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Sample records for cell histiocytosis mimicking

  1. Oral manifestion of Langerhans cell histiocytosis mimicking inflammation

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    K S Divya

    2014-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare idiopathic disease characterized by the clonal proliferation of Langerhans cells. LCH affects five children per million population. The peak incidence is from 1 to 4 years of age. LCH involves the head and neck region quite commonly. Oral soft tissue lesions are also common. The differential diagnosis of oral LCH includes leukemia, neutropenia, prepubertal periodontitis, hypophosphatasia, fibrous dysplasia, and Papillon-Lefevre syndrome. The prognosis of LCH depends on early detection and appropriate management. Surgical management alone is used in 50% of cases with an additional 23% of the lesions being treated with both surgery and radiation therapy. A case of LCH in a 6-year-old girl involving the mid root level of developing first permanent molar with a floating developing tooth bud of permanent second molar mimicking an inflammation is reported.

  2. Generalized eruptive histiocytosis mimicking leprosy

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    B C Sharath Kumar

    2011-01-01

    Full Text Available Generalized eruptive histiocytosis (GEH is a rare cutaneous histiocytosis that mainly affects adults and presents with multiple symmetric papules on face, trunk, and proximal extremities. GEH is included in type IIa (histiocytes involving cells of dermal dendrocyte lineage of histiocytic disorders. Clinical and pathological correlations are required for differentiating GEH from other histiocytic disorders and from lepromatous leprosy which clinically mimic GEH and is prevalent in India. We report a case of a middle-aged woman who presented with generalized asymptomatic papules and nodules and was treated for leprosy but was finally diagnosed to have GEH after clinical, histopathological, and immunohistochemical correlation. Furthermore, the newer lesions also showed features of progressive nodular histiocytosis.

  3. Generalized eruptive histiocytosis mimicking leprosy.

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    Sharath Kumar, B C; Nandini, A S; Niveditha, S R; Gopal, M G

    2011-01-01

    Generalized eruptive histiocytosis (GEH) is a rare cutaneous histiocytosis that mainly affects adults and presents with multiple symmetric papules on face, trunk, and proximal extremities. GEH is included in type IIa (histiocytes involving cells of dermal dendrocyte lineage) of histiocytic disorders. Clinical and pathological correlations are required for differentiating GEH from other histiocytic disorders and from lepromatous leprosy which clinically mimic GEH and is prevalent in India. We report a case of a middle-aged woman who presented with generalized asymptomatic papules and nodules and was treated for leprosy but was finally diagnosed to have GEH after clinical, histopathological, and immunohistochemical correlation. Furthermore, the newer lesions also showed features of progressive nodular histiocytosis.

  4. Histiocytosis mimicking a pineal gland tumour

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    Gizewski, E.R.; Forsting, M. [Dept. of Neuroradiology, Univ. of Essen (Germany)

    2001-08-01

    We report an unusual case of isolated Langerhans cell histiocytosis of the central nervous system. A 19-year-old man presented with an incomplete ocular palsy. MRI revealed a solitary mass in the pineal gland with marked contrast enhancement. Complete microsurgical excision was followed by local radiotherapy. Histological examination revealed histiocytosis. Unifocal brain involvement by histiocytosis X is rare with few cases in the literature; the most commonly involved areas are the hypothalamus and the pituitary gland. (orig.)

  5. Cutaneous T cell lymphoma mimicking cutaneous histiocytosis: differentiation by flow cytometry.

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    Baines, S J; McCormick, D; McInnes, E; Dunn, J K; Dobson, J M; McConnell, I

    2000-07-01

    A two-year-old, neutered female cross-bred labrador had multiple cutaneous nodules, biopsies of which revealed pathological changes consistent with cutaneous histiocytosis. During a period of one month the dog developed multicentric lymphadenopathy, a retrobulbar mass and masses within the quadriceps and cervical muscles. Fine needle aspiration cytology of the cutaneous nodules and lymph nodes and histological examination of the cutaneous nodules and muscle masses suggested the presence of lymphoblastic lymphoma. A definitive diagnosis of CD8+ T cell lymphoma was achieved by immunophenotyping the tumour cells by flow cytometry.

  6. Langerhans Cell Histiocytosis in an Infant Mimicking a Lymphoma at Presentation

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    Madasu, Anjan; Noor Rana, Asim; Banat, Saleh; Humad, Hani; Mustafa, Rashid; AlJassmi, Abdulrahman Mohd

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder characterized by proliferation and accumulation of clonal dendritic cells with varied clinical presentation and an unpredictable course. We report a 5-month-old infant with LCH who presented with severe respiratory distress, a large mediastinal mass, significant generalized lymphadenopathy, and hepatosplenomegaly. Lymphoma, especially T cell lymphoblastic lymphoma, can present with superior mediastinal syndrome needing urgent empirical therapy without biopsy. However, lack of response prompted a biopsy which confirmed it to be a case of LCH and that leads to appropriate therapy and survival. There have been reports of LCH presenting with isolated mediastinal mass or with generalized lymphadenopathy, but the combined presentation of generalized lymphadenopathy with large mediastinal mass, hepatosplenomegaly, and fever in an infant has rarely been reported. Conclusion. LCH should also be considered in the differential diagnosis of an infant presenting with generalized lymphadenopathy, mediastinal mass, hepatosplenomegaly, and fever. PMID:26587301

  7. Langerhans Cell Histiocytosis in an Infant Mimicking a Lymphoma at Presentation

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    Anjan Madasu

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disorder characterized by proliferation and accumulation of clonal dendritic cells with varied clinical presentation and an unpredictable course. We report a 5-month-old infant with LCH who presented with severe respiratory distress, a large mediastinal mass, significant generalized lymphadenopathy, and hepatosplenomegaly. Lymphoma, especially T cell lymphoblastic lymphoma, can present with superior mediastinal syndrome needing urgent empirical therapy without biopsy. However, lack of response prompted a biopsy which confirmed it to be a case of LCH and that leads to appropriate therapy and survival. There have been reports of LCH presenting with isolated mediastinal mass or with generalized lymphadenopathy, but the combined presentation of generalized lymphadenopathy with large mediastinal mass, hepatosplenomegaly, and fever in an infant has rarely been reported. Conclusion. LCH should also be considered in the differential diagnosis of an infant presenting with generalized lymphadenopathy, mediastinal mass, hepatosplenomegaly, and fever.

  8. Benign isolated histiocytosis mimicking chicken pox in a neonate: report of two cases with ultrastructural study.

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    Valderrama, E; Kahn, L B; Festa, R; Lanzkowsky, P

    1985-01-01

    Two cases of benign isolated cutaneous histiocytosis X in newborn infants are reported. Clinically, the lesions mimicked chicken pox, and the histologic findings in skin biopsies were indistinguishable from those described in infantile Letterer-Siwe disease. Electron microscopy showed Langerhans' cells with the classical trilaminar, racket-shaped granules. Our cases a well as 10 of the 21 previously reported cases showed spontaneous resolution of the skin lesions. Since this is a benign spontaneous resolving histiocytosis, we recommend that no therapy be given provided that no other signs of systemic involvement are found.

  9. Indeterminate cell histiocytosis that presented clinically as benign cephalic histiocytosis.

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    Haimovic, Adele; Chernoff, Karen; Hale, Christopher S; Meehan, Shane A; Schaffer, Julie V

    2014-12-16

    Indeterminate cell histiocytosis (ICH) is a rare, heterogeneous disorder that is characterized by immunophenotypic features of both Langerhans cell histiocytosis (LCH) and non-LCH. We describe a 12-month-old boy with a four-month history of asymptomatic, small, pink-tan papules on his face. Histopathologic evaluation showed a superficial, dermal infiltrate of histiocytes that was positive for S100, CD1a, CD68, and Factor XIIIa. To our knowledge, this represents the first report of the clinical presentation of benign cephalic histiocytosis with immunohistochemical findings of ICH. We review the classification of histiocytic disorders and the clinical and immunohistochemical features of both ICH and benign cephalic histiocytosis.

  10. Langerhans cell histiocytosis

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    Arpita J Nishal

    2013-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare proliferative disorder in which Langerhans′s cells accumulate in a variety of organs. LCH affects mostly children and adolescents, involves one or many body systems or tissues such as bone, lung, hypothalamus, posterior pituitary gland, skin, lymph node, liver, and various soft tissues. The clinical features of LCH depend on the extent of involvement. Here in, we report a case of 2-year-old girl presented with fever, swelling in front and behind of right ear and ear discharge. She had anaemia and lytic lesions on skull x-ray and high resolution computed tomography. Biopsy was performed and histopathology and immunohistochemistry findings suggested diagnosis of LCH.

  11. Histiocytosis

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    ... Chakraborty R, Allen CE. Langerhans cell histiocytosis: emerging insights and clinical implications. Oncology . 2016;30(2):122- ... ADAM Health Solutions. About MedlinePlus Site Map FAQs Customer Support Get email updates Subscribe to RSS Follow ...

  12. Pulmonary langerhans cell histiocytosis

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    Suri Harpreet S

    2012-03-01

    Full Text Available Abstract Pulmonary Langerhans Cell Histiocytosis (PLCH is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events resulting in enhanced recruitment and activation of Langerhans cells in small airways. Bronchiolar inflammation may be accompanied by variable lung interstitial and vascular involvement. While cellular inflammation is prominent in early disease, more advanced stages are characterized by cystic lung destruction, cicatricial scarring of airways, and pulmonary vascular remodeling. Pulmonary function is frequently abnormal at presentation. Imaging of the chest with high resolution chest CT scanning may show characteristic nodular and cystic abnormalities. Lung biopsy is necessary for a definitive diagnosis, although may not be required in instances were imaging findings are highly characteristic. There is no general consensus regarding the role of immunosuppressive therapy in smokers with PLCH. All smokers must be counseled on the importance of smoking cessation, which may result in regression of disease and obviate the need for systemic immunosuppressive therapy. The prognosis for most patients is relatively good, particularly if longitudinal lung function testing shows stability. Complications like pneumothoraces and secondary pulmonary hypertension may shorten life expectancy. Patients with progressive disease may require lung transplantation.

  13. [Adult Langerhans cell histiocytosis].

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    de Menthon, Mathilde; Meignin, Véronique; Mahr, Alfred; Tazi, Abdellatif

    2017-01-01

    Langerhans cell histiocytosis (LCH) is a rare disease affecting both genders and can occur at any age. It often evolves through successive flares, and its severity varies from benign forms that don't require treatment to life threatening disease. Some patients have important functional impairment with psychological and social consequences and prolonged disability. LCH may affect only one organ, with uni- or multifocal involvement or be multisystem disease involving multiple organs. The organs most frequently involved are bones, lung, skin and the endocrinal system. Pulmonary LCH is strongly related to smoking. Some patients have mixed histocytosis combining LCH and other histiocytic disorders. The diagnosis relies on the histological study of tissues samples, and shows tissue infiltration with large cell with pale cytoplasm and reniform nucleus, staining for CD1a and Langerin (CD207) on immunohistochemistry. The BRAF(V600E) mutation is observed in tissue samples in approximately half of patients and the activation of the RAS-RAF-MEK-ERK pathway has been shown to be constantly activated in LCH lesions, regardless the BRAF status. These findings represent an important forward step in the understanding of the physiopathology of the disease. Treatment must be adapted to the severity of the disease and goes from conservative observation to systemic chemotherapy. Therapies targeting the RAS-RAF-MEK-ERK pathway are promising treatments for progressive disease.

  14. Adult Onset Langerhans’ Cell Histiocytosis

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    Rahime İnci

    2014-12-01

    Full Text Available Langerhans’ cell histiocytosis (LCH is a group of diseases of unknown cause resulting from abnormal proliferation of bone marrow-originated dendritic cells called histiocytes. The incidence is between 0.5-5.4 per million. More common in childhood, it is extremely rare in adults. In adults, pulmonary involvement with Langerhans’ cell histiocytosis usually occurs as a single-system disease. In this article, the clinical, radiological and histopathological findings of a 51-year-old male patient with both skin, bone and pulmonary involvement were presented and discussed with recent literature.

  15. CASE REPORT OF LANGERHANS CELL HISTIOCYTOSIS

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    Umesh Reddy

    2014-08-01

    Full Text Available : Langerhans cell histiocytosis (LCH is a rare proliferative disorder in which pathological Langerhans cells (LCs accumulate in a variety of organs. This report describes a boy with Langerhans' cell histiocytosis who presented with primarily soft tissue swellings, without pain. A high index of suspicion is required to reach to a diagnosis of langerhans cell histiocytosis to prevent delay in proper management of this disease.

  16. Early-onset sarcoidosis mimicking refractory cutaneous histiocytosis.

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    Ohga, Shouichi; Ichino, Kiyomi; Urabe, Kazunori; Ishimura, Masataka; Takada, Hidetoshi; Nishikomori, Ryuta; Furue, Masutaka; Hara, Toshiro

    2008-03-01

    A 10-year-old female was diagnosed as having early-onset sarcoidosis (EOS) after a prolonged skin disease. A granuloma emerged on the face at age 2 and massive lesions extended to the rest of the body. Repeated biopsies indicated histiocytic proliferation. At age 7, fever, disseminated macular eruptions, and multinucleated giant cells in the bone marrow prompted vinblastine and prednisolone therapy. Five months after stopping therapy, hypercalcemic crisis occurred along with fever, cytopenias, and interferon-gamma-nemia indicating a macrophage activation syndrome. A biopsy of nodules confirmed the diagnosis of sarcoidosis. The atypical EOS should be differentiated from histiocytosis.

