are currently being explored for PTCLs and this review highlights a number of options that appear promising.Keywords: treatment, non-Hodgkin’s lymphoma, novel therapy, natural-killer cells

  4. [MALT B cell lymphoma with kidney damage and monoclonal gammopathy: a case study and literature review].

    Science.gov (United States)

    Peces, R; Vega-Cabrera, C; Peces, C; Pobes, A; Fresno, M F

    2010-01-01

    We report a case of low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) involving the left kidney and simultaneous onset of a monoclonal gammopathy IgM kappa. No predisposing local inflammatory condition was identified. Following left nephrectomy, the renal specimen showed the centrocyte like cells and lymphoid cells in the lymphoepithelial lesions were positive for CD20 and CD79α. The neoplastic cells expressed monotypic cytoplasmic IgM kappa. The demonstration of bone marrow cells of B-lineage expressing the same monoclonal protein as the tumor suggested bone marrow involvement, even in the absence of identical morphology. Despite chemotherapy and rituximab treatment, clinical follow-up showed right kidney extension with high-grade transformation, and finally systemic dissemination. This case illustrates that the kidney is among the sites that may be involved by MALT B-cell lymphomas in a primary or secondary fashion, and the need for expanded investigation of the possible dissemination. We review the literature on this unusual extranodal lymphoma.

  5. Pulmonary intravascular lymphoma detected by FDG PET-CT: a case report.

    Science.gov (United States)

    Kohan, A A; Paganini, L; Biedak, P; Arma, J I; Dalurzo, M C L; Garcia-Monaco, R D

    2013-01-01

    Intravascular lymphoma is a rare subtype of extranodal Non-Hodgkin's lymphoma. Its prognosis is poor in a high percentage of cases due to its insidious appearance and low clinical suspicion. Its diagnosis is usually only reached after an autopsy. It may affect different organs as a whole or only one organ. It is extremely rare that the lung is the only damaged organ. Its diagnosis depends of the clinician's suspicion and proper evaluation with imaging studies as well as correct selection of the organ to be biopsied. When detected on time, the treatment of choice is a combination of a series of chemotherapy associated to a monoclonal antibody (anti-CD20). We present the case of a male patient who underwent a positron emission tomography-computed tomography with 2-[F-18]-fluoro-2 deoxy-D-glucose (FDG) due to symptoms suggestive of a lymphoproliferative disease with no clear structural abnormalities. The images led to a diagnosis of pulmonary intravascular large B cell lymphoma. Copyright © 2012 Elsevier España, S.L. and SEMNIM. All rights reserved.

  6. Fludarabine Phosphate, Cyclophosphamide, Tacrolimus, Mycophenolate Mofetil, Total-Body Irradiation, and Donor Bone Marrow Transplant in Treating Patients With High-Risk Hematologic Cancer

    Science.gov (United States)

    2014-02-27

    Accelerated Phase Chronic Myelogenous Leukemia; Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Childhood Acute Lymphoblastic Leukemia in Remission; Childhood Acute Myeloid Leukemia in Remission; Childhood Burkitt Lymphoma; Childhood Chronic Myelogenous Leukemia; Childhood Myelodysplastic Syndromes; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; de Novo Myelodysplastic Syndromes; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hematopoietic/Lymphoid Cancer; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Peripheral T-cell Lymphoma; Post-transplant Lymphoproliferative Disorder; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Childhood Anaplastic Large Cell Lymphoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma

  7. 原发性皮肤边缘区B细胞淋巴瘤的BCL10表达与染色体异常%BCL10 expression and chromosomal aberration in primary cutaneous marginal zone B-cell lymphoma

    Institute of Scientific and Technical Information of China (English)

    李百周; 孔蕴毅; 杨文涛; 周晓燕; 范月珍; 陆洪芬; 施达仁

    2008-01-01

    目的 探讨原发性皮肤边缘区B细胞淋巴瘤(PCMZL)中BCL10蛋白的表达和相关染色体的异常.方法 收集17例PCMZL,用免疫组化检测BCL10的表达,用荧光原位杂交(FISH)的方法分别检测API2-MALT1、BCL10、MALT1和IgH基因的异常.结果 在17例PCMZL中,BCL10的阳性率为94.1%(16/17),其中细胞质阳性率为64.7%(11/17),细胞核阳性率为29.4%(5/17).在FISH检测中,所有病例都不存在t(11;18),t(1;14)和t(14;18)染色体异常.与其他部位的MALT淋巴瘤相比较,在PCMZL中染色体易位的发生率不常见,可能这些遗传学异常不是PCMZL发生中的重要因素,而有其他目前未知的因素参与肿瘤的发生.结论 BCL10的核表达与是否出现上述的染色体异常无关,是否代表更具侵袭性的一种预后标记,尚需长期随访观察.%Objective To study the expression of BCL10 and associated chromosomal aberration in primary cutaneous marginal zone B-cell lymphoma (PCMZL). Methods Tissue specimens were collected from 17 patients with PCMZL. Immunohistochemistry was used to detect the expression of BCL10. Fluorescence in situ hybridization (FISH) was performed to examine the presence of API2-MALT1 fusion gene and chromosomal aberration in BCL10, MALT1 as well as IgH genes in these cases. Results Of these patients,94.1% (16/17) expressed BCL10 protein. The cytoplasmic expression of BCL10 was observed in 64.7% (11/17) of the patients, and nuclear expression in 29.4% (5/17). As shown by FISH test, neither API2-MALT1 fusion gene nor chromosomal aberration in BCL10, MALT1 or IgH genes was present in these patients. Conclusions Compared with MALT lymphomas originating from tissues other than skin, PCMZL is uncommonly associated with chromosomal abnormalities; it is possible that there are unknown factors contributing to its tumorigenesis. Nuclear BCL10 is unrelated to the presence of chromosomal aberration in BCL10, MALT1 or IgH genes. Further follow-up is required to clarify the

  8. Treatment Effects and Sequelae of Radiation Therapy for Orbital Mucosa-Associated Lymphoid Tissue Lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Hata, Masaharu, E-mail: mhata@syd.odn.ne.jp [Department of Radiology, Yokohama City University Graduate School of Medicine, Yokohama, Kanagawa (Japan); Omura, Motoko; Koike, Izumi [Department of Radiology, Yokohama City University Graduate School of Medicine, Yokohama, Kanagawa (Japan); Tomita, Naoto [Department of Internal Medicine and Clinical Immunology, Yokohama City University Graduate School of Medicine, Yokohama, Kanagawa (Japan); Iijima, Yasuhito [Department of Ophthalmology, Yokohama City University Graduate School of Medicine, Yokohama, Kanagawa (Japan); Tayama, Yoshibumi; Odagiri, Kazumasa; Minagawa, Yumiko [Department of Radiology, Yokohama City University Graduate School of Medicine, Yokohama, Kanagawa (Japan); Ogino, Ichiro [Department of Radiation Oncology, Yokohama City University Medical Center, Yokohama, Kanagawa (Japan); Inoue, Tomio [Department of Radiology, Yokohama City University Graduate School of Medicine, Yokohama, Kanagawa (Japan)

    2011-12-01

    Purpose: Among extranodal lymphomas, orbital mucosa-associated lymphoid tissue (MALT) lymphoma is a relatively rare presentation. We performed a review to ascertain treatment efficacy and toxicity of radiation therapy for orbital MALT lymphoma. We also evaluated changes in visual acuity after irradiation. Methods and Materials: Thirty patients with orbital MALT lymphoma underwent radiation therapy with curative intent. Clinical stages at diagnosis were stage I{sub E}A in 29 patients and stage II{sub E}A in 1 patient. Total doses of 28.8 to 45.8 Gy (median, 30 Gy) in 15 to 26 fractions (median, 16 fractions) were delivered to the tumors. Results: All irradiated tumors were controlled during the follow-up period of 2 to 157 months (median, 35 months) after treatment. Two patients had relapses that arose in the cervical lymph node and the ipsilateral palpebral conjunctiva outside the radiation field at 15 and 67 months after treatment, respectively. The 5-year local progression-free and relapse-free rates were 100% and 96%, respectively. All 30 patients are presently alive; the overall and relapse-free survival rates at 5 years were 100% and 96%, respectively. Although 5 patients developed cataracts of grade 2 at 8 to 45 months after irradiation, they underwent intraocular lens implantation, and their eyesight recovered. Additionally, there was no marked deterioration in the visual acuity of patients due to irradiation, with the exception of cataracts. No therapy-related toxicity of grade 3 or greater was observed. Conclusions: Radiation therapy was effective and safe for patients with orbital MALT lymphoma. Although some patients developed cataracts after irradiation, visual acuity was well preserved.

  9. Lymphoma Research Foundation

    Science.gov (United States)

    ... the stem cell transplantation process. Read More LYMPHOMA RESEARCH Featured Researcher – David Scott, MBChB, PhD Dr. Scott ... and Advocacy News Action Center Advocacy Tool Kit Research LRF Research Portfolio Disease-Specific Focus Areas Grants ...

  10. ALK-positive anaplastic large cell lymphoma presenting as intradural spinal mass: first reported case and review of literature.

    Science.gov (United States)

    Novello, Mariangela; Lauriola, Libero; Della Pepa, Giuseppe Maria; Giuseppe, La Rocca; Coli, Antonella; Visocchi, Massimiliano

    2013-08-01

    Anaplastic large cell lymphoma (ALCL) is characterized by large anaplastic cells of T-cell or null-cell phenotype expressing CD30 (Ki-1 antigen). In most cases this neoplasm expresses the anaplastic lymphoma kinase (ALK), a chimeric protein resulting from the t(2;5)(p23;q35) translocation. ALK-positive anaplastic large cell lymphoma is most frequent in the first three decades of life and shows a male predominance, involving both nodal and extranodal sites, but rarely the CNS. We report a 21-year-old patient with a previous history of nodal ALK-positive ALCL, lymphohistiocytic subtype, who was admitted for recent occurrence of left-sided anesthesia with pain and progressive motor weakness of both legs. An MRI of the spine documented an intradural extramedullary mass dislocating the thoracic cord, suggesting a meningioma and the patient underwent surgical decompression. Histological examination revealed a lymphoproliferative neoplasm with morphology and immunophenotype of ALK-positive anaplastic large cell lymphoma. After surgery, all preoperative symptoms disappeared. To our knowledge, no cases of ALCL presenting as secondary localization with an intradural extramedullary spinal mass have been reported in the literature. © 2012 Japanese Society of Neuropathology.

  11. Combined modality treatment for stage I-II non-Hodgkin's lymphomas: CVP versus BACOP chemotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Bajetta, E.; Valagussa, P.; Bonadonna, G.; Lattuada, A.; Buzzoni, R.; Rilke, F.; Banfi, A.

    1988-07-01

    This paper reports the 5-year results of a prospective randomized study beginning in 1976 on 177 evaluable patients with pathologic Stage I-IE and II-IIE non-Hodgkin's lymphomas with diffuse histology according to the Rappaport classification. Treatment consisted of either CVP or BACOP chemotherapy (3 cycles) followed by regional radiotherapy (40 to 50 Gy) and further cycles of either combination. In both arms, complete remission at the end of combined treatment was high (CVP 93%, BACOP 98%) regardless of age, stage or bulky disease. At 5 years, the comparative freedom from first progression was 62% for CVP vs 78% for BACOP (p = 0.02), respectively. Clinically relevant differences favoring BACOP chemotherapy were essentially documented in patients with large cell lymphomas (International Working Formulation), those with Stage II having more than three involved anatomical sites, bulky disease and age over 60 years. Recurrence within radiation fields was documented in only 5% of complete responders. Combined treatment was, in general, well tolerated particularly when BACOP was used. In only 2 patients given CVP post radiation cutaneous fibrosis was documented. Second solid tumors were detected in 4 patients. One patient started on CVP died because of brain stem necrosis after 45 Gy. We conclude that in Stage I-II patients with nodal and extranodal diffuse non-Hodgkin's lymphomas, particularly large cell lymphomas, combined modality approach with primary Adriamycin and bleomycin containing regimen, such as BACOP, followed by adjuvant radiotherapy offers high chances of cure with minimal toxicity.

  12. [Systemic lymphoma cells with T precursor condition of extreme female genital tract. A case report and literature review].

    Science.gov (United States)

    Butrón Valdez, Karla; Ramírez Galves, Miguel; Germes Piña, Fernando; Ramos Martínez, Ernesto; Zamora Perea, Arturo

    2009-06-01

    Primary female genital tract non Hodgkin's lymphoma is a rare presentation for a common disease in the childhood, and its classification as primary extranodal lymphoma is still controversial. There are a few cases reported as a primary precursor B-cell lymphoblastic lymphoma of the female genital tract, but there is not any case reported as primary precursor T-cell lymphoblastic lymphoma of the ovary in childhood. Herein we describe a 16 years old young woman with bilateral ovarian tumors, paraaortic lymphoadenophaty and disseminate disease to the female genital tract including extension of the tumor to neighboring organs like the omentum and the appendix. Exploratory laparatomy were performed with bilateral salpingo-oophorectomy, hysterectomy, omentectomy, appendectomy, pelvic and para-aortic lymphadenectomy, pelvic washings and with biopsy of vaginal vault. The chemotherapy regimen comprised of CHOP (Cyclophosphamide, Hydroxydaunorubicin, Oncovin, Prednisone/Prednisolone) and methotrexate, 3 months later presents left facial hemiparesia follow by right facial hemiparesia, 7 months later presents more Central Nervous System (CNS) complications and apparently was complicated with acute lymphocitic leukemia and after 16 months from the diagnosis, following by a torpid evolution, the pacient finally died.