  17. Langerhans Cell Histiocytosis in Otorhinolaryngology

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    Fernandes, Humberto de Barros

    2009-12-01

    Full Text Available Introduction: The Langerhans cell histiocytosis is an inflammatory cells proliferative disorder of unknown etiology. It is uncommon disease in children. Objective: To proceed with a literature review on Langerhans cell histiocytosis, with focus on the otorhinolaryngological complications. Materials and methods: The methodology used was advised based on online data from MEDLINE, between 1966 and 2008, with research of terms related to Langerhans cell histiocytosis, temporal bones and otorhinolaryngology. Literature Review: The manifestations in the head and neck are the most common ones and their diagnosis becomes difficult once it mimetizing other more common diseases the otorhinolaryngologist sees as external ear eczema, acute mastoiditis and gingivitis. The temporal bone disease manifests as recurrent otorrhea and external auditory meatus and retroauricular granulomas. The radiological evaluation confirms lytic lesions especially in the cranial cap, jaw, temporal bones and spines. The definitive diagnosis is made by biopsy through the histopathological discoveries and immunohistochemistry detection of the CD1a antigen. The main form of treatment is by chemotherapy and, in a lower scale, radiotherapy or surgery. Conclusions: he otorhinolaryngological manifestations must be suspected for recurrent otological symptoms and the presence of retroauricular granulation tissue or and the external auditory meatus. The biopsy with characteristic histological discoveries and immunohistochemistry positive for CD1a were diagnostic. The chemotherapy may be the initial treatment in most cases or secondary in refractory or recurrent forms.

  18. Multifocal Skeletal Tuberculosis Mimicking Langerhans Cell Histiocytosis in a Child: a Case Report With a Long-Term Follow-Up

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    Haghighatkhah, Hamidreza; Jafroodi, Yousef; Sanei Taheri, Morteza; Pourghorban, Ramin; Sadeghian Dehkordy, Afarin

    2015-01-01

    Introduction: Multifocal skeletal tuberculosis is a rare condition that may masquerade as Langerhans cell histiocytosis, especially in children. Case Presentation: We report a case of multifocal osseous tuberculosis in a 5-year-old female patient admitted to our hospital with a complaint of low back pain but no history of respiratory symptoms or malaise. Radiological findings included vertebra plana and multiple lytic lesions in both the frontal and pelvic bones. An initial diagnosis of Langerhans cell histiocytosis was made based on imaging findings; however, the patient underwent further evaluation for Mycobacterium tuberculosis, and histopathologic findings confirmed the diagnosis of tuberculosis. The patient showed a nearly complete response after receiving a course of anti-tuberculosis drugs. Conclusions: A high index of suspicion is required for the early diagnosis and prompt treatment of patients with osseous tuberculosis. Given the high prevalence of tuberculosis in developing countries, tuberculosis should be considered in the differential diagnosis of multifocal lytic lesions and vertebra plana, especially in children. PMID:26744631

  19. Cerebral and brain stem Langerhans cell histiocytosis

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    Breidahl, W.H. (Dept. of Radiology, Royal Perth Hospital, Nedlands (Australia)); Ives, F.J. (Dept. of Radiology, Royal Perth Hospital, Nedlands (Australia)); Khangure, M.S. (Dept. of Magnetic Resonance Imaging, Sir Charles Gairdner Hospital, Nedlands (Australia))

    1993-05-01

    Two patients with central nervous system manifestations of Langerhans cell histiocytosis, both with brain stem involvement, are reported. The onset of symptoms was at an age when the diagnosis might not have been considered. (orig.)

  20. Langerhans Cell Histiocytosis Involving Maxilla and Mandible

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    M. Guna Shekhar

    2009-06-01

    Full Text Available Langerhans cell histiocytosis is a relatively rare unique disease process characterized by an abnormal proliferation of immature dendritic cells usually affecting children and young adults. Jaws are involved in less than 10% of children with the disease while mandibular involvement in young children is uncommon and bilateral affection is very rare. The purpose of this report is to describe a unique and very rare case of simultaneous and bilateral occurrence of Langerhans cell histiocytosis in both the jaws of a four-year-old boy.

  1. Langerhans cell histiocytosis of the sacrum

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    M.A. Hatem

    2014-01-01

    Full Text Available Langerhans cell histiocytosis is a rare disease with a wide spectrum of clinical presentations. It is a multisystemic disease with organ system involvement ranging from simple—where it involves only one organ—to widespread progressive disease. Although it can affect any age group, the peak incidence is between 1 and 3 years of age.

  2. Langerhans cell histiocytosis of the sacrum

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    Hatem, M.A.

    2015-01-01

    Langerhans cell histiocytosis is a rare disease with a wide spectrum of clinical presentations. It is a multisystemic disease with organ system involvement ranging from simple—where it involves only one organ—to widespread progressive disease. Although it can affect any age group, the peak incidence is between 1 and 3 years of age. PMID:27186251

  3. [Pulmonary manifestations of Langerhans cell histiocytosis].

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    Obert, J; Tazi, A

    2015-10-01

    Pulmonary Langerhans cell histiocytosis is a rare diffuse cystic interstitial pneumonia of unknown etiology that occurs selectively in young smokers of both genders. The multicenter studies conducted by the reference center have better defined the short and medium terms natural history of the disease and the clinical management of patients. A substantial proportion of patients experience a dramatic decline in their lung function soon after diagnosis. Importantly, smoking cessation is associated with a decreased risk of subsequent deterioration. Cladribine, a purine analogue, chemotherapy may dramatically improve lung function in patients with progressive pulmonary Langerhans cell histiocytosis, but this treatment should be used only in the setting of clinical research. Specific pulmonary hypertension therapies (anti-endothelin receptors, inhibitors of phosphodiesterases) may be used with caution in specialized centres for patients with severe pulmonary hypertension, and seem to be well tolerated. The recent identification of the V600E mutation of the BRAF oncogene in approximately half of the Langerhans cell histiocytosis lesions, including pulmonary granulomas, represents an important step forward in the understanding of the pathogenesis of Langerhans cell histiocytosis. Potentially it opens the way to targeted therapies.

  4. Pericardial Effusion in Langerhans Cell Histiocytosis: A Case Report

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    Gholami, Narges

    2016-01-01

    Introduction Langerhans cell histiocytosis (LCH) is a proliferative disorder of histiocytes in multiple organs. Langerhans cell histiocytosis involves bones, skin, lung and other organs. Case Presentation This study describes a seven-month-old Iranian girl who presented with skin rash and cervical lymphadenopathy. Langerhans cell histiocytosis was suspected when it was associated with anemia, splenomegaly and lytic bone lesions. A skin biopsy confirmed the diagnosis of Langerhans cell histiocytosis. During hospitalization, the patient looked ill with respiratory distress. A chest X-ray showed a ground glass view, and echocardiography showed moderate pericardial effusion. Conclusions Pericardial effusion was a rare finding in this case of Langerhans cell histiocytosis. Pericardial effusion in Langerhans cell histiocytosis, which is an unusual presentation, should be considered when the patient experiences respiratory distress. PMID:27621925

  5. Langerhans' Cell Histiocytosis (Histiocytosis X) of the Temporal Bone

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Objectives Langerhans' Cell histiocytosis (LCH) is a rare disease, which remains poorly understood and whose cellular origin remains unknown. To increase understanding of temporal bone LCH, it is necessary to study recent advances in the diagnosis and treatment of this disease. Methods The long term(5 to 30 years) results of 21 temporal bone LCH cases treated between 1973 and 2003 were reviewed. Surgery, radiotherapy,pharmacologic therapy or a combination of these treatments were employed in these cases. Results Eighteen patients were cured (18/21, 85%). Six patients developed residual diabetes insipidus (DI) and dwarfism (28%).Three patients died (14%). Conclusions The Alessi classification system for LCH based on the extent of disease accurately predicts prognosis and is a useful guide in selecting treatment methodologies. X-ray, computed tomography and magnetic resonance imaging have proved useful in defining the extent of osseous and soft tissue diseases. Diagnosis of LCH is based on clinical presentations, radiographic findings and histopathological results.Surgery and radiotherapy are the main treatment modalities. Pharmacologic therapy should be used in patients with aggressive, disseminate, and refractory lesions. LCH has a predilection for children and prognosis depends on age and extent of vital organ involvement.

  6. Langerhans Cell Histiocytosis: A Case Report

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    Roro Suzy-Indharty

    2012-05-01

    Full Text Available Objective: Langerhans Cell Histiocytosis (LCH is a rare proliferative disorder of a distinct cell type that is similar to Langerhans cell. Prevalence is estimated at 1:200.000/year in children. Clinical presentations are variable, ranging from a single location in the bone to severe multivisceral involvement leading to dysfunction of vital organs. Therefore, objective of this study was to explore LCH based on clinical findings, skull x-ray, head CT-Scan, cytology, histopathology. and Immunohistochemistry.Method: We report a case of Langerhans Cell Histiocytosis of a 3-year-old boy. Fine needle aspiration biopsy was performed from a nodule in parietal area on the scalp; dry fixation was made and stained with Giemsa. Immunohistochemistry examination was performed with CD1a and S100. Histopathology examination was done post-operatively and cytology examination were consistent with LCH, composing Langerhans cells with complex, clefted, grooved, irregular or convoluted nuclei with fine chromatins and one or more small nucleoli and moderate to abundant quantities of eosinophilic cytoplasm, mixed with multinucleated eosinophils.Results: The finding of pentalaminar Birbeck granules by electron microscope is diagnostic of LCH, but this examination cannot be performed in our institution, so diagnosis was supported by immunohistochemistry with CD1a and S 100. Conclusions: Based on clinical findings, skull x ray, Head CT-Scan, cytology, histopathology and Immunohistochemistry, this case was concluded as a Langerhans Cell Histiocytosis.

  7. Langerhans cell histiocytosis of the orbit.

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    Shetty S

    2001-01-01

    Full Text Available Langerhans cell histiocytosis (LCH accounts for less than 1% of all orbital tumours. Though rare, orbital involvement in LCH is not uncommon. Most reports so far have been in Western literature. We report here a case of LCH of orbit with intracranial extension. To the best of our knowledge, this is the first case of single system multifocal LCH reported from India.

  8. Paediatric neuroradiological aspects of Langerhans cell histiocytosis

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    Demaerel, Philippe [University Hospital K.U.Leuven, Department of Radiology, Leuven (Belgium); Gool, Stefaan van [University Hospital K.U.Leuven, Paediatric Haemato-oncology/Neuro-oncology, Leuven (Belgium)

    2008-01-15

    Langerhans cell histiocytosis, previously known as histiocytosis X, is a complex disease consisting of three entities that are all characterized by a proliferation of the Langerhans cell. The clinical course is variable and ranges from a solitary lytic bone or skin lesion with complete remission to a multisystem disorder with possible lethal outcome. The clinical suspicion can be increased based on radiological findings that are important criteria in defining the extent of the disease involvement. A biopsy is often necessary for establishing the final diagnosis. The lytic craniofacial bone lesions are the most common craniospinal abnormality in Langerhans cell histiocytosis. Abnormalities in the hypothalamic-pituitary region are the most frequent manifestations, often accompanied with diabetes insipidus as the presenting symptom. A range of different central nervous system abnormalities can be recognized. It is important to be able to recognize the extensive spectrum of neuroradiological abnormalities in order to arrive at the diagnosis. Neuroimaging plays an even more important role in assessing the response to treatment or possible relapse. (orig.)

  9. Langerhans cell histiocytosis of the temporal bone in a child

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    A Chakravarti

    2011-12-01

    Full Text Available Langerhans cell histiocytosis of temporal bone is a benign tumor like condition with variable clinical course. This rare clinical entity may mimic common ENT clinical condition like otitis media , mastoiditis and otitis externa . A high index of suspicion is required to reach to a diagnosis of langerhans cell histiocytosis to prevent delay in proper management of this disease.

  10. Langerhans cell histiocytosis of bone: MR imaging

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    George, J.C. [Indiana Univ., Indianapolis, IN (United States). Dept. of Radiology; Buckwalter, K.A. [Indiana Univ., Indianapolis, IN (United States). Dept. of Radiology; Cohen, M.D. [Indiana Univ., Indianapolis, IN (United States). Dept. of Radiology; Edwards, M.K. [Indiana Univ., Indianapolis, IN (United States). Dept. of Radiology; Smith, R.R. [Indiana Univ., Indianapolis, IN (United States). Dept. of Radiology

    1994-03-01

    Magnetic resonance (MR) images of 12 pathologically proven lesions of Langerhans cell histiocytosis (LCH) of bone were reviewed retrospectively. MR identified all lesions, three of which were not identified on plain radiographs. In all cases, MR showed greater abnormality than did plain radiographs. With one exception, all lesions were hypointense on T1-weighted images and hyperintense on T2-weighted images. The lesions and associated soft tissue abnormalities were very conspicuous on short TI inversion sequences and T1-weighted post-contrast images. Follow-up MR studies in two patients after chemotherapy showed decreased size and enhancement of lesions compared with baseline studies. (orig.)

  11. Liver involvement in Langerhans cell histiocytosis

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    Wong, Adelaine; Ortiz-Neira, Clara L.; Abou Reslan, Walid; Kaura, Deepak [Alberta Children' s Hospital, Department of Diagnostic Imaging, Calgary, Alberta (Canada); Sharon, Raphael; Anderson, Ronald [Alberta Children' s Hospital, Department of Oncology, Calgary, AB (Canada); Pinto-Rojas, Alfredo [Alberta Children' s Hospital, Department of Pathology, Calgary, AB (Canada)

    2006-10-15

    Liver involvement in Langerhans cell histiocytosis (LCH) typically presents with hepatomegaly and other signs of liver dysfunction. We present an 11-month-old child having only minimally elevated liver enzymes as an indication of liver involvement. Using sonography as the initial diagnostic tool followed by MRI, LCH of the liver was revealed. A review of sonographic, CT, MRI and MR cholangiopancreatography findings in liver LCH is presented. We recommend that physicians consider sonography and MRI screening for liver involvement in patients with newly diagnosed LCH, as periportal involvement may be present with little or no liver function abnormality present, as in this patient. (orig.)

  12. Langerhans cell histiocytosis of bone: MR imaging.

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    George, J C; Buckwalter, K A; Cohen, M D; Edwards, M K; Smith, R R

    1994-01-01

    Magnetic resonance (MR) images of 12 pathologically proven lesions of Langerhans cell histiocytosis (LCH) of bone were reviewed retrospectively. MR identified all lesions, three of which were not identified on plain radiographs. In all cases, MR showed greater abnormality than did plain radiographs. With one exception, all lesions were hypointense on T1-weighted images and hyperintense on T2-weighted images. The lesions and associated soft tissue abnormalities were very conspicuous on short TI inversion sequences and T1-weighted post-contrast images. Follow-up MR studies in two patients after chemotherapy showed decreased size and enhancement of lesions compared with baseline studies.

  13. Langerhans Cell Histiocytosis of the Clavicle

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    Wang, Shaowu; Zhang, Weisheng; Na, Shengbo; Zhang, Lina; Lang, Zhijin

    2014-01-01

    Abstract We report a rare case of solitary Langerhans cell histiocytosis (LCH) involving the clavicle of an adult female. The patient was a 32-year-old female presenting with 1 month history of progressive pain, swelling, and tenderness in the region near the left sternoclavicular joint. Radiograph, computed tomography, and magnetic resonance imaging showed an osteolytic lesion in the clavicle with tumor extension and soft tissue edema. Surgical curettage of the lesion was performed, and the histopathologic diagnosis was LCH. Because of its rarity and possibly variable presentation, LCH should be included and considered in the differential diagnosis when we encounter a clavicle lesion. PMID:25365405

  14. Vulvar Langerhans cell histiocytosis: a case report

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    Khoummane, Nadia; Guimeya, Cyriane; Lipombi, Dominique; Gielen, François

    2014-01-01

    Langerhans cell histiocytoses (LCH) are a rare group of disorders that comprise a large spectrum of diseases initially known as histiocytosis X. In this case report, we relate a case of LCH affecting the vulva of a 47-year-old female. The patient presented since 3 years with a vulvar lesion characterized by non-healing ulcers and a perineal granuloma on which she underwent surgery. Professionals should keep in mind not to treat straightforwardly lesions of the genital tract as simple sexually transmitted diseases. Chronic, atypical genital lesions seen in women need to be worked up and dealt with accordingly. PMID:25404979

  15. Radiologic manifestation of pulmonary Langerhans' cell histiocytosis

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    Kim, Jong Sung; Bang, Duk Ja; Rhim, Hyun Chul; Jeon, Seok Chol; Lee, Seung Ro; Hahm, Chang Kok [Hanyang University College of Medicine, Seoul (Korea, Republic of)

    1993-09-15

    Pulmonary Langerhans' cell histiocytosis is an uncommon granulomatous disorder of unknown cause. The authors retrospectively evaluated radiography and computed tomographic findings of five patients with biopsy proven pulmonary Langerhans' cell histiocytosis. The main structural abnormalities consisted of small nodules and cystic air spaces, but one cases showed only pneumothorax due to bullae rupture. Its distribution has been known predominantly in the upper lung fields, but in our cases, the lung lesions were distributed in the entire lung fields or predominently in the lower lung fields. We propose that pulmonary Langerhans' cell histiocytosis is extremely variable of its structural abnormalities and distribution.

  16. Urticating histiocytosis: a mast cell-rich variant of histiocytosis X.

    Science.gov (United States)

    Foucar, E; Piette, W W; Tse, D T; Goeken, J; Olmstead, A D

    1986-05-01

    Histiocytosis X and mastocytosis are proliferative processes that may have similar cutaneous manifestations. However, a positive Darier's sign (urtication on stroking of the lesion) is thought to reliably distinguish between these two diseases. We recently studied a 13-year-old girl with a 2-year history of extensive skin lesions and a positive Darier's sign. Routine histopathologic studies revealed a polymorphous cutaneous infiltrate composed of histiocytes, mast cells, eosinophils, and lymphoid cells. Electron microscopic studies demonstrated Langerhans granules in some of the histiocytes, and immunologic studies of frozen tissue showed that a significant subpopulation of the histiocytes marked as Langerhans cells. Giemsa staining of specimens from eight other cases of cutaneous histiocytosis X from our files revealed mast cells in all of the lesions, although none showed the abundance of mast cells present in the case with urtication. Our studies emphasize the often polymorphous nature of the cell population in cutaneous histiocytosis X and demonstrate that confusing clinical findings can result when the mast cell population in histiocytosis X produces urtication.

  17. An unusual presentation of Langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Palak Agarwal

    2014-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a relatively rare and unique disease. An incidence of 7.9% in the jaws is reported. We report a case of 9-year-old male child referred to us from dental outpatient department, who presented with a firm swelling in right lower jaw along with bilateral submandibular lymphadenopathy for 1-month. Fine-needle aspiration was done from lytic lesion in the body of mandible and multiple smears were prepared. On the basis of the clinical and cytomorphological findings, a diagnosis of LCH was suggested. The diagnosis was confirmed on histology. Thus, a high possibility of LCH should be considered in children presenting with lytic lesions in head and neck region.

  18. An unusual presentation of Langerhans cell histiocytosis

    Science.gov (United States)

    Agarwal, Palak; Kaushal, Manju

    2014-01-01

    Langerhans cell histiocytosis (LCH) is a relatively rare and unique disease. An incidence of 7.9% in the jaws is reported. We report a case of 9-year-old male child referred to us from dental outpatient department, who presented with a firm swelling in right lower jaw along with bilateral submandibular lymphadenopathy for 1-month. Fine-needle aspiration was done from lytic lesion in the body of mandible and multiple smears were prepared. On the basis of the clinical and cytomorphological findings, a diagnosis of LCH was suggested. The diagnosis was confirmed on histology. Thus, a high possibility of LCH should be considered in children presenting with lytic lesions in head and neck region. PMID:25745295

  19. Langerhans Cell Histiocytosis in Bilateral Mastoid Cavity

    Directory of Open Access Journals (Sweden)

    Kazım Bozdemir

    2013-01-01

    Full Text Available A 39-year-old male was admitted to our clinic with symptoms of headache, dizziness, nausea, otalgia, otorrhea, tinnitus, and hearing loss in both ears for 3 weeks. Physical examination revealed edema in the tympanic membrane and external ear canal, and pain by palpation in the mastoid area bilaterally. There was no nystagmus, and the rest of the physical examination was otherwise normal. Temporal bone high resolution computed tomography (CT showed a lesion causing erosion in the mastoid cortex, tegmen tympani, ossicles, and in the bone covering the sigmoid sinus bilaterally. There was also erosion in the superior semicircular canal and petrous bone on the left side. Cortical mastoidectomy was performed under general anesthesia. Histopathologic examination of the tissue revealed Langerhans cell histiocytosis (LCH. In this paper a case with LCH, presenting with bilateral mastoid involvement which has been rarely reported in the literature, is discussed with the existing literature.

  20. The indeterminate cell proliferative disorder: report of a case manifesting as an unusual cutaneous histiocytosis.

    Science.gov (United States)

    Wood, G S; Hu, C H; Beckstead, J H; Turner, R R; Winkelmann, R K

    1985-11-01

    A patient with an unusual, distinctive cutaneous histiocytosis is described. Extensive morphologic, antigenic, and enzymatic studies indicate that this histiocytosis represents a proliferative disorder of cutaneous indeterminate cells. Features that distinguish this disorder from other histiocytoses are discussed.

  1. Cytomegalovirus and Langerhans Cell Histiocytosis: Is There a Link?

    Science.gov (United States)

    Khoddami, Maliheh; Nadji, Seyed-Alireza; Dehghanian, Paria; Vahdatinia, Mahsa; Shamshiri, Ahmad-Reza

    2016-01-01

    Background: Langerhans cell histiocytosis is a rare proliferative histiocytic disease of unknown etiology. Histologically, it is characterized by granuloma-like proliferation of Langerhans-type dendritic cells derived from bone marrow. Many investigators have suggested the possible role of viruses such as Epstein-Barr virus, human herpesvirus-6 (HHV-6), herpes simplex virus (HSV) types 1 and 2, and Cytomegalovirus in the pathogenesis of Langerhans cell histiocytosis. Objectives: In this study, we have investigated the presence of Cytomegalovirus in Langerhans cell histiocytosis in Iranian children. Patients and Methods: In this retrospective study, we have investigated the presence of Cytomegalovirus DNA expression, using paraffin-embedded tissue samples of 30 patients with Langerhans cell histiocytosis and 30 age and site-matched controls by qualitative Polymerase Chain Reaction (PCR) method. Results: No significant difference in prevalence of Cytomegalovirus presence between patients and controls was found. Cytomegalovirus was found by qualitative PCR in only 2 (6.66%) out of 30 patients and in 1 (3.3%) of 30 control samples with a P value of 1 (1.00 > 0.05) using chi-square test with OR: 2.07; 95% CI of OR: 0.18 - 24.15. Conclusions: Our findings do not support the hypothesis of a possible role for Cytomegalovirus in the pathogenesis of Langerhans cell histiocytosis. PMID:27307972

  2. Cytomegalovirus and Langerhans Cell Histiocytosis: Is There a Link?

    Directory of Open Access Journals (Sweden)

    Maliheh Khoddami

    2016-02-01

    Full Text Available Background: Langerhans cell histiocytosis is a rare proliferative histiocytic disease of unknown etiology. Histologically, it is characterized by granuloma-like proliferation of Langerhans-type dendritic cells derived from bone marrow. Many investigators have suggested the possible role of viruses such as Epstein-Barr virus, human herpesvirus-6 (HHV-6, herpes simplex virus (HSV types 1 and 2, and Cytomegalovirus in the pathogenesis of Langerhans cell histiocytosis. Objectives: In this study, we have investigated the presence of Cytomegalovirus in Langerhans cell histiocytosis in Iranian children. Patients and Methods: In this retrospective study, we have investigated the presence of Cytomegalovirus DNA expression, using paraffin-embedded tissue samples of 30 patients with Langerhans cell histiocytosis and 30 age and site-matched controls by qualitative Polymerase Chain Reaction (PCR method. Results: No significant difference in prevalence of Cytomegalovirus presence between patients and controls was found. Cytomegalovirus was found by qualitative PCR in only 2 (6.66% out of 30 patients and in 1 (3.3% of 30 control samples with a P value of 1 (1.00 > 0.05 using chi-square test with OR: 2.07; 95% CI of OR: 0.18 - 24.15. Conclusions: Our findings do not support the hypothesis of a possible role for Cytomegalovirus in the pathogenesis of Langerhans cell histiocytosis.

  3. Squash smear cytology of Langerhans cell histiocytosis

    Science.gov (United States)

    Nahm, Ji Hae; Yoon, Gun; Do, Sung-Im; Kim, Hyun-Soo

    2015-01-01

    Squash smear cytology of Langerhans cell histiocytosis (LCH) has rarely been reported. We described squash cytological findings of cranial LCH. Additionally, based on recent data that suggests an association of LCH with either viral infection or genetic alteration, we investigated the presence of several viruses or mutation of TP53 and BRAF in LCH tissue samples. Intraoperative squash smears of a small tissue fragment excised from the lesion demonstrated a mixed population of eosinophils, neutrophils, small lymphocytes and a high content of histiocytes. The histiocytes possessed abundant dense cytoplasm with round cell shape and eccentrically located nuclei with fine chromatin, delicate nuclear membranes and prominent nuclear grooves, indentations and pseudoinclusions. The cytologic features were consistent with Langerhans cells (LCs). Subsequent histopathologic examination confirmed the diagnosis of LCH. Immunohistochemically, the LCs were positive for S-100, CD1a and langerin, but negative for adenovirus, CMV, EBV, HHV-8, HPV, HSV, SV 40 and p53. BRAF V600E mutation was absent. Our findings did not support the role of viruses and genetic abnormalities in the pathogenesis of LCH. In summary, the presence of a mixed population of inflammatory cells and a high content of histiocytes with characteristic cytomorphology, along with radiologic evidence and appropriate clinical findings, is highly suggestive of LCH on the intraoperative squash smears. Awareness of characteristic cytological features of LCH is necessary for rapid and accurate diagnosis. Squash smear cytology is a potentially useful tool in the intraoperative diagnosis of LCH. PMID:26339366

  4. Langerhans Cell Histiocytosis Presenting as Uncontrolled Asthma

    Directory of Open Access Journals (Sweden)

    Frederic A. Rawlins

    2013-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is an uncommon disorder affecting primarily young adult smokers. It is characterized by abnormal proliferation of Langerhans cells, specialized monocyte-macrophage lineage antigen-presenting cells. LCH can affect the lungs in isolation or as part of a systemic disease. Most commonly, the disease presents in the third or fourth decade without gender predominance. Symptoms typically include dyspnea and cough. Commonly, physical examination is unremarkable but cor pulmonale may be observed in advanced disease. The chest radiograph is typically abnormal with nodular or interstitial infiltrates and cystic changes. High-resolution computed tomography of the chest with these findings in the middle and upper lobes of an adult smoker is virtually diagnostic of LCH. Pulmonary function assessment is variable. Asthma has rarely been reported in association with this disorder. There are only three reported cases of the diagnosis of concomitant asthma which have been made in association with the diagnosis of LCH. We present a case in which our patient presented with signs and symptoms of asthma to include confirmatory findings of airway hyperresponsiveness. The diagnosis of LCH was established after the patient failed to respond to conventional treatment for asthma, and further evaluation was completed.