  13. Insufficiency of Positron Emission Tomography and Magnetic Resonance Spectroscopy in the Diagnosis of Intravascular Lymphoma of the Central Nervous System

    Directory of Open Access Journals (Sweden)

    Nobuyuki Kawai

    2012-07-01

    Full Text Available Intravascular large B-cell lymphoma (IVL is a rare type of extranodal lymphoma with an aggressive clinical course characterized by the proliferation of lymphoma cells within the lumen of small vessels. Diagnosis is often difficult because of marked variability in clinical presentation and nonspecific laboratory and radiological findings, especially when central nervous system (CNS symptoms are the only manifestation. Modern metabolic imaging techniques such as positron emission tomography (PET and 1H-magnetic resonance spectroscopy (MRS have been reported to be useful in the diagnosis of conventional primary CNS lymphoma. We report the case of a 69-year-old man who presented with a progressive leukoencephalopathic syndrome. The patient was examined by 18F-fluorodeoxyglucose and 11C-methionine PET and MRS, but none of these examinations were able to show the presence of a tumor in the lesions or to clarify the tumor characteristics. Brain biopsy was the only way to obtain a definite diagnosis of IVL. The patient was treated intensively with standard immunochemotherapy but died 6 months after the diagnosis. Here, we discuss the insufficiency of modern metabolic imaging techniques, including PET and MRS, and recommend a rapid decision of brain biopsy in the diagnosis of IVL only involving the CNS.

  14. General Information about Adult Non-Hodgkin Lymphoma

    Science.gov (United States)

    ... Lymphoma Treatment AIDS-Related Lymphoma Treatment Chronic Lymphocytic Leukemia Treatment (small lymphocytic lymphoma) Mycosis Fungoides (Including Sézary Syndrome) Treatment (cutaneous T-cell lymphoma) Primary CNS Lymphoma Treatment Non-Hodgkin lymphoma ...

  15. Treatment Option Overview (Adult Non-Hodgkin Lymphoma)

    Science.gov (United States)

    ... Lymphoma Treatment AIDS-Related Lymphoma Treatment Chronic Lymphocytic Leukemia Treatment (small lymphocytic lymphoma) Mycosis Fungoides (Including Sézary Syndrome) Treatment (cutaneous T-cell lymphoma) Primary CNS Lymphoma Treatment Non-Hodgkin lymphoma ...

  16. CD13-positive anaplastic large cell lymphoma of T-cell origin--a diagnostic and histogenetic problem.

    Science.gov (United States)

    Popnikolov, N K; Payne, D A; Hudnall, S D; Hawkins, H K; Kumar, M; Norris, B A; Elghetany, M T

    2000-12-01

    The expression of myelomonocytic-associated antigens in anaplastic large cell lymphomas (ALCLs), particularly those presenting in extranodal sites, can make their distinction from extramedullary myeloid cell tumors (EMCTs) or histiocytic tumors problematic. Yet, this distinction is clinically significant because of its therapeutic and prognostic implications. Herein, we describe a case of extranodal anaplastic lymphoma kinase-positive CD30-positive ALCL of T-cell origin in a 12-year-old boy, which was initially called an EMCT because of the expression of CD13 and HLA-DR detected by flow cytometry and the absence of other T-cell-related surface markers. However, the detection of cytoplasmic CD3 by flow cytometry prompted further studies. The tumor was composed of large cells with abundant slightly eosinophilic vacuolated cytoplasm and ovoid or reniform nuclei with a few small nucleoli. Using immunohistochemistry, the tumor was positive for CD45, CD30, CD45RO, and CD43 with a strong cytoplasmic and nuclear anaplastic lymphoma kinase stain. The tumor cells showed a T-cell clonal genotype. Electron microscopy revealed no ultrastructural features of myelomonocytic or histiocytic origin. The patient responded well to the chemotherapy and was in complete remission for 10 months at the time of submission of this manuscript. Review of the literature showed inconsistencies regarding the diagnosis, nomenclature, and, therefore, treatment and prognosis of these tumors. In addition, the CD13 expression in ALCL raises some histogenetic questions and may indicate origin from a pluripotent stem cell, misprogramming during malignant transformation, or a microenvironmental effect on lymphoid cell expression of surface antigens. Therefore, ALCL should be considered in the differential diagnosis of EMCTs or histiocytic tumors, particularly when surface marker lineage assignment is ambiguous.

  17. Tacrolimus and Mycophenolate Mofetil in Preventing Graft-Versus-Host Disease in Patients Who Have Undergone Total-Body Irradiation With or Without Fludarabine Phosphate Followed by Donor Peripheral Blood Stem Cell Transplant for Hematologic Cancer

    Science.gov (United States)

    2016-01-25

    Accelerated Phase Chronic Myelogenous Leukemia; Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Blastic Phase Chronic Myelogenous Leukemia; Childhood Acute Lymphoblastic Leukemia in Remission; Childhood Acute Myeloid Leukemia in Remission; Childhood Burkitt Lymphoma; Childhood Chronic Myelogenous Leukemia; Childhood Diffuse Large Cell Lymphoma; Childhood Immunoblastic Large Cell Lymphoma; Childhood Myelodysplastic Syndromes; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Chronic Phase Chronic Myelogenous Leukemia; Contiguous Stage II Adult Burkitt Lymphoma; Contiguous Stage II Adult Diffuse Large Cell Lymphoma; Contiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Contiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Contiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Contiguous Stage II Adult Lymphoblastic Lymphoma; Contiguous Stage II Grade 1 Follicular Lymphoma; Contiguous Stage II Grade 2 Follicular Lymphoma; Contiguous Stage II Grade 3 Follicular Lymphoma; Contiguous Stage II Mantle Cell Lymphoma; Contiguous Stage II Marginal Zone Lymphoma; Contiguous Stage II Small Lymphocytic Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; de Novo Myelodysplastic Syndromes; Essential Thrombocythemia; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncontiguous Stage II Adult Burkitt Lymphoma; Noncontiguous Stage II Adult Diffuse Large Cell Lymphoma; Noncontiguous

  18. A 92-year-old man with primary cutaneous diffuse large B-cell non-Hodgkin's lymphoma manifesting as a giant scalp mass

    Science.gov (United States)

    Liao, Chenlong; Yang, Min; Liu, Pengfei; Zhang, Wenchuan

    2017-01-01

    Abstract Rationale: Primary cutaneous non-Hodgkin's lymphoma (NHL) is an uncommon entity, representing 10% of all extranodal NHLs. Among all cutaneous sites, the scalp is a rare site of representation. Patient concerns: A 92-year-old Chinese man visited our hospital with a multiple-nodular huge scalp mass on the right parieto-occipital regions. The mass was of 7-month duration and progressively enlarging in size. Diagnoses: On the basis of the result of biopsy, diffuse large B-cell NHL was diagnosed. Interventions: The mass was partially resected by surgery and no further treatment was conducted due to the advanced age and poor physical status. Outcomes: The tumor relapsed in situ after 6 months and the patient died after 2 years. Lessons: This case highlighted the limited access to standard treatment options in patients with advanced age. A thorough examination is necessary to decide upon the treatment for the primary cutaneous lymphoma. PMID:28272240

  19. True histiocytic lymphoma following B-acute lymphoblastic leukaemia: case report with evidence for a common clonal origin in both neoplasms.

    Science.gov (United States)

    Bouabdallah, R; Abéna, P; Chetaille, B; Aurran-Schleinitz, T; Sainty, D; Dubus, P; Arnoulet, C; Coso, D; Xerri, L; Gastaut, J A

    2001-06-01

    True histiocytic lymphoma (THL) is a very rare type of non-Hodgkin's lymphoma (NHL) in which neoplastic cells exhibit markers of histiocytic differentiation. Some cases of THL have been reported in patients with previous acute lymphoblastic leukaemia (ALL), especially in children and young adults, in whom the acute leukaemia was of T-cell origin. The relationship between the initial lymphoid tumour and the secondary THL remains unclear, as a common monoclonal origin shared by both neoplasms has never been definitively demonstrated. We report a patient with B-ALL who developed a nodal and extranodal tumour with histological and immunohistochemical features of THL 4 years after the initial diagnosis. Genotypic study showed that both neoplasms contained the same immunoglobulin heavy gene rearrangement, which has not been reported previously.

  20. Diverse hematological malignancies including hodgkin-like lymphomas develop in chimeric MHC class II transgenic mice.

    Directory of Open Access Journals (Sweden)

    Silke H Raffegerst

    Full Text Available A chimeric HLA-DR4-H2-E (DR4 homozygous transgenic mouse line spontaneously develops diverse hematological malignancies with high frequency (70%. The majority of malignancies were distributed equally between T and B cell neoplasms and included lymphoblastic T cell lymphoma (LTCL, lymphoblastic B cell lymphoma (LBCL, diffuse large B cell lymphoma (DLBCL, the histiocyte/T cell rich variant of DLBCL (DLBCL-HA/T cell rich DLBCL, splenic marginal zone lymphoma (SMZL, follicular B cell lymphoma (FBL and plasmacytoma (PCT. Most of these neoplasms were highly similar to human diseases. Also, some non-lymphoid malignancies such as acute myeloid leukemia (AML and histiocytic sarcoma were found. Interestingly, composite lymphomas, including Hodgkin-like lymphomas, were also detected that had CD30(+ Hodgkin/Reed-Sternberg (H/RS-like cells, representing a tumor type not previously described in mice. Analysis of microdissected H/RS-like cells revealed their origin as germinal center B cells bearing somatic hypermutations and, in some instances, crippled mutations, as described for human Hodgkin lymphoma (HL. Transgene integration in an oncogene was excluded as an exclusive driving force of tumorigenesis and age-related lymphoma development suggests a multi-step process. Thus, this DR4 line is a useful model to investigate common molecular mechanisms that may contribute to important neoplastic diseases in man.

  1. Final Results of the IELSG-19 Randomized Trial of Mucosa-Associated Lymphoid Tissue Lymphoma: Improved Event-Free and Progression-Free Survival With Rituximab Plus Chlorambucil Versus Either Chlorambucil or Rituximab Monotherapy.

    Science.gov (United States)

    Zucca, Emanuele; Conconi, Annarita; Martinelli, Giovanni; Bouabdallah, Reda; Tucci, Alessandra; Vitolo, Umberto; Martelli, Maurizio; Pettengell, Ruth; Salles, Gilles; Sebban, Catherine; Guillermo, Armando Lopez; Pinotti, Graziella; Devizzi, Liliana; Morschhauser, Franck; Tilly, Hervé; Torri, Valter; Hohaus, Stefan; Ferreri, Andrés J M; Zachée, Pierre; Bosly, André; Haioun, Corinne; Stelitano, Caterina; Bellei, Monica; Ponzoni, Maurilio; Moreau, Anne; Jack, Andrew; Campo, Elias; Mazzucchelli, Luca; Cavalli, Franco; Johnson, Peter; Thieblemont, Catherine

    2017-06-10

    Purpose There is no consensus on the optimal systemic treatment of patients with extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue. The IELSG-19 phase III study, to our knowledge, was the first such study to address the question of first-line treatment in a randomized trial. Patients and Methods Eligible patients were initially randomly assigned (1:1 ratio) to receive either chlorambucil monotherapy (6 mg/m(2)/d orally on weeks 1 to 6, 9 to 10, 13 to 14, 17 to 18, and 21 to 22) or a combination of chlorambucil (same schedule as above) and rituximab (375 mg/m(2) intravenously on day 1 of weeks 1, 2, 3, 4, 9, 13, 17, and 21). After the planned enrollment of 252 patients, the protocol was amended to continue with a three-arm design (1:1:6 ratio), with a new arm that included rituximab alone (same schedule as the combination arm) and with a final sample size of 454 patients. The main end point was event-free survival (EFS). Analysis of chlorambucil versus the combination arm was performed and reported separately before any analysis of the third arm. Results At a median follow-up of 7.4 years, addition of rituximab to chlorambucil led to significantly better EFS (hazard ratio, 0.54; 95% CI, 0.38 to 0.77). EFS at 5 years was 51% (95% CI, 42 to 60) with chlorambucil alone, 50% (95% CI, 42 to 59) with rituximab alone, and 68% (95% CI, 60 to 76) with the combination ( P = .0009). Progression-free survival was also significantly better with the combination ( P = .0119). Five-year overall survival was approximately 90% in each arm. All treatments were well tolerated. No unexpected toxicities were recorded. Conclusion Rituximab in combination with chlorambucil demonstrated superior efficacy in mucosa-associated lymphoid tissue lymphoma; however, improvements in EFS and progression-free survival did not translate into longer overall survival.

  2. Drugs Approved for Hodgkin Lymphoma

    Science.gov (United States)

    ... Ask about Your Treatment Research Drugs Approved for Hodgkin Lymphoma This page lists cancer drugs approved by ... that are not listed here. Drugs Approved for Hodgkin Lymphoma Adcetris (Brentuximab Vedotin) Ambochlorin (Chlorambucil) Amboclorin (Chlorambucil) ...

  3. Fludarabine Phosphate, Melphalan, Total-Body Irradiation, Donor Stem Cell Transplant in Treating Patients With Hematologic Cancer or Bone Marrow Failure Disorders

    Science.gov (United States)

    2016-05-05

    Accelerated Phase Chronic Myelogenous Leukemia; Acute Myeloid Leukemia With Multilineage Dysplasia Following Myelodysplastic Syndrome; Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Aplastic Anemia; Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative; Childhood Acute Lymphoblastic Leukemia in Remission; Childhood Acute Myeloid Leukemia in Remission; Childhood Chronic Myelogenous Leukemia; Childhood Diffuse Large Cell Lymphoma; Childhood Immunoblastic Large Cell Lymphoma; Childhood Myelodysplastic Syndromes; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Chronic Eosinophilic Leukemia; Chronic Myelomonocytic Leukemia; Chronic Neutrophilic Leukemia; Chronic Phase Chronic Myelogenous Leukemia; de Novo Myelodysplastic Syndromes; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Fanconi Anemia; Juvenile Myelomonocytic Leukemia; Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable; Nodal Marginal Zone B-cell Lymphoma; Noncontiguous Stage II Adult Burkitt Lymphoma; Noncontiguous Stage II Adult Diffuse Large Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Noncontiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Noncontiguous Stage II Adult Lymphoblastic Lymphoma; Noncontiguous Stage II Grade 1 Follicular Lymphoma; Noncontiguous Stage II Grade 2 Follicular Lymphoma; Noncontiguous Stage II Grade 3 Follicular Lymphoma; Noncontiguous Stage II Mantle Cell Lymphoma

  4. Safety and Tolerability Study of PCI-32765 in B Cell Lymphoma and Chronic Lymphocytic Leukemia

    Science.gov (United States)

    2017-10-09

    B-cell Chronic Lymphocytic Leukemia; Small Lymphocytic Lymphoma; Diffuse Well-differentiated Lymphocytic Lymphoma; B Cell Lymphoma; Follicular Lymphoma; Mantle Cell Lymphoma; Non-Hodgkin's Lymphoma; Waldenstrom Macroglobulinemia; Burkitt Lymphoma; B-Cell Diffuse Lymphoma

  5. Danish National Lymphoma Registry

    DEFF Research Database (Denmark)

    Arboe, Bente; Josefsson, Pär; Jørgensen, Judit;

    2016-01-01

    AIM OF DATABASE: The Danish National Lymphoma Registry (LYFO) was established in order to monitor and improve the diagnostic evaluation and the quality of treatment of all lymphoma patients in Denmark. STUDY POPULATION: The LYFO database was established in 1982 as a seminational database including...... all lymphoma patients referred to the departments of hematology. The database became nationwide on January 1, 2000. MAIN VARIABLES: The main variables include both clinical and paraclinical variables as well as details of treatment and treatment evaluation. Up to four forms are completed for each...... patient: a primary registration form, a treatment form, a relapse form, and a follow-up form. Variables are used to calculate six result quality indicators (mortality 30 and 180 days after diagnosis, response to first-line treatment, and survival estimates 1, 3, and 5 years after the time of diagnosis...

  6. Primary lymphoma of the colon Linfoma primario de colon

    Directory of Open Access Journals (Sweden)

    Marta Pascual

    2013-02-01

    Full Text Available Background: primary colorectal lymphoma is a very rare disease, representing less than 0.5 % of all primary colorectal neoplasms. The gastrointestinal tract is the most frequently involved site of all extranodal lymphomas, the most common type of that is non-Hodgkin's lymphoma. Early diagnosis is often difficult because of unspecific symptoms. Therapeutic approaches have classically included radical resection, chemotherapy and radiotherapy. Materials and methods: we present our experience in the management of primary colorectal lymphomas over a 17-year period (1994-20011. Results: in this period 7 cases of primary colorectal lymphoma were diagnosed in our institution. Abdominal pain and change in bowel habit were the most frequent symptoms. Five patients underwent emergency surgery because of bleeding or bowel obstruction. All primary intestinal lymphomas studied were of the B-cell phenotype. Patients were followed up for a median of 59 months (range 1-180. Three of them are alive with no evidence of recurrence. Conclusion: combination treatment with chemotherapy and surgery can obtain good remission rate. Surgery can resolve complications such bleeding or intestinal perforation that are implicated in lymphoma mortality.Introducción: el linfoma primario de colon y recto es una patología poco prevalente, representa tan solo el 0,5 % de todas las neoplasias primarias de colon y recto. El tracto gastrointestinal es el lugar donde asientan la gran mayoría de los linfomas extranodales, siendo el más frecuente el tipo linfoma no-Hodking. El diagnóstico precoz es siempre difícil debido a que la sintomatología es muy poco específica. Los algoritmos terapéuticos han incluido clásicamente la resección radical, el tratamiento con quimioterapia y con radioterapia. Materiales y métodos: presentamos nuestra experiencia en el manejo de los linfomas primarios de colon en un periodo de 17 años (1994-2011. Resultados: en dicho periodo en nuestro

  7. Lymphoma of the Cervix

    Directory of Open Access Journals (Sweden)

    Juanita Parnis

    2012-01-01

    Full Text Available Primary non-Hodgkins lymphoma of the uterine cervix is a very rare diagnosis. A 54-year-old woman presented with a 3-month history of postmenopausal bleeding per vaginum. On examination, a friable, fungating lesion was seen on the cervix. Histology revealed a CD 20 positive high-grade non-Hodgkin’s diffuse large B cell lymphoma from cervical biopsies and endometrial curettage. She was diagnosed as stage IE after workup and subsequently treated with six cycles of R-CHOP chemotherapy followed by radiotherapy of the involved field.

  8. MGMT promoter methylation and correlation with protein expression in primary central nervous system lymphoma.

    Science.gov (United States)

    Toffolatti, L; Scquizzato, E; Cavallin, S; Canal, F; Scarpa, M; Stefani, P M; Gherlinzoni, F; Dei Tos, A P

    2014-11-01

    The O (6)-methylguanine-DNA-methyltransferase (MGMT) gene encodes for a DNA repairing enzyme of which silencing by promoter methylation is involved in brain tumorigenesis. MGMT promoter methylation represents a favorable prognostic factor and has been associated with a better response to alkylating agents in glioma and systemic lymphoma. Primary central nervous system lymphoma (PCNSL) is a rare and aggressive extranodal malignant lymphoma. The current standard of care, based on high-dose methotrexate chemotherapy, has improved prognosis but outcome remains poor for a majority of patients. Therapeutic progress in this field is conditioned by limited biological and molecular knowledge about the disease. Temozolomide has recently emerged as an alternative option for PCNSL treatment. We aimed to analyze the MGMT gene methylation status in a series of 24 PCNSLs, to investigate the relationship between methylation status of the gene and immunohistochemical expression of MGMT protein and to evaluate the possible prognostic significance of these biomarkers. Our results confirm that methylation of the MGMT gene and loss of MGMT protein are frequent events in these lymphomas (54 % of our cases) and suggest that they are gender and age related. MGMT methylation showed high correlation with loss of protein expression (concordance correlation coefficient = -0.49; Fisher exact test: p MGMT promoter (n = 4), seems to be associated with a prolonged overall survival (>60 months in three of four patients). The prognostic significance of these molecular markers in PCNSL needs to be further studied in groups of patients treated in a homogeneous way.

  9. Diagnostic and prognostic impact of {sup 18}F-FDG PET/CT in follicular lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Le Dortz, Ludovic; Garin, Etienne [Eugene Marquis Anticancer Centre, Nuclear Medicine Department, Rennes (France); Inserm U991, Rennes (France); Guibert, Sophie de; Houot, Roch [CHU Pontchaillou, Haematological Department, Rennes (France); Bayat, Sahar; Cuggia, Marc [CHU Pontchaillou, Medical Information Department, Rennes (France); Devillers, Anne; Le Jeune, Florence; Bahri, Haifa; Barge, Marie-Luce [Eugene Marquis Anticancer Centre, Nuclear Medicine Department, Rennes (France); Rolland, Yan [Eugene Marquis Anticancer Centre, Medical Imaging Department, Rennes (France); Lamy, Thierry [CHU Pontchaillou, Haematological Department, Rennes (France); Inserm U917, Rennes (France)

    2010-12-15

    The aim of this study was to assess the usefulness of positron emission tomography/computed tomography in staging, prognosis evaluation and restaging of patients with follicular lymphoma. A retrospective study was performed on 45 patients with untreated biopsy-proven follicular lymphoma who underwent {sup 18}F-fluorodeoxyglucose PET/CT (FDG PET/CT) and CT before and after chemoimmunotherapy induction treatment (rituximab combined with cyclophosphamide, doxorubicin, vincristine and prednisone). PET/CT detected more nodal (+51%) and extranodal (+89%) lesions than CT. PET/CT modified Ann Arbor staging in eight patients (18%). Five patients (11%) initially considered as being early stage (I/II) were eventually treated as advanced stage (III/IV). In this study, an initial PET/CT prognostic score was significantly more accurate than the Follicular Lymphoma International Prognostic Index score in identifying patients with poor prognosis (i.e. patients with incomplete therapeutic response or early relapse). The accuracy of PET/CT for therapeutic response assessment was higher than that of CT (0.97 vs 0.64), especially due to its ability to identify inactive residual masses. In addition, post-treatment PET/CT was able to predict patients' outcomes. The median progression-free survival was 48 months in the PET/CT-negative group as compared with 17.2 months for the group with residual uptake (p < 10{sup -4}). FDG PET/CT is useful for staging and assessing the prognosis and therapeutic response of patients with follicular lymphoma. (orig.)

  10. The anti-lymphoma activity of antiviral therapy in HCV-associated B-cell non-Hodgkin lymphomas: a meta-analysis.

    Science.gov (United States)

    Peveling-Oberhag, J; Arcaini, L; Bankov, K; Zeuzem, S; Herrmann, E

    2016-07-01

    Many epidemiological studies provide solid evidence for an association of chronic hepatitis C virus (HCV) infection with B-cell non-Hodgkin's lymphoma (B-NHL). However, the most convincing evidence for a causal relationship between HCV infection and lymphoma development is the observation of B-NHL regression after HCV eradication by antiviral therapy (AVT). We conducted a literature search to identify studies that included patients with HCV-associated B-NHL (HCV-NHL) who received AVT, with the intention to treat lymphoma and viral disease at the same time. The primary end point was the correlation of sustained virological response (SVR) under AVT with lymphoma response. Secondary end points were overall lymphoma response rates and HCV-NHL response in correlation with lymphoma subtypes. We included 20 studies that evaluated the efficacy of AVT in HCV-NHL (n = 254 patients). Overall lymphoma response rate through AVT was 73% [95%>confidence interval, (CI) 67-78%]. Throughout studies there was a strong association between SVR and lymphoma response (83% response rate, 95%>CI, 76-88%) compared to a failure in achieving SVR (53% response rate, 95%>CI, 39-67%, P = 0.0002). There was a trend towards favourable response for AVT in HCV-associated marginal zone lymphomas (response rate 81%, 95%>CI, 74-87%) compared to nonmarginal zone origin (response rate 71%, 95%>CI, 61-79%, P = 0.07). In conclusion, in the current meta-analysis, the overall response rate of HCV-NHL under AVT justifies the recommendation for AVT as first-line treatment in patients who do not need immediate conventional treatment. The strong correlation of SVR and lymphoma regression supports the hypothesis of a causal relationship of HCV and lymphomagenesis.

  11. Bone marrow inifltration and clinical features in lymphoma patients with diffused high bone marrow glucose uptake by18F-FDG PET/CT%伴PET/CT骨髓弥漫性糖代谢增高的淋巴瘤患者骨髓浸润情况及相关因素分析

    Institute of Scientific and Technical Information of China (English)

    顾史洋; 邹善华; 李锋; 王伟光; 袁玲; 季丽莉; 程韵枫

    2015-01-01

    pathological features and bone marrow infiltration status in lymphoma patients with diffused high bone marrow glucose uptake on18F-FDG PET/CT.Methods:It was a retrospective study. Bone marrow infiltration status, pathological and clinical data from 62 cases of pathologically diagnosed lymphoma and diffused high bone marrow glucose uptake were analyzed.Results:Distribution of histopathological subtype in those cases was in accordance with that in previously reported Chinese lymphoma patients. Significant difference was demonstrated in standard uptake value (SUV) between pa-tients with aggressive and indolent histopathological subtypes (8.43vs 5.38,P=0.048), patients with and without B symp-toms (8.30vs 5.72,P=0.033), and patients with and without bone marrow infiltration (8.78vs 6.96,P=0.020). 32 patients were diagnosed as “bone marrow infiltration” by bone marrow biopsy. There was significant difference in histopathologi-cal subtype distribution between patients with and without bone marrow infiltration (P=0.001). In patients with bone marrow infiltration, there were higher proportions of mantle cell lymphoma, nodal marginal zone B cell lymphoma, Burkitt’s lym-phoma and anaplastic large cell lymphoma. In contrast, patients without bone marrow infiltration suffered more from diffuse large B-cell lymphoma, peripheral T cell lymphoma, enteropathic T cell lymphoma and extranodal NK/T-cell lymphoma (nasal type). False positive results in bone marrow glucose uptake may be caused by fever or anemia.Conclusion:Diffused high bone marrow glucose uptake on18F-FDG PET/CT should be evaluated in combination with the uptake values, clinical features and histological subtypes, to minimize the misdiagnosis and to better guide staging and therapy of lymphoma.

  12. Primary Pulmonary Hodgkin Lymphoma

    OpenAIRE

    Shumaila Tanveer; Ahmed El Damati; Ayman El Baz; Ahmed Alsayyah; Tarek ElSharkawy; Mohamed Regal

    2015-01-01

    Primary pulmonary Hodgkin lymphoma (PPHL) is a rare disease. Herein, we report a case of PPHL with diagnostic concerns encountered during initial evaluation which is of paramount importance to keep the differential diagnosis in cases with high index of sus- picion for this rare entity.

  13. Lymphoma: Immune Evasion Strategies

    Energy Technology Data Exchange (ETDEWEB)

    Upadhyay, Ranjan; Hammerich, Linda; Peng, Paul [Division of Hematology and Medical Oncology, Icahn School of Medicine at Mount Sinai, New York, NY 10029 (United States); Brown, Brian [Department of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY 10029 (United States); Merad, Miriam [Department of Oncological Sciences, Icahn School of Medicine at Mount Sinai, New York, NY 10029 (United States); Brody, Joshua D., E-mail: joshua.brody@mssm.edu [Division of Hematology and Medical Oncology, Icahn School of Medicine at Mount Sinai, New York, NY 10029 (United States)

    2015-04-30

    While the cellular origin of lymphoma is often characterized by chromosomal translocations and other genetic aberrations, its growth and development into a malignant neoplasm is highly dependent upon its ability to escape natural host defenses. Neoplastic cells interact with a variety of non-malignant cells in the tumor milieu to create an immunosuppressive microenvironment. The resulting functional impairment and dysregulation of tumor-associated immune cells not only allows for passive growth of the malignancy but may even provide active growth signals upon which the tumor subsequently becomes dependent. In the past decade, the success of immune checkpoint blockade and adoptive cell transfer for relapsed or refractory lymphomas has validated immunotherapy as a possible treatment cornerstone. Here, we review the mechanisms by which lymphomas have been found to evade and even reprogram the immune system, including alterations in surface molecules, recruitment of immunosuppressive subpopulations, and secretion of anti-inflammatory factors. A fundamental understanding of the immune evasion strategies utilized by lymphomas may lead to better prognostic markers and guide the development of targeted interventions that are both safer and more effective than current standards of care.