  5. Langerhans cell histiocytosis : a reactive or neoplastic disease?

    NARCIS (Netherlands)

    Teixeira da Costa, Cristiana Elizabete

    2008-01-01

    Although Langerhans cell histiocytosis (LCH) was first described a century ago, the aetiology is still not understood. Recent studies on the role of cytokines, chemokines, immunologic dysfunction, cell surface antigen expression, clonality and cell cycle regulation have provided new insights into t

  6. The presence of cytokines in Langerhans' cell histiocytosis

    NARCIS (Netherlands)

    deGraaf, JH; Tamminga, RYJ; DamMeiring, A; Kamps, WA; Timens, W

    1996-01-01

    Langerhans' cell histiocytosis (LCH) is characterized by an accumulation and/or proliferation of cells with a Langerhans' cell (LC) phenotype. The aetiology and pathogenesis of LCH are unknown; it is suggested that LCH is caused by an immunological dysregulation. Production of cytokines is a central

  7. CT findings of orbital langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Yi, Gina; Yoon, Hye Kyung; Han, Bokyung Kim; Kim, Kyeong Ah; Choo, In Wook [Samsung Medical Center, School of Medicine, Sungkyunkwan University, Seoul (Korea, Republic of)

    2000-05-01

    To evaluate the CT findings in patients with Langerhans cell histiocytosis (LCH) involving the orbit. Orbital CT scans of six children with pathologically proven LCH were retrospectively analyzed. Follow-up CT (n=3D5) and MR (n=3D1) imaging findings were also reviewed. Initial CT scans revealed varying degree of bone destruction with soft-tissue masses, and on nonenhanced images the mean attenuation value was 44 Hounsfield units (HU). All masses showed mild to moderate enhancement with a mean attenuation value of 74 HU. The bony margins abutting onto soft tissue masses were irregular but clearly demarcated. No evidence of calcification or periosteal reaction was noted. Suprasellar mass and rib involvement was noted in one patient and hepatosplenomegaly in two. Follow-up CT and MR images showed that the soft tissue masses were almost completely resolved, with bone remodeling and reossification. A soft tissue mass with irregular but clearly demarcated bone destruction is thought to be a characteristic finding of LCH involving the orbit. Follow-up images after treatment showed bone remodeling and reossification. (author)

  8. Acute leukemia in association with Langerhans cell histiocytosis

    NARCIS (Netherlands)

    R.M. Egeler (Maarten); J.P. Neglia (J.); M. Aricò (Maurizio); B.E. Favara (B.); A. Heitger (A.); M.E. Nesbit (M.)

    1994-01-01

    textabstractLangerhans cell histiocytosis (LCH) and malignancy occurring in the same individual is unusual and has generally been the subject of isolated case reports. To better define the occurrence of these events a registry of cases with synchronous or asynchronous LCH and malignancy was develope

  9. Thallium-201 uptake in Langerhans` cell histiocytosis of bone

    Energy Technology Data Exchange (ETDEWEB)

    Bar-Sever, Z. [Division of Nuclear Medicine, Department of Radiology, Children`s Hospital and Harvard Medical School, Boston, MA (United States); Connolly, L.P. [Division of Nuclear Medicine, Department of Radiology, Children`s Hospital and Harvard Medical School, Boston, MA (United States); Jaramillo, D. [Division of Nuclear Medicine, Department of Radiology, Children`s Hospital and Harvard Medical School, Boston, MA (United States); Treves, S.T. [Division of Nuclear Medicine, Department of Radiology, Children`s Hospital and Harvard Medical School, Boston, MA (United States)

    1996-10-01

    A case in which there was thallium-201 ({sup 201}Tl) uptake in Langerhans` cell histiocytosis (LCH) of the left femoral diaphysis is presented. The authors propose that {sup 201}Tl scintigraphy is potentially useful in the diagnosis and follow-up of patients with LCH. (orig.). With 4 figs.

  10. Langerhans cell histiocytosis of the clavicle: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Verbist, B.; Geusens, E.; Brys, P.; Verslegers, I.; Baert, A.L. [Department of Radiology, University Hospitals, Leuven (Belgium); Samson, I. [Department of Orthopedic Surgery, University Hospitals, Leuven (Belgium); Sciot, R. [Department of Pathology II, University Hospitals, Leuven (Belgium)

    1998-10-01

    A case of Langerhans cell histiocytosis in a 47-year-old male presenting as an aggressive appearing lesion of the clavicle is reported. It illustrates the difficulties of the radiological diagnosis of a solitary bone lesion. (orig.) (orig.) With 3 figs., 7 refs.

  11. Langerhans cell histiocytosis with involvement of the pons: case report

    Energy Technology Data Exchange (ETDEWEB)

    Vourtsi, A. [Xatzopoulou, Athens (Greece)]|[Department of Radiology, University of Athens Medical School, Athens (Greece); Papadopoulos, A.; Moulopoulos, L.A.; Vlahos, L. [Department of Radiology, University of Athens Medical School, Athens (Greece); Xenellis, J. [Department of Otorhinolaryngology, University of Athens Medical School, Athens (Greece)

    1998-03-01

    Central nervous system involvement is uncommon in Langerhans cell histiocytosis. The suprasellar region is more frequently affected. There have been few reports of involvement of the brain parenchyma shown on CT or MRI. We present a case of involvement of the pons, showing marked contrast enhancement on MRI. (orig.) With 2 figs., 17 refs.

  12. Radiotherapy for Langerhans Cell Histiocytosis of Bilateral Eyelids

    Science.gov (United States)

    Bourque, Jean-Marc; Lukovic, Jelena; Dar, A. Rashid

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder with numerous clinicopathological variants with differing clinical courses, treatment methods, and prognoses. We report one patient with atypical LCH of the bilateral lower eyelids and subsequent successful treatment with local radiation therapy. PMID:27004151

  13. Pulmonary Langerhans cell histiocytosis causing spontaneous bilateral pneumothorax in a child

    Directory of Open Access Journals (Sweden)

    Anupam Patra

    2015-01-01

    Full Text Available Bilateral pneumothorax is very rare in childhood. Moreover, if it is due to pulmonary involvement of Langerhans cell histiocytosis, it is even rarer in childhood. In our case, a nonsmoker 12-year-old boy presented with bilateral pneumothorax, whose high-resolution computed tomography scan was highly suggestive of pulmonary Langerhans cell histiocytosis. Excision biopsy of a clinically palpable cervical lymph node and histopathological examination and immunohistochemistry positivity for CD1a indicated a diagnosis of Langerhans cell histiocytosis. Clinicians should consider pulmonary Langerhans cell histiocytosis in differential diagnoses in dealing such a case.

  14. Uncommon features of pulmonary Langerhans' cell histiocytosis:analysis of 11 cases and a review of the literature

    Institute of Scientific and Technical Information of China (English)

    LING Chun-hua; JI Cheng; Daniel P Raymond; Patricia A Bourne; XU Hao-dong

    2010-01-01

    @@ Pulmonary Langerhans' cell histiocytosis (PLCH) is an isolated form of Langerhans' cell histiocytosis that usually develops in cigarette smokers.~(1,2) PLCH usually has typical light microscopic morphology with a spectrum of progressive changes.~(2-4)

  15. Langerhans cell histiocytosis: Current concepts in dentistry and case report

    OpenAIRE

    Ramos-Gutierrez, Efraín; Alejo-Gonzalez, Francisco; Ruiz Rodríguez, Socorro; Garrocho Rangel, Arturo; Pozos Guillén, Amaury de Jesús

    2016-01-01

    Langerhans cell histiocytosis (LCH), which is a rare granulomatous pediatric disease of unknown etiology, is characterized by the idiopathic proliferation and accumulation of abnormal and clonal Langerhans cells or their marrow precursors, resulting in localized, solitary or multiple destructive lesions. These lesions are most commonly eosinophilic granuloma, which are found in craniofacial bone structures such as the skull and mandible, skin and other organs. In children, the disease has a v...

  16. Langerhans cell histiocytosis: Current concepts in dentistry and case report

    Science.gov (United States)

    Ramos-Gutiérrez, Efraín; Alejo-González, Francisco; Ruiz-Rodríguez, Socorro; Garrocho-Rangel, José-Arturo

    2016-01-01

    Langerhans cell histiocytosis (LCH), which is a rare granulomatous pediatric disease of unknown etiology, is characterized by the idiopathic proliferation and accumulation of abnormal and clonal Langerhans cells or their marrow precursors, resulting in localized, solitary or multiple destructive lesions. These lesions are most commonly eosinophilic granuloma, which are found in craniofacial bone structures such as the skull and mandible, skin and other organs. In children, the disease has a variable initial presentation, and the clinical course, prognosis and survival are unpredictable. The aims of this report were to present an LCH case in a girl aged 2 years, 8 months and her clinicopathological features, to describe the bucodental management provided, and to discuss special dental considerations of this disease. Key words:Children, dental management, histiocytosis, Langerhans cells. PMID:26855698

  17. Cerebellar and basal ganglion involvement in Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Saatci, I.; Baskan, O.; Haliloglu, M.; Aydingoz, U. [Department of Radiology, Hacettepe University Hospital, Sihhiye 06100, Ankara (Turkey)

    1999-06-01

    Langerhans cell histiocytosis (LCH) is a disease of unknown cause characterised by proliferation of histiocytic granulomas in tissues; the primary cerebral manifestation is diabetes insipidus caused by hypothalamic infiltration. We present a patient in whom, except for the absence of high signal on T 1 weighting in the posterior pituitary, consistent with central diabetes insipidus, MRI showed no evidence of hypothalamic involvement by histiocytosis, despite the long duration of the disease. However, there was bilateral, symmetrical involvement of the cerebellum and globus pallidus in addition to a calvarial lesion. High signal in the cerebellar white matter on T 2-weighted images may represent demyelination, gliosis and cell loss, as previously reported on pathologic examination. (orig.) With 5 figs., 22 refs.

  18. Disseminated Langerhans Cell Histiocytosis Presenting as Cholestatic Jaundice

    Science.gov (United States)

    Loizides, Anthony M.; Sachdeva, Soumya; Paul, Premila

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a disorder associated with proliferation of Langerhans cells in various organs. LCH secondary to multisystem involvement can present in a variety of ways. Because of its infiltrative nature, LCH can involve the skin, lymph nodes, the lung or the liver. Jaundice in LCH is a manifestation of liver disease; biliary dilatation secondary to lithiasis or may be due to coexistent Niemann-Pick disease. However, a case of cholestasis has been very rarely described. Cholestasis may result from lymph nodes obstructing the porta hepatis. In this report, we describe a case of type II histiocytosis X with obstructive cholestasis and pulmonary involvement in the form of cysts without significant lymphadenopathy at the porta. PMID:25859497

  19. Photodynamic therapy for multi-resistant cutaneous Langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Arjen F. Nikkels

    2010-06-01

    Full Text Available Langerhans cell histiocytosis is a rare group of proliferative disorders. Beside cutaneous involvement, other internal organs can be affected. The treatment of cutaneous lesions is difficult and relies on topical corticosteroids, carmustine, nitrogen mustard, and photochemotherapy. Systemic steroids and vinblastine are used for recalcitrant skin lesions. However, some cases fail to respond. An 18-month old boy presented a CD1a+, S100a+ Langerhans cell histocytosis with cutaneous and severe scalp involvement. Topical corticosteroids and nitrogen mustard failed to improve the skin lesions. Systemic corticosteroids and vinblastine improved the truncal involvement but had no effect on the scalp lesions. Methyl-aminolevulinate (MAL based photodynamic therapy (PDT resulted in a significant regression of the scalp lesions. Control histology revealed an almost complete clearance of the tumor infiltrate. Clinical follow-up after six months showed no recurrence. Although spontaneous regression of cutaneous Langerhans cell histiocytosis is observed, the rapid effect of photodynamic therapy after several failures of other treatment suggests that photodynamic therapy was successful. As far as we know this is the first report of photodynamic therapy for refractory skin lesions. Larger series are needed to determine whether photodynamic therapy deserves a place in the treatment of multiresistant cutaneous Langerhans cell histiocytosis.

  20. Langerhans cell histiocytosis involving central nervous system: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Moon, Won Jin; Park, Dong Woo; Lee, Seung Ro; Hahm, Chang Kok; Ju, Kyung Bin [Hanyang University College of Medicine, Seoul (Korea, Republic of); Kim, Sung Tae [Ulsan University College of Medicine, Seoul (Korea, Republic of)

    1997-01-01

    Langerhans cell histiocytosis(LCH) is a systemic disorder characterized by idiopathic proliferation of histiocytes in the reticuloendothelial system; CNS involvement outside the hypothalamus or pituitary gland is uncommon. We present a case of LCH involving the brainstem, cerebellum, and temporal lobes, and also showing hypothalamic involvement. The lesions were isointense or hypointense on T1WI and hyperintense on T2WI, and showed multifocal enhancing nodules on post-contrast CT and Gd-enhanced MRI.

  1. High expression of markers of apoptosis in Langerhans cell histiocytosis

    DEFF Research Database (Denmark)

    Petersen, Bodil Laub; Lundegaard, Pia Rengtved; Bank, M I;

    2003-01-01

    53 and the number of cells in apoptosis detected with TUNEL. Langerhans cell histiocytosis cells showed strong expression of p53 and in some cases co-expression of Fas and Fas-L. The expression of Fas-L was significantly higher in infiltrates from patients with single-system disease. The actual...... number of pathological Langerhans cells in apoptosis as estimated by TUNEL was low. CONCLUSIONS: The low number of TUNEL-reactive cells can be explained by the rapid turnover of apoptotic cells in the tissue, not leaving the apoptotic cells long enough in the tissue to be detected. The co...

  2. MR and CT findings of temporal bone langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Bae, Jae Ig; Lee, Hee Jung; Kim, Heung Sik [Keimyung Univ. School of Medicine, Dongsan Medical Center, Taegu (Korea, Republic of)

    2001-11-01

    To describe the MRI and CT findings of temperal bone langerhans cell histiocytosis. The MRI (n=8) and CT (n=7) findings of nine lesions of temporal bone Langerhans cell histiocytosis in six children were retrospectively reviewed. Eight lesions were pathologically confirmed and one was clinically diagnosed. The findings were analyzed for bilaterality, location, lesion extent, signal intensity, the attenuation of soft tissue lesions seen at MRI or precontrast CT, enhancement pattern at MRI or CT, and the pattern of bony destruction at CT. Bilateral involvement was present in three of six patients (50%). Lesions were most frequently located in the mastoid (n=8, 89%), followed by the petrous ridge (n=6, 67%), and the squamous portion (n=3, 33%). Seven (78%) lesions extended to the ipsilateral cavernous sinus (n=3), sphenoid bone (n=3), orbit (n=2), or epidural space (n=2). The signals of the soft tissue lesions were isointense in five cases (63%) on T1-weighted images and hyperintense in six (75%) at MRI, and homogeneous in five (71%) at CT. All lesions demonstrated bony destruction without periosteal reaction and five (71%) showed ill-defined destruction, with crossing sutures. Familiarity with findings of predominant mastoid involvement, isointense or isodense soft tissue lesions seen on T1-weighted images or at precontrast CT, with relatively homogeneous enhancement at CT, and irregular bony destruction with crossing sutures may be helpful in narrowing the diagnosis of temporal bone langerhans cell histiocytosis.

  3. A child with pulmonary and liver Langerhans'-cell histiocytosis

    Institute of Scientific and Technical Information of China (English)

    MA Xiao-li; SHEN Kun-ling; WANG Bin

    2012-01-01

    Clinical categories of Langerhans cell histiocytosis (LCH) include single and multi-system disease.Pulmonary LCH is rare,which is an unusual interstitial lung disease with the characteristics of monoclonal proliferation and infiltration of Langerhans' cells to organs.We report our experience of a rare LCH case of multiple organs such as pulmonary and liver as the main clinical manifestation.The patient was treated with chemotherapy which included prednisone,vinblastine,methotrexate and 6-mercaptopurine for 52 weeks and follow up all along.The patient has a favorable clinical outcome.

  4. Langerhans cell histiocytosis presenting as hypothyroid goitre: a unique presentation.

    Science.gov (United States)

    Marupudi, Krishna Chaitanya; Karanth, Suman S; Thomas, Joseph

    2014-12-02

    Langerhans cell histiocytosis (LCH) is a disease involving the antigen presenting cells, which can range from a single system involvement with an indolent course to a multisystem disease with increased morbidity. We present a rare case of LCH presenting as hypothyroid goitre that was successfully treated as per the LCH III trial protocol with a combination of vinblastine and oral prednisolone. We highlight the need for awareness of this rare presentation of LCH as a painless goitre which is often misdiagnosed as poorly differentiated thyroid carcinoma or overlooked as a benign disease.

  5. Langerhans cell histiocytosis: An uncommon presentation, successfully treated by thalidomide

    Directory of Open Access Journals (Sweden)

    Mohammad Shahidi-Dadras

    2011-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disease and generally affects children under 15 years of age. Adult onset form and cutaneous features at presentation are uncommon. There are some options for treatment of the skin lesions of LCH such as topical and intralesional corticosteroid, nitrogen mustard, etc., which are not completely curative. Herein, we report a case of perianal LCH in a 20-year-old man with one-year history of recalcitrant well-demarcated, erythematous, and ulcerated plaque surrounding the anal orifice, with pain and difficulty in defecation that was successfully treated with thalidomide.

  6. Vulvar ulcer as a presentation of systemic langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Nina A Madnani

    2011-01-01

    Full Text Available We report a 38-year-old housewife with systemic Langerhans cell histiocytosis (LCH presenting as a chronic vulvar and peri-anal ulcer. She had systemic involvement in the form of diabetes insipidus and bone "hot-spots". She responded favorably to etoposide, 6-mercaptopurine, and systemic steroids, and has been in remission since 10 years. Chronic vulvar ulcers not responding to routine therapy should not be neglected and need to be biopsied repeatedly to come to a specific diagnosis. The vulvar ulcer in our case provided a vital clue to a systemic LCH, with a successful outcome.

  7. A rare occurrence of Langerhans cell histiocytosis in an adult

    Science.gov (United States)

    Shevale, Vruturaj V; Ekta, K; Snehal, T; Geetanjal, M

    2014-01-01

    Langerhans Cell Histiocytosis (LCH) is a disease process characterized by accumulation and infiltration of cells, showing ultrastructural and immunohistochemical similarities to Langerhans’ cell, in the affected tissues. It exhibits extreme clinical heterogeneity. LCH was historically divided into 3 clinical entities based on extent of tissue involvement and severity of presentation. These 3 entities were eosinophilic granuloma, Hand-Schuler-Christian disease, Letterer-Siwe disease. Owing to similarities of their histologic appearance, they were grouped together under the term histiocytosis X. It was recently changed to LCH, emphasizing the primary cell involved in the disease process. LCH is a rare disease with an incidenceestimated to be 4.0 to 5.4 per million population. Males are affected twice as frequently as females. The disease may occur at any age with peak incidence in children aged 1 to 3 years. We describe an unusual case of a 65-year-old man who presented with painless swelling in anterior region of mandible. PMID:25948998

  8. Clinical and radiological evolution in patients with pulmonary Langerhans' cell histiocytosis

    NARCIS (Netherlands)

    Westerlaan, HE; van der Valk, PDLPM

    2002-01-01

    Background: Pulmonary Langerhans' cell histiocytosis (LCH) is a diffuse, smoking-related lung disease characterised pathologically by proliferation of abnormal Langerhans' cells, cyst formation and vascular abnormalities, and physiologically by a decreased diffusing capacity. The aim of this study w

  9. Otic Langerhans' Cell Histiocytosis in an Adult: A Case Report and Review of the Literature

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    Anil Gungadeen

    2013-01-01

    Full Text Available Objective. To present a case of otic Langerhans' cell histiocytosis in an adult. Also included the diagnosis and management of the condition and a review of the relevant literature. Case Report. We report a case of a 41-year-old man with a history of persistent unilateral ear discharge associated with an aural polyp. Radiological imaging showed bony lesions of the skull and a soft-tissue mass within the middle ear. Histological analysis of the polyp demonstrated Langerhans' cell histiocytosis. His otological symptoms were completely resolved with the systemic therapy. Conclusions. Otic Langerhans' cell histiocytosis can present in adults. Persistent ear symptoms along with evidence of soft-tissue masses within the ear and bony lesions of the skull or elsewhere should prompt the otolaryngologists to include Langerhans' cell histiocytosis in their differential diagnosis. Management should be with systemic therapy rather than local surgical treatment.

  10. A Retrospective Analysis of Oral Langerhans Cell Histiocytosis in an Iranian Population: a 20-year Evaluation

    Science.gov (United States)

    Atarbashi Moghadam, Saede; Lotfi, Ali; Piroozhashemi, Batool; Mokhtari, Sepideh

    2015-01-01

    Statement of the Problem Langerhans cell histiocytosis is a rare disease with unknown pathogenesis and is characterized by local or disseminated proliferation of Langerhans cells. There is no previous investigation on prevalence of oral Langerhans cell histiocytosis in Iranian population. Purpose The purpose of this study was to assess the relative frequency of oral Langerhans cell histiocytosis in an Iranian population and to compare the data with previous reports. Materials and Method Pathology files of Oral and Maxillofacial Pathology Department of Dental School of Shahid Beheshti University of Medical Sciences from 1992 to 2012 were searched for cases recorded as oral Langerhans cell histiocytosis. A total number of 20 cases were found and the clinical information of patients was recorded. Results The relative frequency of oral Langerhans cell histiocytosis was 0.34% and the most common location was the posterior mandible. In addition, the mean age of patients was 27 years and there was a definite male predominance. Most lesions were localized and tooth mobility was the most common oral presentation. Conclusion In Iranian population as in many other countries, the relative frequency of oral Langerhans cell histiocytosis is low. Moreover, tooth mobility and periodontal lesions are the frequent early signs of disease. Therefore, in patients with periodontal problems, good oral health, and no response to the treatment; Langerhans cell histiocytosis must be considered. Additionally, although most cases of oral Langerhans cell histiocytosis are localized, systemic involvement must also be considered and dental professionals have an important role in early detection of the disease. PMID:26535408

  11. A Retrospective Analysis of Oral Langerhans Cell Histiocytosis in an Iranian Population: a 20-year Evaluation

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    Saede Atarbashi Moghadam

    2015-09-01

    Full Text Available Statement of the Problem: Langerhans cell histiocytosis is a rare disease with unknown pathogenesis and is characterized by local or disseminated proliferation of Langerhans cells. There is no previous investigation on prevalence of oral Langerhans cell histiocytosis in Iranian population. Purpose: The purpose of this study was to assess the relative frequency of oral Langerhans cell histiocytosis in an Iranian population and to compare the data with previous reports. Materials and Method: Pathology files of Oral and Maxillofacial Pathology Department of Dental School of Shahid Beheshti University of Medical Sciences from 1992 to 2012 were searched for cases recorded as oral Langerhans cell histiocytosis. A total number of 20 cases were found and the clinical information of patients was recorded. Results: The relative frequency of oral Langerhans cell histiocytosis was 0.34% and the most common location was the posterior mandible. In addition, the mean age of patients was 27 years and there was a definite male predominance. Most lesions were localized and tooth mobility was the most common oral presentation. Conclusion: In Iranian population as in many other countries, the relative frequency of oral Langerhans cell histiocytosis is low. Moreover, tooth mobility and periodontal lesions are the frequent early signs of disease. Therefore, in patients with periodontal problems, good oral health, and no response to the treatment; Langerhans cell histiocytosis must be considered. Additionally, although most cases of oral Langerhans cell histiocytosis are localized, systemic involvement must also be considered and dental professionals have an important role in early detection of the disease.

  12. Langerhans Cell Histiocytosis Followed by Hodgkin Lymphoma: A Case Report

    Science.gov (United States)

    Safaei, Akbar; Bagheri, Mandana; Shahryari, Jahanbanoo; Noori, Sadat; Esmailzade, Elmira

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare neoplasm defined as the proliferation of bone marrow langerhans cells, which is a kind of dendritic cells. The major pathological features of LCH are expression of CD1a and S100 as well as Birbeck granules. Its presentation can differ from a mild bone lesion to a multi-systemic evolved malignant neoplasm; however, the latter outcome is almost rare. Thus, LCH is mostly known as a benign neoplasm. In this study, we present a case of LCH followed by Hodgkin lymphoma (HL). Accompaniment of this disease with malignant lymphoma is rare and considered as case report. Several cases in which malignant lymphoma occurred prior to LCH are reported; however, few cases can be found with LCH followed by malignant lymphomas. PMID:25999631

  13. Langerhans cell histiocytosis revisited: Case report with review

    Science.gov (United States)

    Kumar, Y. Pavan; Agrawal, Jayshree; Mohanlakshmi, J.; Kumar, P. Suresh

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by proliferation of bone marrow derived Langerhans cells and mature eosinophils. Their clinical features simulate common oral findings such as gingival enlargement, oral ulcers, and mobility of teeth, along with nonspecific radiographic features; hence, diagnosing such lesions becomes difficult for the oral physicians. These lesions are commonly seen in childhood; however, we are reporting a case of LCH in 29-year-old adult male. A provisional diagnosis of giant cell granuloma was considered based on history and examination, although the lesion was histologically proven to be LCH and was confirmed with immunohistochemical staining of S100 protein and CD1a antigen. The purpose of this paper is to enhance the understanding of diverse, nonpathognomical oral presentation of LCH that is easily misdiagnosed and overlooked by dentist. PMID:26321851

  14. Langerhans cell histiocytosis revisited: Case report with review

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    Y Pavan Kumar

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a group of idiopathic disorders characterized by proliferation of bone marrow derived Langerhans cells and mature eosinophils. Their clinical features simulate common oral findings such as gingival enlargement, oral ulcers, and mobility of teeth, along with nonspecific radiographic features; hence, diagnosing such lesions becomes difficult for the oral physicians. These lesions are commonly seen in childhood; however, we are reporting a case of LCH in 29-year-old adult male. A provisional diagnosis of giant cell granuloma was considered based on history and examination, although the lesion was histologically proven to be LCH and was confirmed with immunohistochemical staining of S100 protein and CD1a antigen. The purpose of this paper is to enhance the understanding of diverse, nonpathognomical oral presentation of LCH that is easily misdiagnosed and overlooked by dentist.