  14. Lymphatic system and lymphoma

    Institute of Scientific and Technical Information of China (English)

    2009-01-01

    2009236 Clinical significance in detection of immunoglobulin heavy chain clonal rearrangement in bone marrow of patients with B cell lymphoma.CHEN Zhiyu(陈治宇),et al.Dept Med Oncol,Cancer Hosp,Fudan Univ;Dept Oncel,Shanghai Med Coll,Fudan Univ,Shanghai 200032,Chin J Oncol,2009;3193):183-188.

  15. Lymphatic system and lymphoma

    Institute of Scientific and Technical Information of China (English)

    1997-01-01

    970385 The changes of cell immune function in ap-tients with non-Hodgkin’s lymphoma by flow cytome-try analysis. LU Ming(吕鸣), et al. Clin ImmunolCenter, Changzheng Hosp, 2nd Milit Med Univ, Shang-hai, 200003. Shanghai Med J 1997; 20(2): 73-75.

  16. Nuclear overexpression of lymphoid-enhancer-binding factor 1 identifies chronic lymphocytic leukemia/small lymphocytic lymphoma in small B-cell lymphomas.

    Science.gov (United States)

    Tandon, Bevan; Peterson, Loann; Gao, Juehua; Nelson, Beverly; Ma, Shuo; Rosen, Steven; Chen, Yi-Hua

    2011-11-01

    Lymphoid-enhancer-binding factor 1 (LEF1), coupling with β-catenin, functions as a key nuclear mediator of WNT/β-catenin signaling, which regulates cell proliferation and survival. LEF1 has an important role in lymphopoiesis, and is normally expressed in T and pro-B cells but not mature B cells. However, gene expression profiling demonstrates overexpression of LEF1 in chronic lymphocytic leukemia, and knockdown of LEF1 decreases the survival of the leukemic cells. So far, the data on LEF1 expression in B-cell lymphomas are limited. This study represents the first attempt to assess LEF1 by immunohistochemistry in a large series (290 cases) of B-cell lymphomas. Strong nuclear staining of LEF1 was observed in virtually all neoplastic cells in 92 of 92 (100%) chronic lymphocytic leukemia/small lymphocytic lymphomas including two CD5- cases, with strongest staining in cells with Richter's transformation. LEF1 also highlighted the morphologically inconspicuous small lymphocytic lymphoma component in three composite lymphomas. All 53 mantle cell lymphomas, 31 low-grade follicular lymphomas and 31 marginal zone lymphomas, including 3 CD5+ cases, were negative. In 12 grade 3 follicular lymphomas, LEF1 was positive in a small subset (5-15%) of cells. Diffuse large B-cell lymphoma, however, demonstrated significant variability in LEF1 expression with overall positivity in 27 of 71 (38%) cases. Our results demonstrate that nuclear overexpression of LEF1 is highly associated with chronic lymphocytic leukemia/small lymphocytic lymphoma, and may serve as a convenient marker for differential diagnosis of small B-cell lymphomas. The expression of β-catenin, the coactivator of LEF1 in WNT signaling, was examined in 50 chronic lymphocytic leukemia/small lymphocytic lymphomas, of which 44 (88%) showed negative nuclear staining. The findings of universal nuclear overexpression of LEF1 but lack of nuclear β-catenin in the majority of chronic lymphocytic leukemia/small lymphocytic

  17. An uncommon clinical presentation of retroperitoneal non-Hodgkin lymphoma successfully treated with chemotherapy: A case report

    Institute of Scientific and Technical Information of China (English)

    Chiara Fulignati; Pietro Pantaleo; Greta Cipriani; Marianna Turrini; Rosalia Nicastro; Roberto Mazzanti; Bruno Neri

    2005-01-01

    We report the case of apetient affedted by an extra-nodal non-Hodgkin lymphoma presenting as a unique, large retroperitoneal mass with an unusual clinical presentation mimicking gastric peptic or neoplastic disease. The patient was successfully treated with a first generation therapy, CHOP modified regimen (cyclophosphamide 600 mg/m2 intravenously on d 1, epirubicin 55 mg/m2 intravenously on d 1, vincristine 1.2 mg/m2 intravenously on d 1, prednisone 60 mg/m2 on d 1-5), and a complete response was achieved. The (18)F-fluorodeoxyglucose positron emission tomography was used to assess the therapy outcome. A brief review of literature is provided.

  18. Total skin electron beam and total nodal irradiation for treatment of patients with cutaneous T-cell lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Micaily, B.; Vonderheid, E.C.; Brady, L.W.; Andrews, C.

    1985-06-01

    Sixteen patients with advanced cutaneous T-cell lymphoma (CTCL) with or without lymph node involvement, but without evidence of extranodal manifestations, were treated with a combination of total skin electron beam therapy (TSEB) and total nodal irradiation (TNI). Fourteen (87%) patients achieved a complete response (CR) lasting from 1 to 84+ months (median, 8+ months) from the completion of treatment. The best results occurred in 6 patients with pretumorous intracutaneous CTCL (Stages IB and IIA) where the CR has lasted in all patients from 8 to 84+ months (median about 27+ months). Radiotherapy was well tolerated with the major toxicity being bone marrow suppression. The authors conclude that combined TSEB and TNI is a relatively safe and effective treatment for patients with CTCL prior to the development of lymph node involvement. Long-term follow-up is needed to assess the curative potential of this treatment.

  19. Four Lymphomas in 1 Patient: A Unique Case of Triple Composite Non-Hodgkin Lymphoma Followed by Classical Hodgkin Lymphoma.

    Science.gov (United States)

    Tennese, Alysa; Skrabek, Pamela J; Nasr, Michel R; Sekiguchi, Debora R; Morales, Carmen; Brown, Theresa C; Weisenburger, Dennis D; Perry, Anamarija M

    2017-05-01

    Composite lymphomas consist of 2 or more distinct lymphomas occurring in a single anatomical site or simultaneously in different sites and can be composed of any combination of B-cell non-Hodgkin lymphoma (NHL), T-cell NHL, or Hodgkin lymphoma (HL). Cases of composite lymphomas with more than 2 lymphomas are extremely rare, with only 4 reports in the literature. We report the case of a 49-year-old man with a triple composite lymphoma in a single lymph node, consisting of small lymphocytic lymphoma, follicular lymphoma, and mantle cell lymphoma in situ. The patient received multiple courses of chemotherapy and an autologous stem cell transplant, which resulted in complete remission. Then, 6 years after the stem cell transplant, he developed classical HL. This unique case is, to our knowledge, the first report of a patient with triple composite lymphoma consisting of 3 small mature B-cell NHLs, who subsequently developed a fourth lymphoma.

  20. Computed tomography of the liver in newly diagnosed Hodgkin disease and non-Hodgkin lymphoma: Staging implications

    Energy Technology Data Exchange (ETDEWEB)

    Neumann, C.H.; Hussain, S.; Seltzer, S.E.; Chiles, C.; Castellino, R.A.

    1986-02-01

    In newly diagnosed patients with Hodgkin disease and non-Hodgkin lymphoma, the value of computed tomography (CT) of the liver was assessed as regards impact on the staging process. 201 patients at two medical centers had pretreatment abdominal CT within two weeks of liver biopsy. CT sensitivity, specificity and accuracy in both groups were determined and sensitivity in both groups was very low (8%). If liver biopsy results had been omitted, reliance on CT and other clinical staging procedures alone would have led to important staging errors in 18 of these 201 patients (9%) - overstaging would have occurred twice and understaging 16 times. In 7 additional patients, the lack of demonstration by CT of documented liver disease was without clinical consequence because disseminated extranodal lymphoma was visible at other sites or at extrahepatic regions of the same CT scan. In patients with newly diagnosed Hodgkin disease and non-Hodgkin lymphoma, CT is an unreliable indicator of liver status and cannot replace liver biopsy in supplying the data required for optimal management.

  1. A clinicopathologic study of mantle cell lymphoma in a single center study in India

    Directory of Open Access Journals (Sweden)

    Gujral S

    2008-07-01

    Full Text Available We present clinical features, histopathology and results of treatment in cases of mantle cell lymphoma (MCL at our hospital. We had 93 cases (2.1% of MCL out of total 4301 cases of non-Hodgkin′s lymphoma (NHL in a 4-year period. It included 68 cases (1.7% of MCL from 3987 cases of NHL diagnosed on histopathology. Remaining 25 cases (7.9% diagnosed solely on peripheral blood examination were excluded. Thirty-six (85% patients had advanced-stage disease. Sixty-three were nodal and five were extranodal (all gastrointestinal tract. Common patterns were diffuse (64%, nodular (25% and mantle zone type (11%. Sixty-two cases had lymphocytic while six had blastic morphology (all nodal. Tumor cells expressed CD20 (100%, CD43 (94%, CD5 (89% and cyclin D1 (85%. Bone marrow was involved in 25 (59% cases. Thirty-two patients could be treated. Median recurrence-free survival was 22.23 months. Diffuse pattern of nodal involvement had a lower overall survival.

  2. Asymptomatic Multiple Lymphomatous Polyposis Identified during Staging Bidirectional Endoscopy of Mantle Cell Lymphoma

    Directory of Open Access Journals (Sweden)

    Sonja P. Dawsey

    2016-10-01

    Full Text Available Multiple lymphomatous polyposis (MLP as an extranodal manifestation of mantle cell lymphoma (MCL in the gastrointestinal tract is rare and not often reported in the literature. We describe the case of a 63-year-old female with asymptomatic MLP found during staging bidirectional endoscopy of MCL. The patient presented only with dyspnea, but was found on physical exam to have diffuse lymphadenopathy, and subsequent positron emission tomography (PET CT showed extensive lymph node adenopathy consistent with lymphoma. Excisional lymph node biopsy revealed high-risk MCL. Prior to therapy, staging bidirectional endoscopy was performed, which revealed duodenal bulb polyps and diffuse polyposis in the colon. Biopsies showed atypical lymphoid infiltrate identical to the initial excisional lymph node biopsy. The patient underwent aggressive induction therapy, chemotherapy and bone marrow transplantation. Four months later, repeat colonoscopy and biopsies showed normal mucosa, and repeat PET CT showed no evidence of systemic disease. Eight months later, the patient began having symptoms consistent with cauda equina syndrome, and she was found to have leptomeningeal recurrence of MCL. In spite of other medical treatment, the patient’s MCL progressed and she passed away 3 years after the initial presentation.

  3. A clinicopathologic study of mantle cell lymphoma in a single center study in India.

    Science.gov (United States)

    Gujral, S; Agarwal, A; Gota, V; Nair, R; Gupta, S; Pai, S K; Sanger, M; Shet, T; Subramanian, P G; Muckaden, M; Laskar, S

    2008-01-01

    We present clinical features, histopathology and results of treatment in cases of mantle cell lymphoma (MCL) at our hospital. We had 93 cases (2.1%) of MCL out of total 4301 cases of non-Hodgkin's lymphoma (NHL) in a 4-year period. It included 68 cases (1.7%) of MCL from 3987 cases of NHL diagnosed on histopathology. Remaining 25 cases (7.9%) diagnosed solely on peripheral blood examination were excluded. Thirty-six (85%) patients had advanced-stage disease. Sixty-three were nodal and five were extranodal (all gastrointestinal tract). Common patterns were diffuse (64%), nodular (25%) and mantle zone type (11%). Sixty-two cases had lymphocytic while six had blastic morphology (all nodal). Tumor cells expressed CD20 (100%), CD43 (94%), CD5 (89%) and cyclin D1 (85%). Bone marrow was involved in 25 (59%) cases. Thirty-two patients could be treated. Median recurrence-free survival was 22.23 months. Diffuse pattern of nodal involvement had a lower overall survival.