  15. Langerhans cell histiocytosis in monozygotic twins with central diabetes insipidus and hypophyseal masses

    Science.gov (United States)

    Wei, Sung-Tai; Chen, Der-Cherng; Cho, Der-Yang; Lin, Hung-Lin

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a systemic disease mainly affecting children and young adults. It can manifest as single system disorder or multi-system involvement. When the central nervous system is involved, the hypothalamic–pituitary axis is the most common location affected. Herein we report a rare case of Langerhans cell histiocytosis in monozygotic twins both with central diabetes and hypophyseal masses. This is the first report about LCH in monozygotic twins with hypophyseal lesions. PMID:25972939

  16. Redefining Langerhans Cell Histiocytosis as a Myeloid Dysplasia and Identifying B | Division of Cancer Prevention

    Science.gov (United States)

    DESCRIPTION (provided by applicant): Redefining Langerhans Cell Histiocytosis as a Myeloid Dysplasia and Identifying Biomarkers for Early Detection and Risk Assessment. This application addresses Program Announcement PA-09-197: Biomarkers for Early Detection of Hematopoietic Malignancies (R01). The overall aim of this project is to identify novel biomarkers that may be used to diagnose and treat patients with Langerhans Cell Histiocytosis (LCH). LCH occurs with similar frequency as other rare malignancies including Hodgkin's lymphoma and AML. |

  17. Langerhans cell histiocytosis : genetic and immunologic fingerprinting

    NARCIS (Netherlands)

    Quispel, W.T.

    2016-01-01

    LCH lesions are characterized by accumulating LCH-cells, which are related to Langerhans and/or Dendritic cells, and the presence of other elements of the immune system. A significant proportion of LCH-cells display somatic mutations in proteins that drive the constitutive activation of the MAPK-pat

  18. Langerhans cell histiocytosis with presentation as orbital disease

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    Ashok B Bhanage

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs. In rare instances, the affection of the orbit is the only and the first symptom. We report an unusual case of an 18-month-old male who presented with orbital disease as the first symptom, in the form of chronic presentation of periorbital swelling (2 months duration with acute inflammation (1-week duration giving a suspicion of orbital cellulitis. Histopathology after radical excision confirmed the diagnosis of LCH and was advised initial therapy as per Histiocyte Society Evaluation and Treatment Guidelines (2009 but was lost to follow-up only reappearing with progression (multisystem LCH with risk organ involvement and developed progressive active disease on treatment after 5 weeks. He was treated with salvage therapy for risk patients achieving complete remission.

  19. Langerhans cell histiocytosis with presentation as orbital disease

    Science.gov (United States)

    Bhanage, Ashok B.; Katkar, Anand D.; Ghate, Prajakta S.

    2015-01-01

    Langerhans cell histiocytosis (LCH) is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs. In rare instances, the affection of the orbit is the only and the first symptom. We report an unusual case of an 18-month-old male who presented with orbital disease as the first symptom, in the form of chronic presentation of periorbital swelling (2 months duration) with acute inflammation (1-week duration) giving a suspicion of orbital cellulitis. Histopathology after radical excision confirmed the diagnosis of LCH and was advised initial therapy as per Histiocyte Society Evaluation and Treatment Guidelines (2009) but was lost to follow-up only reappearing with progression (multisystem LCH with risk organ involvement) and developed progressive active disease on treatment after 5 weeks. He was treated with salvage therapy for risk patients achieving complete remission. PMID:26167225

  20. Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary Involvement

    Science.gov (United States)

    Araujo, Bruno; Costa, Francisco; Lopes, Joanne; Castro, Ricardo

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Although common among children, liver involvement is relatively rare in adults and frequently overlooked. Natural history of liver LCH fits into two stages: an early stage with infiltration by histiocytes and a late stage with sclerosis of the biliary tree. Pulmonary findings are more common and include multiple nodules in different stages of cavitation, predominantly in the upper lobes. We present a case of adult LCH with pulmonary and biopsy proven liver involvement with resolution of the hepatic findings after treatment. PMID:25977828

  1. Cytological diagnosis of Langerhans cell histiocytosis with cutaneous involvement

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    Sushama A Chandekar

    2013-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disease affecting predominantly children. The course of the disease varies, from spontaneous resolution to a progressive multisystem disorder with organ dysfunction and potential life-threatening complications. Diagnosis of LCH is often difficult and may be delayed because of its rarity and especially so if it occurs with unusual presentation. Fine needle aspiration cytology of a 4 year old male child, a case of LCH is presented with a purpose of highlighting the characteristic cytological features. A high index of suspicion, awareness of characteristic cytological features of LCH and its differential diagnoses is necessary. This can obviate the need of biopsy and electron microscopy. Immunohistochemistry if available can be performed on cytology smear and cell block.

  2. Multisystem Langerhans cell histiocytosis presenting as an oral lesion

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    Kallarakkal Thomas George

    2013-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare proliferative disorder in which the pathologic Langerhans cells infiltrate and destroy the tissues. Patients with LCH present varied clinical manifestations. Cutaneous lesions in LCH manifest as vesiculopapular eruptions that often mimic various infectious diseases particularly in infants. We present a case of a female infant with an ulcerative lesion intraorally. The baby was asymptomatic otherwise. A detailed history revealed the presence of cutaneous lesions that was overlooked by her parents. Conclusion: This report tries to briefly discuss the current concepts regarding the etiology of LCH. An attempt has been made to emphasis the need for a through systemic examination. The protocol of investigative procedures to be adopted in LCH is also discussed.

  3. Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis

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    Stephen C Juvet

    2010-01-01

    Full Text Available Pulmonary Langerhans’ cell histiocytosis (PLCH is an unusual cystic lung disease that is also characterized by extrapulmonary manifestations. The current review discusses the presenting features and relevant diagnostic testing and treatment options for PLCH in the context of a clinical case. While the focus of the present article is adult PLCH and its pulmonary manifestations, it is important for clinicians to distinguish the adult and pediatric forms of the disease, as well as to be alert for possible extrapulmonary complications. A major theme of the current series of articles on rare lung diseases has been the translation of insights gained from fundamental research to the clinic. Accordingly, the understanding of dendritic cell biology in this disease has led to important advances in the care of patients with PLCH.

  4. Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary Involvement

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    Bruno Araujo

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Although common among children, liver involvement is relatively rare in adults and frequently overlooked. Natural history of liver LCH fits into two stages: an early stage with infiltration by histiocytes and a late stage with sclerosis of the biliary tree. Pulmonary findings are more common and include multiple nodules in different stages of cavitation, predominantly in the upper lobes. We present a case of adult LCH with pulmonary and biopsy proven liver involvement with resolution of the hepatic findings after treatment.

  5. EXPRESSION OF CELLULAR ADHESION MOLECULES IN LANGERHANS CELL HISTIOCYTOSIS AND NORMAL LANGERHANS CELLS

    NARCIS (Netherlands)

    DEGRAAF, JH; TAMMINGA, RYJ; KAMPS, WA; TIMENS, W

    1995-01-01

    Langerhans cell histiocytosis (LCH) is characterized by lesions with an accumulation and/or proliferation of Langerhans cells (LCs). Little is known of the etiology and pathogenesis of LCH. Although the relation between the LCH cell and normal LCs is currently uncertain, the localizations of the LCH

  6. Gene expression analysis of dendritic/Langerhans cells and Langerhans cell histiocytosis

    NARCIS (Netherlands)

    Rust, Renata; Kluiver, J.; Visser, Lydia; Harms, G.; Blokzijl, T.; Kamps, W.A.; Poppema, Sibrand; van den Berg, Anke

    2006-01-01

    Langerhans cell histiocytosis (LCH) is a neoplastic disorder that results in clonal proliferation of cells with a Langerhans cell (LQ phenotype. The pathogenesis of LCH is still poorly understood. In the present study, serial analysis of gene expression (SAGE) was applied to LCs generated from umbil

  7. Langerhans' cell histiocytosis of the temporal fossa: A case report

    Science.gov (United States)

    LIANG, CHEN; LIANG, QIANLEI; DU, CHANGWANG; ZHANG, XIAODONG; GUO, SHIWEN

    2016-01-01

    Langerhans' cell histiocytosis (LCH) is a rare disease with a wide spectrum of clinical manifestations, varying from an isolated lesion to systemic involvement. The etiology of this disease remains to be elucidated. The present study reports a case of LCH with temporal fossa localization in an 8-year-old male patient, who had exhibited left temporal pain and headache for 1 month. Physical examination revealed slight exophthalmos and conjunctival hemorrhage in the patient's left eye, and non-contrast computed tomography imaging of the head revealed a soft tissue mass with unclear margins located in the left temporal fossa, as well as a wide bony defect. Magnetic resonance imaging revealed a heterogeneously contrast-enhanced mass near the left temporal pole, which eroded into the patient's left orbit and maxillary sinus. The lesion was totally excised and confirmed to be LCH through biopsy. PMID:27073529

  8. Solitary Extragnathic Langerhans Cell Histiocytosis – A Rare Case

    Science.gov (United States)

    Reddy, Eppalapally Sharath Kumar; Bhavani, Sangala Naga; A, Krishna; Sekhar, Mane Srinivas Muni

    2015-01-01

    Langerhans cell histiocytosis (LCH), mainly affects the skull, vertebrae, ribs and mandible in children and the long bones of adults. Symptoms range from none to pain, swelling and tenderness over the site of the lesion. This disease presents oral manifestations which can sometimes be the first expression of the condition. It occurs in three forms namely eosinophilic granuloma in which isolated or multiple bones are involved, But has a good prognosis whereas other variants Hand-Shuller-Christian disease (chronic dessiminated variant) and Letterer-Siwe disease (acute dessiminated form) have poor prognosis. Occasionally only soft tissues are affected without bony involvement. Males are more commonly affected than females. This article describes a rare variant of eosinophilic granuloma of labial mucosa without bony involvement. PMID:25954715

  9. A patient with lichen sclerosus, Langerhans cell histiocytosis, and invasive squamous cell carcinoma of the vulva

    NARCIS (Netherlands)

    Simons, M.; Nieuwenhof, H.P. van de; Avoort, I.A.M. van der; Bulten, J.; Hullu, J.A. de

    2010-01-01

    We report a patient with vulvar lichen sclerosus, Langerhans cell histiocytosis (LCH), and later vulvar cancer. In LCH, high amounts of non functional Langerhans cells are present in the affected tissue, making it possible that LCH may have contributed to vulvar cancer development in this patient.

  10. How I treat Langerhans cell histiocytosis

    Science.gov (United States)

    Allen, Carl E.; Ladisch, Stephan

    2015-01-01

    “Langerhans cell histiocytosis” (LCH) describes a spectrum of clinical presentations ranging from a single bone lesion or trivial skin rash to an explosive disseminated disease. Regardless of clinical severity, LCH lesions share the common histology of CD1a+/CD207+ dendritic cells with characteristic morphology among an inflammatory infiltrate. Despite historical uncertainty defining LCH as inflammatory vs neoplastic and incomplete understanding of mechanisms of pathogenesis, clinical outcomes have improved markedly over the past decades through cooperative randomized clinical trials based on empiric therapeutic strategies. Significant advances include recognition of high- and low-risk clinical groups defined by hematopoietic and/or hepatic involvement, and of the importance of optimal intensity and of duration of chemotherapy. Nevertheless, mortality of high-risk patients, disease recurrence, lack of robustly tested salvage strategies, and significant disease morbidity of both high- and low-risk patients remain challenges. Recent discovery of recurrent somatic mutations in mitogen-activated protein kinase pathway genes at critical stages of myeloid hematopoietic differentiation in LCH patients supports redefinition of the disease as a myeloproliferative disorder and provides opportunities to develop novel approaches to diagnosis and therapy. PMID:25827831

  11. Langerhans cell histiocytosis of the female genital tract.

    Science.gov (United States)

    Axiotis, C A; Merino, M J; Duray, P H

    1991-03-15

    Langerhans cell histiocytosis (LCH) of the female genital tract is rare. Four new cases are reported, and there is a review of the 38 cases in the literature. This disease may involve the vulva, vagina, cervix, endometrium, and ovary. Four distinct patient groups, segregated on the basis of initial presentation and subsequent anatomic extent of disease, were categorized as follows: (1) "pure" genital LCH, (2) genital LCH with subsequent multi-organ involvement, (3) oral or cutaneous LCH with subsequent genital and multi-organ involvement, and (4) diabetes insipidus with subsequent genital and multi-organ disease. Although involvement of the genital tract can occur at any age, it is most common in young adulthood. Clinically, LCH may mimic either primary neoplasia or various inflammatory lesions; the major pathologic differential diagnosis is venereal and other inflammatory diseases. The pure genital form may have a distinct nosologic position in the spectrum of LCH similar to the "pure," self-limited cutaneous histiocytosis seen in infants. There is no correlation between histologic findings and the outcome of the genital lesions. There is also no correlation between clinical presentation and/or the extent of involvement and outcome of genital lesions; complete regression, partial improvement, persistent lesions, and recurrences were seen in all four groups of patients. The treatment of genital LCH is not well defined and is highly individualized. Therapy has included surgery, radiation, topical corticosteroids, topical nitrogen mustard, systemic chemotherapy, and combination therapy; mixed results were obtained with all treatment modalities. Although no modality has been shown to yield a superior outcome, complete surgical excision is advocated as initial therapy.

  12. A case of Langerhans' cell histiocytosis following Hodgkin's disease

    Science.gov (United States)

    LI, XIN; DENG, QI; LI, YU-MING

    2016-01-01

    Langerhans' cell histiocytosis (LCH) is a group of disorders in various tissues characterized by the proliferation of Langerhans cells. It is rarely observed in adults. Langerhans cells are dendritic cells that express cluster of differentiation 1a (CD1a) and S100 protein, and contain Birbeck granules. Its etiopathogenesis remains to be elucidated. One possible etiological cause is a reactive proliferation of Langerhans cells following chemotherapy or radiotherapy for Hodgkin's disease (HD). A number of cases of LCH associated with malignant lymphoma have been reported previously. It may follow after the malignant lymphoma, or occur with it. However, fewer cases have been reported where the LCH followed after HD. In the present case report, a patient was diagnosed with HD following chemotherapy for LCH. As LCH was diagnosed, the patient was treated with a combination of various chemotherapeutic agents in two cycles of cyclophosphamide, vincristine, and prednisolone (COP), and eight cycles of cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP). The patient went into a successful clinical remission. One year later, computed tomographic (CT) scans of the thorax and abdomen revealed augmentation of the tumor mass in the mediastinum. An excisional biopsy of the right inguinal lymph node was performed. The patient was diagnosed with nodular sclerosing Hodgkin's disease. Following four cycles of doxorubicin (Adriamycin), bleomycin, vinblastine and dacarbazine (ABVD) chemotherapy, a whole-body positron emission tomographic CT scan revealed a decrease in tumor mass in the mediastinum. At present, the patient remains in treatment, and the prognosis has yet to be fully determined. PMID:27330759

  13. Langerhans Cell Histiocytosis of the Clavicle in a 13-Year-Old Boy

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    Shital N. Parikh

    2014-01-01

    Full Text Available Langerhans Cell Histiocytosis (LCH is a rare neoplasm characterized by abnormal proliferation of histiocytic cells. In this case report, we describe a unique case of a 13-year-old boy who presented to the clinic with an insidious onset of mid-clavicular pain. The provisional radiologic diagnosis of Langerhans Cell Histiocytosis of the clavicle was confirmed by an incisional biopsy of the left mid-clavicle lesion. The patient’s lesion was treated by curettage, bone grafting, and internal fixation, due to the presence of pathologic fracture. At the 2-year followup, the patient was asymptomatic, and the lesion showed complete resolution without recurrence. The case report highlights the characteristic features of Langerhans Cell Histiocytosis in an unusual location, the knowledge of which would help avoid delayed or missed diagnosis in the future.

  14. LANGERHANS CELL HISTIOCYTOSIS - EXPRESSION OF LEUKOCYTE CELLULAR ADHESION MOLECULES SUGGESTS ABNORMAL HOMING AND DIFFERENTIATION

    NARCIS (Netherlands)

    DEGRAAF, JH; TAMMINGA, RYJ; KAMPS, WA; TIMENS, W

    1994-01-01

    Langerhans' cell histiocytosis (LCH) is characterized by an accumulation of cells with a Langerhans' cell (LC) phenotype. Most patients present with solitary skin or bone lesions, but multi-organ lesions may appear Twenty-two LCH-tissue sections from 13 children and adolescents, with lesions at diff

  15. Bisphosphonates in Langerhans Cell Histiocytosis: An International Retrospective Case Series

    Science.gov (United States)

    Chellapandian, Deepak; Makras, Polyzois; Kaltsas, Gregory; van den Bos, Cor; Naccache, Lamia; Rampal, Raajit; Carret, Anne-Sophie; Weitzman, Sheila; Egeler, R. Maarten; Abla, Oussama

    2016-01-01

    Background Bone is the most common organ of involvement in patients with Langerhans cell histiocytosis (LCH), which is often painful and associated with significant morbidity from pathological fractures. Current first-line treatments include chemotherapy and steroids that are effective but often associated with adverse effects, whereas the disease may reactivate despite an initial response to first-line agents. Bisphosphonates are osteoclast inhibitors that have shown to be helpful in treating bone lesions of LCH. To date, there are no large international studies to describe their role in treating bone lesions of LCH. Method We conducted a multicenter retrospective review of 13 patients with histologically proven LCH, who had received bisphosphonates either at diagnosis or at disease reactivation. Results Ten patients (77%) had a single system bone disease, and 3 (23%) had bone lesions as part of multisystem disease. Median follow-up time post-bisphosphonate therapy was 4.6 years (range, 0.8 to 8.2 years). Treatment with bisphosphonates was associated with significant pain relief in almost all patients. Twelve (92%) achieved resolution of active bone lesions, and 10 out of them had no active disease for a median of 3.5 years (range, 0.8 to 5 years). One patient did not respond. No major adverse effects were reported in this series. Conclusion Bisphosphonates are well-tolerated drugs that can significantly improve bone pain and induce remission in active bone LCH. Future prospective studies evaluating the role of bisphosphonates in LCH are warranted. PMID:27413525

  16. Liver involvement of Langerhans’ cell histiocytosis in children

    Science.gov (United States)

    Yi, Xiaoping; Han, Tong; Zai, Hongyan; Long, Xueying; Wang, Xiaoyi; Li, Wenzheng

    2015-01-01

    Objective: Liver involvement is relatively frequent in children with Langerhans cell histiocytosis (LCH). Its features remain poorly defined. Methods: A retrospective study was carried out on 14 hepatic LCH children in our hospital. The Clinicopathological and radiological features of this disease was discussed. Results: The rate of liver involvement in children LCH patients is 51.9%. Majority of the patients were disseminated cases. Hepatomegaly was clinically confirmed in 11 cases (78.6%). Liver function dysfunction was seen in nine (64.3%) children. The association of multi-modal imaging significantly yielded more diagnostic information. There are some imaging characteristics of this disease, CT and MRI could help to assess the staging, extent of the hepatic lesions. We found that liver involvement had a significant impact on survival. Patients treated with systemic chemotherapy earlier from time of diagnosis had a relatively better outcome. Conclusions: The rate of liver involvement in children LCH patients maybe much higher than that of expected. We suggest that clinical and biological liver evaluation and abdominal imaging must be performed regularly onwards to screen every LCH children patient from the time of the initial diagnosis. Patient should be treated with systemic chemotherapy earlier. PMID:26221247

  17. Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy

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    Santos-Machado T.M.

    1999-01-01

    Full Text Available Symptomatic involvement of the gastrointestinal (GI tract as a prominent symptom in Langerhans' cell histiocytosis (LCH is uncommon, occurring in less than 1 to 5% of all cases, even when the disease is in its disseminated form. Up to now, there have been reports of 18 cases of LCH with GI manifestations, including our 2 cases, with diarrhea (77.7%, protein-losing enteropathy (33.3% and bloody stool being the most frequent findings. The authors present two patients with severe diarrhea and refractory hypoalbuminemia, and with the protein-losing enteropathy documented by Cr51-labeled albumin studies. A review of the literature indicated that the presence of GI symptoms is often associated with systemic disease as well as with poor prognosis, mainly under 2 years of age. Radioisotopes are useful for documenting protein loss in several diseases with high specificity and sensitivity, and their utilization in the cases reviewed here permitted diagnoses in 6 children, as well as improved therapeutic management.

  18. Langerhans cell histiocytosis of the mandible in a six-year-old child

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    Ramani Pratibha

    2007-01-01

    Full Text Available Histiocytosis is a term applied to a group of rare disorders of the reticuloendothelial system. Eosinophilic granuloma, the most benign and localized of the three Langerhans cell histiocytosis entities, may be solitary or multiple. Eosinophilic granuloma can affect almost any bone, but commonly involves the mandible when the jaws are affected. Conventional treatment of LCH is with surgery, radiotherapy, chemotherapy and steroid injections, alone or in combination. Spontaneous regression of localized disease has also been reported. We report a six and a half-year-old patient with Langerhans cell histiocytosis - solitary eosinophilic granuloma of the mandible that initially regressed but rapidly recurred even after radical treatment and had a fatal outcome.

  19. Unusual location of central nervous system langerhans cell histiocytosis: case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, E. Yup; Lee, Jae Kyu; Kim, Chan Kyo; Lee, Chang Hyun; Kang, Chang Ho; Chung, Phil Wook [Armed Forces Capital Hospital, Seongnam (Korea, Republic of)

    2003-03-01

    Langerhans cell histiocytosis of the central nervous system (CNS) usually involves the hypothalamic-pituitary axis, and T1-weighted MR images normally demonstrate infundibular thickening and/or a mass lesion in the hypothalamus and the absence of a posterior pituitary 'bright spot'. We recently encountered a case of CNS langerhans cell histiocytosis with no posterior pituitary 'bright spot' and with lesions involving the cerebellum and basal ganglia but not the hypothalamic-pituitary axis.

  20. Langerhans' cell histiocytosis presenting with an intracranial epidural hematoma

    Energy Technology Data Exchange (ETDEWEB)

    Lee, K.-W. [Department of Pediatrics, Loma Linda Children' s Hospital and University Medical Center, Loma Linda, CA (United States); McLeary, M.S. [Div. of Pediatric Radiology, Loma Linda Children' s Hospital and University Medical Center, Loma Linda, CA (United States); Zuppan, C.W. [Dept. of Pathology, Loma Linda Children' s Hospital and University Medical Center, Loma Linda, CA (United States); Won, D.J. [Div. of Pediatric Neurosurgery, Loma Linda University Children' s Hospital, Loma Linda, CA (United States)

    2000-05-01

    An 8-year-old boy developed vomiting and severe headache following minor head trauma. A CT scan of the head demonstrated a lytic lesion of the skull and adjacent epidural hematoma. Surgical evacuation and removal of the skull lesion and hematoma were carried out, and pathologic evaluation resulted in a diagnosis of Langerhans' cell histiocytosis (LCH). Epidural involvement of Langerhans' cell histiocytosis is very rare, and we report the first case of LCH presenting as an intracranial epidural hematoma. (orig.)

  1. Differentiating skin-limited and multisystem Langerhans cell histiocytosis

    Science.gov (United States)

    Simko, Stephen J.; Garmezy, Benjamin; Abhyankar, Harshal; Lupo, Philip J.; Chakraborty, Rikhia; Lim, Karen Phaik Har; Shih, Albert; Hicks, M. John; Wright, Teresa S.; Levy, Moise L.; McClain, Kenneth L.; Allen, Carl E.

    2014-01-01

    Objective To identify features associated with multisystem involvement and therapeutic failure in patients with skin Langerhans cell histiocytosis (LCH). Study design We reviewed medical records of 71 consecutive LCH patients with skin involvement evaluated at Texas Children’s Hospital and analyzed clinical features, laboratory results, and presence of circulating cells with the BRAF-V600E mutation, with respect to initial staging and clinical outcomes. Results Skin disease in patients older than 18 months at diagnosis was associated with presence of multisystem disease (OR 9.65, 95% CI 1.17–79.4). Forty percent of patients referred for presumed skin-limited LCH had underlying multisystem involvement, half of these with risk-organ involvement. Patients with skin-limited LCH had 3-year progression-free survival (PFS) of 89% after initial therapy, and none developed multisystem disease. Patients with skin/multisystem involvement had 3 year PFS of 44% with vinblastine/prednisone therapy, and risk-organ involvement did not correlate with failure to achieve non-active disease. Circulating cells with BRAF-V600E were detected at higher frequency in multisystem patients (8/11 skin/multisystem, 1/13 skin-limited, P=0.002). Conclusions Skin-limited LCH requires infrequent therapeutic intervention and has lower risk of progression relative to skin plus multisystem LCH. The less aggressive clinical course and lack of circulating cells with BRAF-V600E mutation in skin-limited LCH suggest a different mechanism of disease origin compared with multisystem or risk-organ disease. PMID:25441388

  2. Melanotic paraganglioma arising in the temporal horn following Langerhans cell histiocytosis

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    Yoo, Jeong Hyun [Baylor College of Medicine, Neuroradiology Department, Houston, TX (United States); Ewha Womans University, Radiology Department, School of Medicine, Seoul (Korea); Rivera, Andreana [Baylor College of Medicine, Pathology Department, Houston, TX (United States); Naeini, Ramin M.; Yedururi, Sireesha; Megahead, Hatem [Baylor College of Medicine, Radiology Department, Houston, TX (United States); Bayindir, Petek [Ege Universitesi, Radiology Department, Lojmanlari (Turkey); Fuller, Gregory N. [MD Anderson Cancer Center, Pathology Department, Houston, TX (United States); Suh, Jeong Soo [Ewha Womans University, Radiology Department, School of Medicine, Seoul (Korea); Adesina, Adekunle M. [Baylor College of Medicine, Pathology Department, Houston, TX (United States); Texas Children' s Cancer Center, Houston, TX (United States); Hunter, Jill V. [Texas Children' s Cancer Center, Houston, TX (United States); Baylor College of Medicine, Neuroradiology Department, Houston, TX (United States)

    2008-05-15

    Intracerebral paragangliomas are rare because of the lack of paraganglial cells in the cerebral tissue. We report a rare case of melanotic paraganglioma arising from the temporal horn of the lateral ventricle in a patient with prior Langerhans cell histiocytosis (LCH) treated with chemotherapy and radiation. (orig.)