  4. Fludarabine Phosphate, Melphalan, and Low-Dose Total-Body Irradiation Followed by Donor Peripheral Blood Stem Cell Transplant in Treating Patients With Hematologic Malignancies

    Science.gov (United States)

    2016-10-26

    Accelerated Phase Chronic Myelogenous Leukemia; Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Grade III Lymphomatoid Granulomatosis; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Aplastic Anemia; Burkitt Lymphoma; Childhood Acute Lymphoblastic Leukemia in Remission; Childhood Acute Myeloid Leukemia in Remission; Childhood Chronic Myelogenous Leukemia; Childhood Diffuse Large Cell Lymphoma; Childhood Grade III Lymphomatoid Granulomatosis; Childhood Immunoblastic Large Cell Lymphoma; Childhood Myelodysplastic Syndromes; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Chronic Myelomonocytic Leukemia; Chronic Phase Chronic Myelogenous Leukemia; Congenital Amegakaryocytic Thrombocytopenia; Diamond-Blackfan Anemia; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Juvenile Myelomonocytic Leukemia; Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable; Nodal Marginal Zone B-cell Lymphoma; Paroxysmal Nocturnal Hemoglobinuria; Peripheral T-cell Lymphoma; Polycythemia Vera; Post-transplant Lymphoproliferative Disorder; Previously Treated Myelodysplastic Syndromes; Primary Myelofibrosis; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell

  5. C-MYC and BCL2 translocation frequency in diffuse large B-cell lymphomas: A study of 97 patients

    Directory of Open Access Journals (Sweden)

    Bahar Akkaya

    2016-01-01

    Full Text Available Purpose: Diffuse large B-cell lymphoma (DLBCL is an aggressive non-Hodgkin lymphoma with marked biologic heterogeneity. MYC and BCL2 rearrangements have been reported in a proportion of DLBCLs, where they may be associated with an adverse clinical outcome. The aim of this study was to determine the frequency of MYC and BCL2 translocations in DLBCL and assess the prognostic impact in DLBCL patients. Materials and Methods:   In the present study, we evaluated the expression patterns of CD 10, BCL6, and MUM 1 by immunohistochemistry in 121 cases with DLBCL in tissue microarray (TMA: 62 cases in germinal center B-cells (GCBs; and 59 cases in activated B-cells (ABCs of which 60 were females and 61 were males. MYC and BCL2 rearrangements were investigated by interphase fluorescence in situ hybridization on TMAs in 97 DLBCLs. Result: MYC rearrangements were observed in 11 of 97 cases. There was no association with other clinical features, including age, sex, and nodal/extranodal disease. MYC rearrangement was associated with significantly worse overall survival (P < 0.01. BCL2 rearrangements were observed in 14 of 97 cases. There was no association with other clinical features including age and sex. BCL2 rearrangement had a worse outcome (P < 0.01. MYC and BCL2 rearrangements were observed in 3 of 97 cases with the age of  53 (female, 53, 63 years old, respectively, died in 24, 18, and 35 months after the diagnosis. Two cases had primary nodal and one case primary extranodal presentations. All these patients had stage IV disease. Conclusion: We concluded that C-MYC and BCL2 may contribute to aggressive transformation, and more mechanism-based therapy should be explored. Targeted therapies involving these rearrangements and its associated pathways may change the fate of DLBCLs. Analysis of MYC gene rearrangement along with BCL2 is critical in the identification of high-risk patients with poor prognosis.

  6. Treatment Options for Childhood Hodgkin Lymphoma

    Science.gov (United States)

    ... Hodgkin lymphoma. Lymphocyte-depleted Hodgkin lymphoma. Epstein-Barr virus infection increases the risk of childhood Hodgkin lymphoma. ... about health care. Reviewers and Updates Editorial Boards write the PDQ cancer information summaries and keep them ...

  7. General Information about Primary CNS Lymphoma

    Science.gov (United States)

    ... Research Primary CNS Lymphoma Treatment (PDQ®)–Patient Version General Information About Primary CNS Lymphoma Go to Health ... start in the eye (called ocular lymphoma). Enlarge Anatomy of the lymph system, showing the lymph vessels ...

  8. ProMACE-C-MOPP in aggressive non-Hodgkin's lymphoma. Long-term results in 45 patients treated in a single institution.

    Science.gov (United States)

    Carrión, J R; Delgado, J R; Dominguez, S; Flores, E; Garcia, P; Jaen, J; Santos, J A

    1991-01-01

    Forty-five previously untreated patients with intermediate or high-grade non-Hodgkin's lymphoma were treated with the Pro-MACE-C-MOPP regimen (flexitherapy). The median age of the patients was 51 years, 51% had constitutional symptoms, 78% were in Ann Arbor stage III-IV, 40% had two or more involved extranodal sites and 87% had serum lactate dehydrogenase (LDH) above 225 U/l. Twenty-two (49%) patients had immunoblastic lymphoma (Working Formulation). Overall, 40% of the patients attained complete response (CR) and there were no relapses. The dose-limiting toxicity was myelosuppression (69% of the patients with WBC less than 1.9 x 10(9)/l). Three deaths were attributed primarily to chemotherapy, but another two patients died of long-term complications of therapy. After a median follow-up of 50 months (18-80), 15 patients (33%) were alive without lymphoma. Only histologic subtype (intermediate vs. high) and abdominal involvement were prognostic factors for CR rate. Our results indicate that ProMACE-C-MOPP is an effective regimen for intermediate-grade lymphomas. However, in high-risk patients the regimen seems to be less effective than originally reported.

  9. Intravascular large B-cell lymphoma involving mainly the uterus: report of a case using liquid-based cytology of the endometrium.

    Science.gov (United States)

    Fujiwara, Arisa; Nagayama, Shiho; Amada, Satoshi; Shimamoto, Tomihiro; Shimao, Yoshiya; Hayashi, Tohru

    2010-01-01

    Intravascular lymphoma is a rare subtype of extranodal lymphoma. Most instances of the disease are of B-cell lineage. Diagnosis is difficult because of its nonspecific clinical signs, and many cases are diagnosed at autopsy. Uterine involvement is rare, and it is commonly manifested as genital bleeding. In this case, the chief complaint was fever, which is also very rare. A 62-year-old woman presented with fever of unknown origin. Computed tomography revealed no localized lesion except for swelling of the right internal iliac nodes. A cytologic smear of the endometrium by liquid-based cytology demonstrated malignant cells. Based on the curettage material, the lesion was diagnosed as an undifferentiated malignant tumor. Total abdominal hysterectomy with bilateral salpingo-oophorectomy and pelvic/paraaortic lymphadenectomy revealed widely scattered lymphoma cells of B-cell lineage mainly in the vascular lumina of the uterus, right ovary and lymph nodes. The final histologic type was established on the basis of the surgical material of hysterectomy. Diagnosis was difficult because of prominent cellular atypia and rare location of the tumor. Immunocytochemical examination of liquid-based samples can lead to a correct diagnosis of malignant lymphoma, even at the stage of endometrial cytologic examination.

  10. Marginalization of the Youth

    DEFF Research Database (Denmark)

    Jensen, Niels Rosendal

    2009-01-01

    The article is based on a key note speach in Bielefeld on the subject "welfare state and marginalized youth", focusing upon the high ambition of expanding schooling in Denmark from 9 to 12 years. The unintended effect may be a new kind of marginalization.......The article is based on a key note speach in Bielefeld on the subject "welfare state and marginalized youth", focusing upon the high ambition of expanding schooling in Denmark from 9 to 12 years. The unintended effect may be a new kind of marginalization....

  11. Expression and clinic significance of gene-associated lymphoma of salivary glands%涎腺非霍奇金淋巴瘤相关基因表达及意义

    Institute of Scientific and Technical Information of China (English)

    蔡杰; 张品南; 郭群

    2012-01-01

    Objective: To investigate clinicopathological and genetic characteristics of Lymphoma of salivary glands. Methods: Clinical, morphological and immunohistochemical feature of 32 cases of lymphoma in salivary glands were studied,including 5 cases of rective lymphoid hyperplasia and 32 lymphomas, retrospectivily. Classification of the lymphomas were made assording to the WHO classification of tumors of haemtopoietic and lymphoid tissues. All cases were studied with interphase fluorescence in situ hybridization (FISH) using dual color break apart probes of IgH, MALT1, bc1-6, c-myc, bc1-2, CCND1, bc1-10, and FOXP1 for detection of chromosomal aberrations, involving IgH, MALT1, bcl-6, c-myc, bcl-2, cyclinDl, bcl-10 and F0XP1 gene, repectively. FISH with IgH/bc1-2 dual fusion probe was used for detection of t (14 ; 18)(q32; q21)/IgH-bc1-2. CEP18 sepctrum orange probe was used for detection of aneuploidy of the chromosome 18. Results: Among 32 cases of lymphoma, 28 cases (87.5%) were extranodal marginal zone B-cell lymphoma of mucosa associated lymphoid tissue (MALT lymphoma), 2 cases were fol-licular lymphoma (FL) and 2 cases diffuse large B-cell lymphoma (DLBCL). Among the 28 cases of MALT lymphoma, chromosomal aberration were found in 60.7% (17/28) with interphase FISH analysis. One case showed positive IgH break-apart signal with unknown partner, 16 cases showed three copies of different genes, of which, three copies of MALT1, bcl-6, and c-myc were identified in 7 cases (25%), 12 cases (43%),and 2 cases (8%) of MALT lymphomas, respectively. In addition, 5 cases showed two genes, including three copies of bcl-6 and MALT1 in 4 cases, and three copies of bcl-6 together with c-myc in one case. Furthermore, all cases with three copies of MALT1 had trisomy 18(14; 18) (q32; q2l)was detected in both follicular lymphomas. Of the 2 DLBCL cases, one showed three copies of bc1-6 together with trisomy18 and the other one showed three copies of bcl-6 together with IgH and c

  12. Multimodality imaging of cardiothoracic lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Carter, Brett W., E-mail: bcarter2@mdanderson.org [The University of Texas MD Anderson Cancer Center, Department of Diagnostic Radiology, Section of Thoracic Imaging, 1515 Holcombe Blvd., Unit 1478, Houston, TX 77030 (United States); Wu, Carol C. [Department of Radiology, Massachusetts General Hospital, 55 Fruit Street, FND-202, Boston, MA 02114 (United States); Khorashadi, Leila [Department of Radiology, Mount Auburn Hospital, Cambridge, MA 02138 (United States); Godoy, Myrna C.B.; Groot, Patricia M. de [The University of Texas MD Anderson Cancer Center, Department of Diagnostic Radiology, Section of Thoracic Imaging, 1515 Holcombe Blvd., Unit 1478, Houston, TX 77030 (United States); Abbott, Gerald F. [Department of Radiology, Massachusetts General Hospital, 55 Fruit Street, FND-202, Boston, MA 02114 (United States); Lichtenberger III, John P. [Department of Radiology, David Grant Medical Center, Travis AFB, CA 94535 (United States)

    2014-08-15

    Lymphoma is the most common hematologic malignancy and represents approximately 5.3% of all cancers. The World Health Organization published a revised classification scheme in 2008 that groups lymphomas by cell type and molecular, cytogenetic, and phenotypic characteristics. Most lymphomas affect the thorax at some stage during the course of the disease. Affected structures within the chest may include the lungs, mediastinum, pleura, and chest wall, and lymphomas may originate from these sites as primary malignancies or secondarily involve these structures after arising from other intrathoracic or extrathoracic sources. Pulmonary lymphomas are classified into one of four types: primary pulmonary lymphoma, secondary pulmonary lymphoma, acquired immunodeficiency syndrome-related lymphoma, and post-transplantation lymphoproliferative disorders. Although pulmonary lymphomas may produce a myriad of diverse findings within the lungs, specific individual features or combinations of features can be used, in combination with secondary manifestations of the disease such as involvement of the mediastinum, pleura, and chest wall, to narrow the differential diagnosis. While findings of thoracic lymphoma may be evident on chest radiography, computed tomography has traditionally been the imaging modality used to evaluate the disease and effectively demonstrates the extent of intrathoracic involvement and the presence and extent of extrathoracic spread. However, additional modalities such as magnetic resonance imaging of the thorax and {sup 18}F-FDG PET/CT have emerged in recent years and are complementary to CT in the evaluation of patients with lymphoma. Thoracic MRI is useful in assessing vascular, cardiac, and chest wall involvement, and PET/CT is more accurate in the overall staging of lymphoma than CT and can be used to evaluate treatment response.

  13. Hypothermia & Hodgkin lymphoma in children

    OpenAIRE

    Köse, Doğan; Köksal, Yavuz; Çalışkan, Ümran

    2016-01-01

    Hypothermia associated with Hodgkin lymphoma is defined rarely. This may be caused by a dysfunction that shall occur in hypothalamus, central and peripheral vascular system, skin and muscles. In this study, two Hodgkin lymphoma cases with developed hypothermia are presented. Case 1: An “Hodgkin lymphoma, mixed cellular type” was diagnosed by a biopsy conducted due to lesions found in her spleen on a girl in 7 ages, who applied to the hospital with complaints such as fever, weight loss and nig...

  14. High-Dose Chemotherapy With or Without Total-Body Irradiation Followed by Autologous Stem Cell Transplant in Treating Patients With Hematologic Cancer or Solid Tumors

    Science.gov (United States)

    2016-11-07

    Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Nasal Type Extranodal NK/T-cell Lymphoma; Childhood Acute Lymphoblastic Leukemia in Remission; Childhood Acute Myeloid Leukemia in Remission; Childhood Burkitt Lymphoma; Childhood Diffuse Large Cell Lymphoma; Childhood Immunoblastic Large Cell Lymphoma; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor (PNET); Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Peripheral T-cell Lymphoma; Plasma Cell Neoplasm; Primary Systemic Amyloidosis; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Childhood Anaplastic Large Cell Lymphoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2

  15. Primary parotid B-cell lymphoma successfully treated with chemotherapy plus highly active antiretroviral therapy with prolonged survival and immune reconstitution in an acquired immunodeficiency syndrome patient: Case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Marcelo Corti

    2014-01-01

    Full Text Available Non-Hodgkin′s lymphoma (NHL is the second most common acquired immunodeficiency syndrome (AIDS-defining cancer. In this population, up to 70-80% of cases may present as extranodal location as the primary clinical manifestation of the neoplasm disease. Gastrointestinal tract is the most frequent location of AIDS-associated NHL. However, salivary gland involvement, including the parotid gland is a rare complication in human immunodeficiency virus (HIV-patients. Here, we describe a patient seropositive for the HIV, who developed a primary NHL of the parotid gland histologically classified as a high-grade diffuse large B-cell lymphoma. Patient was treated with a combination of chemotherapy plus highly active antiretroviral therapy with a good clinical, virological and immunological response and a prolonged survival, more than 5 years, without evidence of neoplasm relapse.

  16. Lymphoma-associated dysimmune polyneuropathies.

    Science.gov (United States)

    Stübgen, Joerg-Patrick

    2015-08-15

    Lymphoma consists of a variety of malignancies of lymphocyte origin. A spectrum of clinical peripheral neuropathy syndromes with different disease mechanisms occurs in about 5% of lymphoma patients. There exists a complex inter-relationship between lymphoproliferative malignancies and autoimmunity. An imbalance in the regulation of the immune system presumably underlies various immune-mediated neuropathies in patients with lymphoma. This article reviews lymphoma and more-or-less well-defined dysimmune neuropathy subgroups that are caused by humoral and/or cell-mediated immune disease mechanisms directed against known or undetermined peripheral nerve antigens.

  17. Atypical Epstein-Barr viral genomic structure in lymphoma tissue and lymphoid cell lines.

    Science.gov (United States)

    Tang, Weihua; Fan, Hongxin; Schroeder, Jane; Dunphy, Cherie H; Bryant, Ronald J; Fedoriw, Yuri; Gulley, Margaret L

    2013-06-01

    Epstein-Barr virus (EBV) DNA is found within the malignant cells of some subtypes of lymphoma, and viral presence is being exploited for improved diagnosis, monitoring, and management of affected patients. Recent work suggests that viral genomic polymorphism, such as partial deletion of the viral genome, could interfere with virus detection in tumor tissues. To test for atypical forms of the EBV genome, 98 lymphomas and 6 infected cell lines were studied using a battery of 6 quantitative polymerase chain reaction assays targeting disparate sections of EBV DNA. Fifty of the lymphomas (51%) had no amplifiable EBV DNA, and 38 lymphomas (39%) had low-level EBV infection that was deemed incidental based on EBV-encoded RNA (EBER) in situ hybridization results. The remaining 10 lymphomas (10%) had high EBV loads and EBER localization to malignant cells by EBER in situ hybridization. All 10 represented lymphoma subtypes were previously associated with EBV (Burkitt, diffuse large B-cell, or T-cell type), whereas no remnants of EBV were detected in other lymphoma subtypes (follicular, small lymphocytic, mantle cell, or marginal zone type). Interestingly, 4 of the 10 infected lymphomas had evidence of atypical viral genomes, including 3 of 4 infected T-cell lymphomas with aberrant loss of LMP2 amplicons, and a single diffuse large B-cell lymphoma lacking the central part of the viral genome spanning BamH1W, BZLF1, and EBNA1 gene segments. A reasonable screening strategy for infected malignancy involves applying EBER1 and LMP1 quantitative polymerase chain reaction assays and confirming that values exceeding 2000 copies of EBV per 100,000 cells have EBER localization to malignant cells.

  18. Deferasirox in Treating Iron Overload Caused By Blood Transfusions in Patients With Hematologic Malignancies

    Science.gov (United States)

    2014-12-09

    Acute Undifferentiated Leukemia; Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Grade III Lymphomatoid Granulomatosis; Adult Langerhans Cell Histiocytosis; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Contiguous Stage II Adult Burkitt Lymphoma; Contiguous Stage II Adult Diffuse Large Cell Lymphoma; Contiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Contiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Contiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Contiguous Stage II Adult Lymphoblastic Lymphoma; Contiguous Stage II Grade 1 Follicular Lymphoma; Contiguous Stage II Grade 2 Follicular Lymphoma; Contiguous Stage II Grade 3 Follicular Lymphoma; Contiguous Stage II Mantle Cell Lymphoma; Contiguous Stage II Marginal Zone Lymphoma; Contiguous Stage II Small Lymphocytic Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; de Novo Myelodysplastic Syndromes; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Mast Cell Leukemia; Myelodysplastic Syndrome With Isolated Del(5q); Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable; Myeloid/NK-cell Acute Leukemia; Nodal Marginal Zone B-cell Lymphoma; Noncontiguous Stage II Adult Burkitt Lymphoma; Noncontiguous Stage II Adult Diffuse Large Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Noncontiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Noncontiguous Stage II Adult Lymphoblastic Lymphoma

  19. Pathobiology of Hodgkin Lymphoma

    Directory of Open Access Journals (Sweden)

    Pier Paolo Piccaluga

    2011-01-01

    Full Text Available Despite its well-known histological and clinical features, Hodgkin's lymphoma (HL has recently been the object of intense research activity, leading to a better understanding of its phenotype, molecular characteristics, histogenesis, and possible mechanisms of lymphomagenesis. There is complete consensus on the B-cell derivation of the tumor in most cases, and on the relevance of Epstein-Barr virus infection and defective cytokinesis in at least a proportion of patients. The REAL/WHO classification recognizes a basic distinction between lymphocyte predominance HL (LP-HL and classic HL (cHL, reflecting the differences in clinical presentation and behavior, morphology, phenotype, and molecular features. cHL has been classified into four subtypes: lymphocyte rich, nodular sclerosing, with mixed cellularity, and lymphocyte depleted. The borders between cHL and anaplastic large-cell lymphoma have become sharper, whereas those between LP-HL and T-cell-rich B-cell lymphoma remain ill defined. Treatments adjusted to the pathobiological characteristics of the tumor in at-risk patients have been proposed and are on the way to being applied.

  20. Intravascular lymphoma mimicking vasculitis.

    Science.gov (United States)

    Prayson, Richard A

    2016-12-01

    Intravascular lymphoma is a rare malignancy which is characterized by a proliferation of atypical appearing B cells, generally confined to vascular lumina. A tissue biopsy demonstrating the pathology is required to make a diagnosis. The tumor is often disseminated at the time of diagnosis and prognosis is poor, even with aggressive chemotherapy. Neurologic presentations of this neoplasm can be quite varied. This report documents the presence of intravascular lymphoma diagnosed on a brain biopsy in a 60-year-old man. He initially presented 6months before brain biopsy with chest pain and hypotension, warranting coronary artery bypass graft surgery. Four months later, he presented with signs attributed to a stroke (diaphoresis, slumped over in a chair and left hand weakness). He subsequently developed a sudden onset wide-based gait, left leg numbness, word finding difficulties and worsening confusion. A MRI study showed multiple infarcts in the brain, including cerebellum. Invasive angiogram suggested vasculitis. He was started on a course of treatment for presumed central nervous system vasculitis. He continued to develop signs suggestive of ongoing infarct development and a biopsy from the right parietal was taken. The biopsy showed atypical intravascular CD20 positive staining B cells, consistent with intravascular lymphoma. Copyright © 2016 Elsevier Ltd. All rights reserved.

  1. Coordinate suppression of B cell lymphoma by PTEN and SHIP phosphatases

    DEFF Research Database (Denmark)

    Miletic, Ana V; Anzelon-Mills, Amy N; Mills, David M

    2010-01-01

    results in lethal T cell lymphomas, we find that animals lacking PTEN or SHIP in B cells show no evidence of malignancy. However, concomitant deletion of PTEN and SHIP (bPTEN/SHIP(-/-)) results in spontaneous and lethal mature B cell neoplasms consistent with marginal zone lymphoma or, less frequently......, follicular or centroblastic lymphoma. bPTEN/SHIP(-/-) B cells exhibit enhanced survival and express more MCL1 and less Bim. These cells also express low amounts of p27(kip1) and high amounts of cyclin D3 and thus appear poised to undergo proliferative expansion. Unlike normal B cells, bPTEN/SHIP(-/-) B cells...... proliferate to the prosurvival factor B cell activating factor (BAFF). Interestingly, although BAFF availability may promote lymphoma progression, we demonstrate that BAFF is not required for the expansion of transferred bPTEN/SHIP(-/-) B cells. This study reveals that PTEN and SHIP act cooperatively...

  2. Practical Marginalized Multilevel Models.

    Science.gov (United States)

    Griswold, Michael E; Swihart, Bruce J; Caffo, Brian S; Zeger, Scott L

    2013-01-01

    Clustered data analysis is characterized by the need to describe both systematic variation in a mean model and cluster-dependent random variation in an association model. Marginalized multilevel models embrace the robustness and interpretations of a marginal mean model, while retaining the likelihood inference capabilities and flexible dependence structures of a conditional association model. Although there has been increasing recognition of the attractiveness of marginalized multilevel models, there has been a gap in their practical application arising from a lack of readily available estimation procedures. We extend the marginalized multilevel model to allow for nonlinear functions in both the mean and association aspects. We then formulate marginal models through conditional specifications to facilitate estimation with mixed model computational solutions already in place. We illustrate the MMM and approximate MMM approaches on a cerebrovascular deficiency crossover trial using SAS and an epidemiological study on race and visual impairment using R. Datasets, SAS and R code are included as supplemental materials.

  3. The NOTCH pathway is recurrently mutated in diffuse large B-cell lymphoma associated with hepatitis C virus infection.

    Science.gov (United States)

    Arcaini, Luca; Rossi, Davide; Lucioni, Marco; Nicola, Marta; Bruscaggin, Alessio; Fiaccadori, Valeria; Riboni, Roberta; Ramponi, Antonio; Ferretti, Virginia V; Cresta, Stefania; Casaluci, Gloria Margiotta; Bonfichi, Maurizio; Gotti, Manuel; Merli, Michele; Maffi, Aldo; Arra, Mariarosa; Varettoni, Marzia; Rattotti, Sara; Morello, Lucia; Guerrera, Maria Luisa; Sciarra, Roberta; Gaidano, Gianluca; Cazzola, Mario; Paulli, Marco

    2015-02-01

    Hepatitis C virus has been found to be associated with B-cell non-Hodgkin lymphomas, mostly marginal zone lymphomas and diffuse large B-cell lymphoma. Deregulation of signaling pathways involved in normal marginal zone development (NOTCH pathway, NF-κB, and BCR signaling) has been demonstrated in splenic marginal zone lymphoma. We studied mutations of NOTCH pathway signaling in 46 patients with hepatitis C virus-positive diffuse large B-cell lymphoma and in 64 patients with diffuse large B-cell lymphoma unrelated to HCV. NOTCH2 mutations were detected in 9 of 46 (20%) hepatitis C virus-positive patients, and NOTCH1 mutations in 2 of 46 (4%). By contrast, only one of 64 HCV-negative patients had a NOTCH1 or NOTCH2 mutation. The frequency of the NOTCH pathway lesions was significantly higher in hepatitis C virus-positive patients (P=0.002). The 5-year overall survival was 27% (95%CI: 5%-56%) for hepatitis C virus-positive diffuse large B-cell lymphoma patients carrying a NOTCH pathway mutation versus 62% (95%CI: 42%-77%) for those without these genetic lesions. By univariate analysis, age over 60 years, NOTCH2 mutation, and any mutation of the NOTCH pathway (NOTCH2, NOTCH1, SPEN) were associated with shorter overall survival. Mutation of the NOTCH pathway retained an independent significance (P=0.029). In conclusion, a subset of patients with hepatitis C virus-positive diffuse large B-cell lymphoma displays a molecular signature of splenic marginal zone and has a worse clinical outcome.

  4. Danish National Lymphoma Registry

    Directory of Open Access Journals (Sweden)

    Arboe B

    2016-10-01

    Full Text Available Bente Arboe,1 Pär Josefsson,2 Judit Jørgensen,3 Jacob Haaber,4 Paw Jensen,5 Christian Poulsen,6 Dorthe Rønnov-Jessen,7 Robert S Pedersen,8 Per Pedersen,9 Mikael Frederiksen,10 Michael Pedersen,1 Peter de Nully Brown1 1Department of Hematology, Copenhagen University Hospital, Rigshospitalet, 2Department of Hematology, Copenhagen University Hospital, Herlev Hospital, Copenhagen, 3Department of Hematology, Aarhus University Hospital, Aarhus, 4Department of Hematology, Odense University Hospital, Odense, 5Department of Hematology, Aalborg University Hospital, Aalborg, 6Department of Hematology, Roskilde Hospital, Roskilde, 7Department of Hematology, Vejle Hospital, Vejle, 8Department of Hematology, Holstebro Hospital, Holstebro, 9Department of Hematology, Esbjerg Hospital, Esbjerg, 10Department of Hematology, Haderslev Hospital, Haderslev, Denmark Aim of database: The Danish National Lymphoma Registry (LYFO was established in order to monitor and improve the diagnostic evaluation and the quality of treatment of all lymphoma patients in Denmark. Study population: The LYFO database was established in 1982 as a seminational database including all lymphoma patients referred to the departments of hematology. The database became nationwide on January 1, 2000. Main variables: The main variables include both clinical and paraclinical variables as well as details of treatment and treatment evaluation. Up to four forms are completed for each patient: a primary registration form, a treatment form, a relapse form, and a follow-up form. Variables are used to calculate six result quality indicators (mortality 30 and 180 days after diagnosis, response to first-line treatment, and survival estimates 1, 3, and 5 years after the time of diagnosis, and three process quality indicators (time from diagnosis until the start of treatment, the presence of relevant diagnostic markers, and inclusion rate in clinical protocols. Descriptive data: Approximately 23

  5. [Clinical features in DLBCL and translocation BCL2/c-MYC "double hit" lymphoma].

    Science.gov (United States)

    Škunca, Željka; Domimis, Mara; Plninc-Peraica, Ana; Jakšić, Branimir

    2014-06-01

    Diffuse large B-cell lymphoma (DLBCL) is classified as lymphoma and various entities using the gene expression of proteins are classified into three groups. The aim of this study was to clarify the clinical, biological, immunophenotypic and cytogenetic features of DLBCL with translocation t (14; 18) and 8q24/c-MYC. Eleven DLBCL patients with dual translation were monitored during the 2000-2009 period. The characteristics of these patients included morphological, immunohistochemical and cytogenetic analysis. Study results showed that all patients had aggressive characteristics, presence of B symptoms (64%), general patient condition according to ECOG scale ≥ 2 (55%), elevated serum lactate dehydrogenase activity (73%), clinical stage III and IV (82%), extranodal involvement of the disease (73%), and IPI ≥ 2 (73%). Partial remission was achieved in 73% of all patients and all patients (73%) died within a short time. Patients were treated with CHOP and similar protocols (COP, CVP, CNOP), with the addition of MabThera. Immunophenotyping was performed and determined expression of the CD20, CD3, CD10, BCL6 and MUM1 markers. The cytogenetic analysis/fluorescence in situ hybridization revealed complex karyotype changes. Thus, we analyzed the presence of BCL2, BCL6 and c-MYC genes and found eight patients to have BCL2 and c-MYC translocation genes, while three had translocation of the BCL6 and c-MYC genes. Despite appropriate therapy, the patient prognosis is poor. The median survival in these patients was 1.85 years. DLBCL with BCL2 and c-MYC rearrangement of the subgroups of lymphoma is associated with very poor survival. The presence of these two translocations has an aggressive clinical course.

  6. Does Gender Matter in Non-Hodgkin Lymphoma? Differences in Epidemiology, Clinical Behavior, and Therapy

    Directory of Open Access Journals (Sweden)

    Nurit Horesh

    2014-10-01

    Full Text Available Non-Hodgkin lymphoma (NHL is one of the most common hematologic malignancies worldwide. The incidence of NHL has been rising for several decades; however, in the last 20 years, it reached a plateau. NHL incidence among males is significantly higher than in females. In addition to gender itself, gravidity has a protective role against NHL occurrence. Gender also matters in terms of NHL clinical characteristics. For example, female predominance was found in three extra-nodal sites (the breast, thyroid, and the respiratory system occasionally involved in NHL. The diagnosis of NHL during pregnancy is associated with a unique clinical behavior. It is usually diagnosed in the second or third trimester and in advanced stage. Furthermore, the histological subtype is highly aggressive, and reproductive organ involvement is common. The reduced rate of NHL among females may be explained by direct effects of estrogens on lymphoma cell proliferation or by its effect on anti-tumor immune response. Gender has an important role in responsiveness to standard B cell NHL treatment. Among older adults, women benefited more from the addition of the anti-CD20 antibody rituximab to standard chemotherapy regimens. This phenomenon can be explained by the difference in clearance rate of rituximab that was found to be significantly lower among older females than older males. In mantle cell lymphoma, women receiving lenalidomide have higher rates of response. An understanding of the mechanisms responsible for gender-associated NHL differences will ultimately improve the clinical approach, allowing for a more accurate assessment of prognosis and patient-tailored treatment.

  7. Polymyalgia Rheumatica Revealing a Lymphoma: A Two-Case Report

    Directory of Open Access Journals (Sweden)

    Frank Verhoeven

    2016-01-01

    Full Text Available Introduction. Polymyalgia rheumatica (PMR is one of the most common inflammatory rheumatism types in elderly population. The link between cancer and PMR is a matter of debate. Methods. We report two cases of PMR leading to the diagnosis of lymphoma and the growing interest of PET-TDM in this indication. Results. A 84-year-old man known for idiopathic neutropenia presented an inflammatory arthromyalgia of the limb girdle since one month. Blood exams highlighted the presence of a monoclonal B cell clone. Bone marrow concluded to a B cell lymphoma of the marginal zone. He was successfully treated with 0.3 mg/kg/d of prednisone, and response was sustained after 6 months. A 73-year-old man known for prostatic neoplasia in remission for 5 years presented arthromyalgia of the limb girdle since one month. PET-CT revealed bursitis of the hips and the shoulders, no prostatic cancer recurrence, and a metabolically active iliac lymphadenopathy whose pathologic exam concluded to a low grade follicular lymphoma. He was successfully treated with 0.3 mg/kg/d of prednisone. Conclusion. These observations may imply that lymphoma is sometimes already present when PMR is diagnosed and PET-CT is a useful tool in the initial assessment of PMR to avoid missing neoplasia.

  8. Tyrosine phosphorylation in human lymphomas

    NARCIS (Netherlands)

    Haralambieva, E; Jones, M.; Roncador, GM; Cerroni, L; Lamant, L; Ott, G; Rosenwald, A; Sherman, C; Thorner, P; Kusec, R; Wood, KM; Campo, E; Falini, B; Ramsay, A; Marafioti, T; Stein, H; Kluin, PM; Pulford, K; Mason, DY

    2002-01-01

    In a previous study, we showed that the high level of protein tyrosine phosphorylation present in lymphomas containing an anaplastic lymphoma kinase (ALK) can be demonstrated in routinely processed paraffin tissue sections using immunolabelling techniques. In the present study we investigated

  9. Lymphoma risk in systemic lupus

    DEFF Research Database (Denmark)

    Bernatsky, Sasha; Ramsey-Goldman, Rosalind; Joseph, Lawrence

    2014-01-01

    OBJECTIVE: To examine disease activity versus treatment as lymphoma risk factors in systemic lupus erythematosus (SLE). METHODS: We performed case-cohort analyses within a multisite SLE cohort. Cancers were ascertained by regional registry linkages. Adjusted HRs for lymphoma were generated...

  10. Lymphoma of the Urinary Bladder

    Directory of Open Access Journals (Sweden)

    Anthony Kodzo-Grey Venyo

    2014-01-01

    Full Text Available Background. Lymphoma of the urinary bladder (LUB is rare. Aims. To review the literature on LUB. Methods. Various internet databases were used. Results. LUB can be either primary or secondary. The tumour has female predominance; most cases occur in middle-age women. Secondary LUB occurs in 10% to 25% of leukemias/lymphomas and in advanced-stage systemic lymphoma. Less than 100 cases have been reported. MALT typically affects adults older than 60 years; 75% are female. Diffuse large B-cell lymphoma is also common and may arise from transformation of MALT. LUB presents with haematuria, dysuria, urinary frequency, nocturia, and abdominal or back pain. Macroscopic examination of LUBs show large discrete tumours centred in the dome or lateral walls of the bladder. Positive staining of LUB varies by the subtype of lymphoma; B-cell lymphomas are CD20 positive. MALT lymphoma is positively stained for CD20, CD19, and FMC7 and negatively stained for CD5, CD10, and CD11c. LUB stains negatively with Pan-keratin, vimentin, CK20, and CK7. MALT lymphoma exhibits t(11; 18(q21: 21. Radiotherapy is an effective treatment for the MALT type of LUB with no recurrence. Conclusions. LUB is diagnosed by its characteristic morphology and immunohistochemical characteristics. Radiotherapy is a useful treatment.

  11. [Malignant non-Hodgkin's lymphoma].

    Science.gov (United States)

    Bourrier, P; Grodner, F; Ruf, R; Texier, J; Cottencin, R; Cousteau, C; Deslandre, A; Gounant, C; Szpirglas, H; Laufer, J

    1983-01-01

    Rapid regression of all symptoms was obtained after moderate chemotherapy in two women aged 69 and 77 years respectively with malignant non-Hodgkin's lymphomas. Cervico-facial locations of these tumors are discussed in relation to definition, etiology, geographic factors, genetic markers, and associated immunologic disorders. Diagnosis requires a series of explorations including, obviously as a last resort, exploratory cervicotomy. Other regions may be involved and must be investigated, but lesions not affecting lymph nodes occur in only approximately 2 p. cent of patients with cervico-facial malignant non-Hodgkin's lymphoma (approximately 10 p. cent of all malignant non-Hodgkin's lymphomas). Other localizations include the hard palate, gums, sinuses, and salivary glands. Burkitt's lymphoma represents, on the contrary, 30 p. cent of malignant non-Hodgkin's lymphoma seen in European children. The different therapeutic modalities available are discussed.

  12. Ocean margins workshop

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1990-12-31

    The Department of Energy (DOE) is announcing the refocusing of its marine research program to emphasize the study of ocean margins and their role in modulating, controlling, and driving Global Change phenomena. This is a proposal to conduct a workshop that will establish priorities and an implementation plan for a new research initiative by the Department of Energy on the ocean margins. The workshop will be attended by about 70 scientists who specialize in ocean margin research. The workshop will be held in the Norfolk, Virginia area in late June 1990.

  13. "We call ourselves marginalized"

    DEFF Research Database (Denmark)

    Jørgensen, Nanna Jordt

    2014-01-01

    In recent decades, indigenous knowledge has been added to the environmental education agenda in an attempt to address the marginalization of non-western perspectives. While these efforts are necessary, the debate is often framed in terms of a discourse of victimization that overlooks the agency o...... argue that researchers not only need to pay attention to how certain voices are marginalized in Environmental Education research and practice, but also to how learners as agents respond to, use and negotiate the marginalization of their perspectives....

  14. Burkitt lymphoma in adolescents and young adults: management challenges

    Science.gov (United States)

    Dozzo, Massimo; Carobolante, Francesca; Donisi, Pietro Maria; Scattolin, Annamaria; Maino, Elena; Sancetta, Rosaria; Viero, Piera; Bassan, Renato

    2017-01-01

    About one-half of all Burkitt lymphoma (BL) patients are younger than 40 years, and one-third belong to the adolescent and young adult (AYA) subset, defined by an age between 15 and 25–40 years, based on selection criteria used in different reports. BL is an aggressive B-cell neoplasm displaying highly characteristic clinico-diagnostic features, the biologic hallmark of which is a translocation involving immunoglobulin and c-MYC genes. It presents as sporadic, endemic, or epidemic disease. Endemicity is pathogenetically linked to an imbalance of the immune system which occurs in African children infected by malaria parasites and Epstein–Barr virus, while the epidemic form strictly follows the pattern of infection by HIV. BL shows propensity to extranodal involvement of abdominal organs, bone marrow, and central nervous system, and can cause severe metabolic and renal impairment. Nevertheless, BL is highly responsive to specifically designed short-intensive, rotational multiagent chemotherapy programs, empowered by the anti-CD20 monoclonal antibody rituximab. When carefully applied with appropriate supportive measures, these modern programs achieve a cure rate of approximately 90% in the average AYA patient, irrespective of clinical stage, which is the best result achievable in any aggressive lymphoid malignancy to date. The challenges ahead concern the following: optimization of management in underdeveloped countries, with reduction of diagnostic and referral-for-care intervals, and the applicability of currently curative regimens; the development of lower intensity but equally effective treatments for frail or immunocompromised patients at risk of death by complications; the identification of very high-risk patients through positron-emission tomography and minimal residual disease assays; and the assessment in these and the few refractory/relapsed ones of new monoclonals (ofatumumab, blinatumomab, inotuzumab ozogamicin) and new molecules targeting c-MYC and

  15. Iodine I 131 Monoclonal Antibody BC8 Before Autologous Stem Cell Transplant in Treating Patients With Relapsed or Refractory Hodgkin Lymphoma or Non-Hodgkin Lymphoma

    Science.gov (United States)

    2016-06-10

    Recurrent B-Cell Non-Hodgkin Lymphoma; Recurrent Hodgkin Lymphoma; Recurrent T-Cell Non-Hodgkin Lymphoma; Refractory B-Cell Non-Hodgkin Lymphoma; Refractory Hodgkin Lymphoma; Refractory T-Cell Non-Hodgkin Lymphoma

  16. Modern Radiation Therapy for Hodgkin Lymphoma

    DEFF Research Database (Denmark)

    Specht, Lena; Yahalom, Joachim; Illidge, Tim

    2014-01-01

    by the use of limited volumes, based solely on detectable nodal (and extranodal extension) involvement at presentation, using contrast-enhanced computed tomography, positron emission tomography/computed tomography, magnetic resonance imaging, or a combination of these techniques. The International Commission...

  17. Clinical Analysis of 202 Cases of Extranodal NK/T-Cell Lymphoma%结外NK/T细胞淋巴瘤202例临床分析

    Institute of Scientific and Technical Information of China (English)

    马超; 薄剑; 刘婷; 姚盛; 赵瑜

    2014-01-01

    本研究通过对202例结外NK/T细胞淋巴瘤分子生物学特征、治疗及预后状况分析,探讨该病的治疗策略及其预后影响因素.对2005年至2013年我院收治的202例结外NK/T细胞淋巴瘤患者临床及基因异常、治疗及预后状况进行了回顾分析.以Ann Arbor分期、B组症状、国际预后指标(IPI)、乳酸脱氢酶(LDH)、β2微球蛋白及MYC、HXO11、BCL-2基因表达作为研究参数,运用Kaplan-Meier方法进行生存分析,并采用COX回归分析模型进行预后的多变量分析.结果表明,全部病例5年的总生存(overall-survival,OS)率与无事件生存(event-free survival,EFS)率分别为61.9%和53.9%;采用放-化疗综合治疗的患者与使用单纯化学疗法的患者相比,OS率及EFS率均显著提高,两者比较有显著差异(P<0.05);携带BCL-2基因表达将明显降低患者的OS率和EFS率,该结果有统计学差异(P <0.05);COX回归分析模型多变量分析显示,Ann Arbor分期、BCL2基因高表达是独立的预后影响因素.结论:放-化疗综合治疗方法明显优于单纯化疗方法;Ann Arbor分期、BCL-2表达是结外NK/T细胞淋巴瘤独立的预后影响因素.

  18. Gastric Lymphoma with Secondary Trigeminal Nerve Lymphoma: A Case Report

    Directory of Open Access Journals (Sweden)

    Warissara Rongthong

    2017-05-01

    Full Text Available Data supporting the role of radiotherapy in secondary trigeminal nerve lymphoma is scarce. Here, I report the case of 64-year-old Thai male diagnosed as gastric diffuse large B cell lymphoma with secondary trigeminal nerve lymphoma. He had previously received one cycle of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP, followed by five cycles of rituximab plus CHOP (R-CHOP with intrathecal methotrexate (MTX and cytarabine (Ara-C. One month after the last cycle of R-CHOP, he developed a headache and numbness on the left side of his face. MRI revealed thickening of the left trigeminal nerve. He received one intrathecal injection of MTX and Ara-C, followed by systemic chemotherapy. After receiving intrathecal chemotherapy, his symptoms disappeared. Clinical response and MRI studies suggested secondary trigeminal nerve lymphoma. Two months later, our patient’s secondary trigeminal nerve lymphoma had progressed. Salvage whole brain irradiation (36 Gy with boost dose (50 Gy along the left trigeminal nerve was given. Unfortunately, our patient developed heart failure and expired during the radiotherapy session. In conclusion and specific to secondary central nervous system lymphoma (SCNSL, radiotherapy may benefit patients who fail to respond to systemic chemotherapy and palliative treatment. The results this report fail to support the role of radiotherapy in secondary trigeminal nerve lymphoma.

  19. Indian Ocean margins

    Digital Repository Service at National Institute of Oceanography (India)

    Naqvi, S.W.A.

    The most important biogeochemical transformations and boundary exchanges in the Indian Ocean seem to occur in the northern region, where the processes originating at the land-ocean boundary extend far beyond the continental margins. Exchanges across...

  20. Learning unbelievable marginal probabilities

    CERN Document Server

    Pitkow, Xaq; Miller, Ken D

    2011-01-01

    Loopy belief propagation performs approximate inference on graphical models with loops. One might hope to compensate for the approximation by adjusting model parameters. Learning algorithms for this purpose have been explored previously, and the claim has been made that every set of locally consistent marginals can arise from belief propagation run on a graphical model. On the contrary, here we show that many probability distributions have marginals that cannot be reached by belief propagation using any set of model parameters or any learning algorithm. We call such marginals `unbelievable.' This problem occurs whenever the Hessian of the Bethe free energy is not positive-definite at the target marginals. All learning algorithms for belief propagation necessarily fail in these cases, producing beliefs or sets of beliefs that may even be worse than the pre-learning approximation. We then show that averaging inaccurate beliefs, each obtained from belief propagation using model parameters perturbed about some le...

  1. Central nervous system involvement in diffuse large B-cell lymphoma: an analysis of risks and prevention strategies in the post-rituximab era.

    Science.gov (United States)

    Fletcher, Christopher D; Kahl, Brad S

    2014-10-01

    Central nervous system (CNS) relapse in patients with diffuse large B-cell lymphoma (DLBCL) occurs infrequently (approximately 5%), but is almost universally fatal. Controversy exists regarding which factors most reliably identify high risk patients in the post-rituximab era. Clarification is also needed regarding the value of prophylaxis strategies when contemporary rituximab-based chemotherapy regimens (chemoimmunotherapy) are used. A systematic review with focus on the era of chemoimmunotherapy has been performed. Involvement of > 1 extranodal site plus an elevated lactate dehydrogenase level identifies individuals at highest risk (> 20%) for CNS recurrence who merit additional evaluation. Only certain solitary extranodal sites (testis, kidney and breast, but not bones, orbit or epidural space) appear to confer higher risk in patients receiving chemoimmunotherapy. Data from studies employing modern regimens suggest that intrathecal prophylaxis is ineffective even for high risk populations. Systemic prophylaxis (e.g. high dose methotrexate) may be useful, but does not have strong support in the literature. A significant portion of patients with high risk features (˜25%) may already have subclinical CNS disease, which requires alternative detection and treatment strategies. Flow cytometry is a promising approach with increased sensitivity. Widespread use of this approach could redefine what risk and prophylaxis mean. An algorithm for incorporating risk factors, evaluation and treatment is presented.

  2. Lymphatic system and lymphoma

    Institute of Scientific and Technical Information of China (English)

    1993-01-01

    930583 Analysis of therapeutic efficacy of com- bination chemotherapy and adjuvant radiothera-py in 207 cases of diffuse non—Hodgkin’s lym-phoma.YONG Weiben(勇威本),et al.BeijingCancer Res Instit,Beijing,100000. Chin J Hema-tol 1992;13(12):638—640.Two hundred and seven cases of diffuse non—Hodgkin’s lymphoma(D—NHL)were treatedwith combination chemotherapy(cyclophospha-mide,vincristine,procarbazine,prednisone andpingyingmycin or adriamycin)and adjuvant ra-diotherapy.Complete remission(CR)wasachieved in 94 of 207 patients(45.4%),partial

  3. The predictive significance of CD20 expression in B-cell lymphomas

    Directory of Open Access Journals (Sweden)

    Horvat Mateja

    2011-04-01

    Full Text Available Abstract Background In our recent study, we determined the cut-off value of CD20 expression at the level of 25 000 molecules of equivalent soluble fluorochrome (MESF to be the predictor of response to rituximab containing treatment in patients with B-cell lymphomas. In 17.5% of patients, who had the level of CD20 expression below the cut-off value, the response to rituximab containing treatment was significantly worse than in the rest of the patients with the level of CD20 expression above the cut-off value. The proportion of patients with low CD20 expression who might not benefit from rituximab containing treatment was not necessarily representative. Therefore the aim of this study was to quantify the CD20 expression in a larger series of patients with B-cell lymphomas which might allow us to determine more reliably the proportion of patients with the CD20 expression below the cut-off. Methods Cytological samples of 64 diffuse large B-cell lymphomas (DLBCL, 56 follicular lymphomas (FL, 31 chronic lymphocytic leukemias (CLL, 34 mantle cell lymphomas (MCL, 18 marginal zone lymphomas (MZL and 15 B-cell lymphomas unclassified were analyzed for CD20 expression by quantitative four-color flow cytometric measurements using FACSCalibur flow cytometer (BD Biosciences. Results The range of CD20 expression in different B-cell lymphomas was very broad, varying from 2 737 to 115 623 MESF in CLL and 3 549 to 679 577 MESF in DLBCL. However, when we compared the CD20 expression in the groups of patients with DLBCL, FL, MCL, MZL, CLL and B-cell lymphomas unclassified, it was found to be significantly lower (p = 0.002 only in CLL but did not significantly differ in other lymphoma types (p = NS. Fifty-three out of 218 (24.3% patients with B-cell lymphomas had the CD20 expression below the cut-off value. Conclusions The CD20 expression in CLL is significantly lower than in most histological types of mature B-cell lymphomas in which it appears to be comparable

  4. Solvent exposure and malignant lymphoma: a population-based case-control study in Germany

    Directory of Open Access Journals (Sweden)

    Deeg Evelin

    2007-04-01

    Full Text Available Abstract Aims To analyze the relationship between exposure to chlorinated and aromatic organic solvents and malignant lymphoma in a multi-centre, population-based case-control study. Methods Male and female patients with malignant lymphoma (n = 710 between 18 and 80 years of age were prospectively recruited in six study regions in Germany (Ludwigshafen/Upper Palatinate, Heidelberg/Rhine-Neckar-County, Würzburg/Lower Frankonia, Hamburg, Bielefeld/Gütersloh, and Munich. For each newly recruited lymphoma case, a gender, region and age-matched (± 1 year of birth population control was drawn from the population registers. In a structured personal interview, we elicited a complete occupational history, including every occupational period that lasted at least one year. On the basis of job task-specific supplementary questionnaires, a trained occupational physician assessed the exposure to chlorinated hydrocarbons (trichloroethylene, tetrachloroethylene, dichloromethane, carbon tetrachloride and aromatic hydrocarbons (benzene, toluene, xylene, styrene. Odds ratios (OR and 95% confidence intervals (CI were calculated using conditional logistic regression analysis, adjusted for smoking (in pack years and alcohol consumption. To increase the statistical power, patients with specific lymphoma subentities were additionally compared with the entire control group using unconditional logistic regression analysis. Results We observed a statistically significant association between high exposure to chlorinated hydrocarbons and malignant lymphoma (Odds ratio = 2.1; 95% confidence interval 1.1–4.3. In the analysis of lymphoma subentities, a pronounced risk elevation was found for follicular lymphoma and marginal zone lymphoma. When specific substances were considered, the association between trichloroethylene and malignant lymphoma was of borderline statistical significance. Aromatic hydrocarbons were not significantly associated with the lymphoma diagnosis

  5. Burkitt's lymphoma of the duodenum in a patient with AIDS Linfoma de Burkitt do duodeno em um paciente com AIDS

    Directory of Open Access Journals (Sweden)

    Marcelo Corti

    2007-06-01

    Full Text Available Non-Hodgkin's lymphoma of B-cell type is the second most common neoplasm after Kaposi's sarcoma, among patients with human immunodeficiency virus infection. Most non-Hodgkin's lymphoma cases that are associated with acquired immunodeficiency syndrome involve extranodal sites, especially the digestive tract and the central nervous system. We report a case of primary lymphoma of the duodenum in a patient with AIDS. Upper gastrointestinal endoscopy revealed pseudopolypoid masses found in the second portion of the duodenum. A complete diagnostic study including histological, immunohistochemical and virological analyses showed high-grade B-cell Burkitt's lymphoma. The Epstein-Barr virus genome was detected in biopsies by immunohistochemical and in situ hybridization.O linfoma não-Hodgkin de células B é a segunda neoplasia mais comum em pacientes com infecção pelo vírus da imunodeficiência humana depois do sarcoma de Kaposi. A maioria dos casos de linfoma não-Hodgkin associados com a síndrome da imunodeficiência adquirida envolve locais extraganglionares, especialmente o trato digestivo e o sistema nervoso central. Nós relatamos um caso de linfoma primário do duodeno em um paciente com AIDS. Uma endoscopia digestiva alta mostrou massas pseudopolipóides encontradas na segunda porção do duodeno. Um estudo diagnóstico completo incluindo exames histológicos, imunohistoquímicos e virológicos mostrou um linfoma de células B tipo Burkitt. Detectou-se genoma do vírus Epstein-Barr em biópsias por hibridização in situ e imuno-histoquímica.

  6. Clinicopathological correlates of primary central nervous system lymphoma: Experience from a tertiary care center in South India

    Directory of Open Access Journals (Sweden)

    Bimal Patel

    2015-01-01

    Full Text Available Background: Primary central nervous system lymphomas (PCNSL constitute a rare group of extranodal non-Hodgkin′s lymphomas (NHLs. Aim: To study the clinical and immunophenotypic profile of patients with a PCNSL who presented between the years 2000 and 2013 in a tertiary care center in South India. Materials and Methods: This was a retrospective study. Demographic and clinical data were obtained from the clinical case records. Inclusion criteria: Cases of PCNSL involving brain. Exclusion criteria: Cases of PCNSL involving the spinal cord, meninges and orbit as well as intravascular large B-cell lymphoma, lymphomas with evidence of systemic disease or secondary lymphomas. Archived slides and tissue blocks were retrieved. All cases had hematoxylin and eosin stained sections and immunohistochemistry for CD20, CD3, and MIB-1. Additional immunohistochemistry was performed for CD10, BCL6, and MUM1 on paraffin blocks with sufficient tissue. Results: There were a total of 73 cases with the mean age of presentation being 45.9 years (range 8-71 years and with a male predominance (male: female (M:F = 2.3:1. Headache was the commonest presenting complaint. The mean duration of symptoms was 10.6 weeks. All patients were immunocompetent. Most tumors were supratentorial in location. Out of 73 cases, 70 presented with a diffuse large B-cell lymphoma (DLBCL, two with a Burkitt′s lymphoma, and one with a lymphomatoid granulomatosis. Only 51 of the DLBCL cases had sufficient tissue for additional studies. Non-germinal center was the most common phenotype seen in 65.7% (33/51 of cases. Germinal center B-cell (GCB phenotype was seen in 18/51 cases (34.3%. Conclusion: DLBCL constituted the majority of PCNSLs and although non-germinal center was the predominant phenotype, more than a third of the cases were of the GCB phenotype. As the germinal center phenotype is known to have a better prognosis, further studies to explore its relevance in the Asian population are

  7. Gene Therapy in Treating Patients With Human Immunodeficiency Virus-Related Lymphoma Receiving Stem Cell Transplant

    Science.gov (United States)

    2016-12-15

    HIV Infection; Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma; Plasmablastic Lymphoma; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Non-Hodgkin Lymphoma; Recurrent Burkitt Lymphoma; Recurrent Follicular Lymphoma; Stage III Follicular Lymphoma; Stage III Mantle Cell Lymphoma; Stage IV Follicular Lymphoma; Stage IV Mantle Cell Lymphoma

  8. Burkitt lymphoma in adolescents and young adults: management challenges

    Directory of Open Access Journals (Sweden)

    Dozzo M

    2016-12-01

    Full Text Available Massimo Dozzo,1 Francesca Carobolante,1 Pietro Maria Donisi,2 Annamaria Scattolin,1 Elena Maino,1 Rosaria Sancetta,1 Piera Viero,1 Renato Bassan1 1Complex Operative Unit of Hematology, Ospedale dell’Angelo, 2Simple Departmental Operative Unit of Anatomic Pathology, Ospedale Ss. Giovanni e Paolo, Venice, Italy Abstract: About one-half of all Burkitt lymphoma (BL patients are younger than 40 years, and one-third belong to the adolescent and young adult (AYA subset, defined by an age between 15 and 25–40 years, based on selection criteria used in different reports. BL is an aggressive B-cell neoplasm displaying highly characteristic clinico-diagnostic features, the biologic hallmark of which is a translocation involving immunoglobulin and c-MYC genes. It presents as sporadic, endemic, or epidemic disease. Endemicity is pathogenetically linked to an imbalance of the immune system which occurs in African children infected by malaria parasites and Epstein–Barr virus, while the epidemic form strictly follows the pattern of infection by HIV. BL shows propensity to extranodal involvement of abdominal organs, bone marrow, and central nervous system, and can cause severe metabolic and renal impairment. Nevertheless, BL is highly responsive to specifically designed short-intensive, rotational multiagent chemotherapy programs, empowered by the anti-CD20 monoclonal antibody rituximab. When carefully applied with appropriate supportive measures, these modern programs achieve a cure rate of approximately 90% in the average AYA patient, irrespective of clinical stage, which is the best result achievable in any aggressive lymphoid malignancy to date. The challenges ahead concern the following: optimization of management in underdeveloped countries, with reduction of diagnostic and referral-for-care intervals, and the applicability of currently curative regimens; the development of lower intensity but equally effective treatments for frail or

  9. 胃MALT淋巴瘤中API2-MALT1的检测及其临床病理意义%Detection of API2-MALT1 fusion transcript in gastric MALT lymphoma and its clinicopathological significance

    Institute of Scientific and Technical Info