  3. Adult Langerhans cell histiocytosis presenting as metachronous colonic polyps

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    Aloísio Felipe-Silva

    2013-03-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disease characterized by proliferation of Langerhans-type cells that express CD1a, Langerin (CD207 and S100 protein. Birbeck granules are a hallmark by ultrastructural examination. LCH presents with a wide clinical spectrum, ranging from solitary lesions of a single site (usually bone or skin to multiple or disseminated multisystemic lesions, which can lead to severe organ dysfunction. Most cases occur in children. Gastrointestinal tract involvement is rare and has been associated with systemic illness and poor prognosis especially in children under the age of 2 years. Adult gastrointestinal LCH is very rare. We report a case of a previously healthy, nonsmoking 48-year-old male who was referred for routine screening colonoscopy. Two sessile, smooth, firm and yellowish LCH polyps measuring 0.2 cm and 0.3 cm were detected in the sigmoid colon. Fifteen months later a second colonoscopy found two histologically confirmed hyperplastic polyps at the sigmoid colon. No other LCH lesions were seen. A third colonoscopy after 28 months of follow-up found a submucosal 0.5 cm infiltrated and ulcerated LCH polyp in the cecum, close to the ostium of the appendix. The patient had been asymptomatic for all this period. Imaging investigation for systemic or multiorgan disease did not find any sign of extracolonic involvement. On histology all lesions showed typical LCH features and immunohistochemical analysis showed strong and diffuse staining for CD1a and CD207. This case illustrates two distinct clinicopathologic features not previously reported in this particular clinical setting: metachronous colonic involvement and positivity for CD207.

  4. Dendritic Cells Cause Bone Lesions in a New Mouse Model of Histiocytosis.

    Science.gov (United States)

    Grosjean, Frédéric; Nasi, Sonia; Schneider, Pascal; Chobaz, Véronique; Liu, Alexandra; Mordasini, Vanessa; Moullec, Kristell; Vezzoni, Paolo; Lavanchy, Christine; Busso, Nathalie; Acha-Orbea, Hans; Ehirchiou, Driss

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare disease caused by the clonal accumulation of dendritic Langerhans cells, which is often accompanied by osteolytic lesions. It has been reported that osteoclast-like cells play a major role in the pathogenic bone destruction seen in patients with LCH and these cells are postulated to originate from the fusion of DCs. However, due to the lack of reliable animal models the pathogenesis of LCH is still poorly understood. In this study, we have established a mouse model of histiocytosis- recapitulating human disease for osteolytic lesions seen in LCH patients. At 12 weeks after birth, severe bone lesions were observed in our multisystem histiocytosis (Mushi) model, when CD8α conventional dendritic cells (DCs) are transformed (MuTuDC) and accumulate. Most importantly, our study demonstrates that bone loss in LCH can be accounted for the transdifferentiation of MuTuDCs into functional osteoclasts both in vivo and in vitro. Moreover, we have shown that injected MuTuDCs reverse the osteopetrotic phenotype of oc/oc mice in vivo. In conclusion, our results support a crucial role of DCs in bone lesions in histiocytosis patients. Furthermore, our new model of LCH based on adoptive transfer of MuTuDC lines, leading to bone lesions within 1-2 weeks, will be an important tool for investigating the pathophysiology of this disease and ultimately for evaluating the potential of anti-resorptive drugs for the treatment of bone lesions.

  5. BISPHOSPHONATES IN LANGERHANS CELL HISTIOCYTOSIS: AN INTERNATIONAL RETROSPECTIVE CASE SERIES

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    Deepak Chellapandian

    2016-07-01

    Full Text Available Background: Bone is the most common organ of involvement in patients with Langerhans cell histiocytosis (LCH, which is often painful and associated with significant morbidity from pathological fractures. Current first-line treatments include chemotherapy and steroids that are effective but often associated with adverse effects, whereas the disease may reactivate despite an initial response to first-line agents. Bisphosphonates are osteoclast inhibitors that have shown to be helpful in treating bone lesions of LCH. To date, there are no large international studies to describe their role in treating bone lesions of LCH. Method: We conducted a multicenter retrospective review of 13 patients with histologically proven LCH, who had received bisphosphonates either at diagnosis or at disease reactivation. Results: Ten patients (77% had a single system bone disease, and 3 (23% had bone lesions as part of multisystem disease. Median follow-up time post-bisphosphonate therapy was 4.6 years (range, 0.8 to 8.2 years. Treatment with bisphosphonates was associated with significant pain relief in almost all patients. Twelve  (92% achieved resolution of active bone lesions, and 10 out of them had no active disease for a median of 3.5 years (range, 0.8 to 5 years. One patient did not respond. No major adverse effects were reported in this series.  Conclusion: Bisphosphonates are well-tolerated drugs that can significantly improve bone pain and induce remission in active bone LCH. Future prospective studies evaluating the role of bisphosphonates in LCH are warranted.

  6. Early Diagnosis and Monitoring of Neurodegenerative Langerhans Cell Histiocytosis

    Science.gov (United States)

    Mortilla, Marzia; Savelli, Sara; Grisotto, Laura; Di Giacomo, Gianpiero; Romano, Katiuscia; Fonda, Claudio; Biggeri, Annibale; Guerrini, Renzo; Aricò, Maurizio

    2015-01-01

    Background Neurodegenerative Langerhans Cell Histiocytosis (ND-LCH) is a rare, unpredictable consequence that may devastate the quality of life of patients cured from LCH. We prospectively applied a multidisciplinary diagnostic work-up to early identify and follow-up patients with ND-LCH, with the ultimate goal of better determining the appropriate time for starting therapy. Methods We studied 27 children and young adults with either ND-LCH verified by structural magnetic resonance imaging (MRI) (group 1) or specific risk factors for (diabetes insipidus, craniofacial bone lesions), but no evidence of, neurodegenerative MRI changes (group 2). All patients underwent clinical, neurophysiological and MRI studies. Results Seventeen patients had MRI alterations typical for ND-LCH. Nine showed neurological impairment but only three were symptomatic; 11 had abnormal somatosensory evoked potentials (SEPs), and five had abnormal brainstem auditory evoked potentials (BAEPs). MR spectroscopy (MRS) showed reduced cerebellar NAA/Cr ratio in nine patients. SEPs showed sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) for predicting ND-LCH of 70.6% (95%CI, 44.0%-89.7%), 100% (69.2%-100%), 100% (73.5%-100%), and 66.7% (38.4%-88.2%), respectively. Repeated investigations in group 1 revealed increasingly abnormal EP parameters, or neurological examination, or both, in nine of fifteen patients while MRI remained unchanged in all but one patient. Conclusion A targeted MRI study should be performed in all patients with risk factors for ND-LCH for early identification of demyelination. The combined use of SEPs and careful neurological evaluation may represent a valuable, low-cost, well-tolerated and easily available methodology to monitor patients from pre-symptomatic to symptomatic stages. We suggest a multidisciplinary protocol including clinical, MRS, and neurophysiological investigations to identify a population target for future

  7. Pulmonary Langerhans Cell Histiocytosis: Case Series and Literature Review

    Science.gov (United States)

    Wei, Ping; Lu, Hai-Wen; Jiang, Sen; Fan, Li-Chao; Li, Hui-Ping; Xu, Jin-Fu

    2014-01-01

    Abstract Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease with insidious onset and nonspecific manifestations. The objective of this article was to characterize the clinical manifestations and features of PLCH by retrospectively analyzing clinical data of patients with PLCH in addition to simultaneous review of literature. A retrospective analysis was conducted on clinical data of patients with PLCH (n = 7), whose conditions were diagnosed by biopsy from pulmonary tissue (n = 6) or enlarged lymph nodes in the neck (n = 1) and confirmed by PLCH typical radiological features on computed tomography (CT) scan, between January 2001 and September 2012 at the Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China. The review of published reports was made to further emphasize the clinical manifestation and radiological features of PLCH. Long history of cigarette smoking was found in 6 patients. Two patients had recurrent pneumothorax and the other 2 had pulmonary arterial hypertension (World Health Organization group 5 pulmonary hypertension), diagnosed through ultrasonic cardiogram. The nodular shadows were revealed by chest CT scan in 5 patients, cystic shadows in 5 patients, and reticular shadows in 2 patients, as major manifestations, respectively; most of the lesions were located in the middle or upper segments of the lung. The obvious shrank of lesion was found in 1 patient after completely quitting smoking. The pathogenesis of PLCH might be closely associated with smoking. The cystic or nodular lesion was the typical radiological features. Further prospective studies with large sample size are required to further validate the study results and understand the clinical characteristics of PLCH to avoid misdiagnosis. PMID:25415669

  8. The cognitive spectrum in neurodegenerative Langerhans cell histiocytosis.

    Science.gov (United States)

    Le Guennec, Loïc; Decaix, Caroline; Donadieu, Jean; Santiago-Ribeiro, Maria; Martin-Duverneuil, Nadine; Levy, Richard; Delgadillo, Daniel; Kas, Aurélie; Drier, Aurélie; Magy, Laurent; Bayen, Eleonore; Hoang-Xuan, Khe; Idbaih, Ahmed

    2014-08-01

    Clinical spectrum of cognitive troubles complicating neurodegenerative Langerhans cell histiocytosis (ND-LCH) is poorly known. The aim of this study is to evaluate cognitive functions in ND-LCH. The cognitive functions of a series of eight adult patients (7 males and 1 female; mean age 26 years IQ 25-75; range 20-33) suffering from clinical and/or radiological ND-LCH were evaluated using the following tests: (1) forward/backward digit and spatial span tasks of the WAIS-R scale and the Corsi block task, (2) the French version of the free and cued selective reminding test, (3) verbal fluency tests, (4) the Frontal Assessment Battery (FAB), (5) backward measurement of the verbal and visuospatial memories of the WAIS-R scale, (6) the Rey complex figure test, (7) the trail making tests A and B, (8) digit symbol and symbol search of the WAIS-IV scale, and (9) the Stroop test. Episodic (i.e. autobiographical or personal) memory free recall, categorical verbal fluency, phonological verbal fluency, visuospatial processing skills, attention, speed of processing, and sensitivity to interference were impaired in ND-LCH patients. In contrast, verbal and visuospatial short-term memories (i.e. immediate memories or forward span tasks) were preserved in all patients. Adult ND-LCH patients suffer from a severe but dissociated dysexecutive syndrome, mostly affecting executive strategies and relatively sparing short-term memory. Our study supports the need of assessing executive functions using comprehensive cognitive evaluation in ND-LCH patients for early diagnosis.

  9. Early Diagnosis and Monitoring of Neurodegenerative Langerhans Cell Histiocytosis.

    Directory of Open Access Journals (Sweden)

    Elena Sieni

    Full Text Available Neurodegenerative Langerhans Cell Histiocytosis (ND-LCH is a rare, unpredictable consequence that may devastate the quality of life of patients cured from LCH. We prospectively applied a multidisciplinary diagnostic work-up to early identify and follow-up patients with ND-LCH, with the ultimate goal of better determining the appropriate time for starting therapy.We studied 27 children and young adults with either ND-LCH verified by structural magnetic resonance imaging (MRI (group 1 or specific risk factors for (diabetes insipidus, craniofacial bone lesions, but no evidence of, neurodegenerative MRI changes (group 2. All patients underwent clinical, neurophysiological and MRI studies.Seventeen patients had MRI alterations typical for ND-LCH. Nine showed neurological impairment but only three were symptomatic; 11 had abnormal somatosensory evoked potentials (SEPs, and five had abnormal brainstem auditory evoked potentials (BAEPs. MR spectroscopy (MRS showed reduced cerebellar NAA/Cr ratio in nine patients. SEPs showed sensitivity, specificity, positive predictive value (PPV and negative predictive value (NPV for predicting ND-LCH of 70.6% (95%CI, 44.0%-89.7%, 100% (69.2%-100%, 100% (73.5%-100%, and 66.7% (38.4%-88.2%, respectively. Repeated investigations in group 1 revealed increasingly abnormal EP parameters, or neurological examination, or both, in nine of fifteen patients while MRI remained unchanged in all but one patient.A targeted MRI study should be performed in all patients with risk factors for ND-LCH for early identification of demyelination. The combined use of SEPs and careful neurological evaluation may represent a valuable, low-cost, well-tolerated and easily available methodology to monitor patients from pre-symptomatic to symptomatic stages. We suggest a multidisciplinary protocol including clinical, MRS, and neurophysiological investigations to identify a population target for future therapeutic trials.

  10. Optimal therapy for adults with Langerhans cell histiocytosis bone lesions.

    Directory of Open Access Journals (Sweden)

    Maria A Cantu

    Full Text Available BACKGROUND: There is little data on treatment of Langerhans cell histiocytosis (LCH in adults. Available data is on small numbers of patients with short follow-up times and no comparison of results from different treatment regimens. We analyzed the responses of adult LCH patients with bone lesions to three primary chemotherapy treatments to define the optimal one. METHODS AND FINDINGS: Fifty-eight adult patients with bone lesions, either as a solitary site or as a component of multisystem disease, were analyzed for disease location and response to surgery, curettage, steroids, radiation, vinblastine/prednisone, 2-Chlorodeoxyadenosine (2-CdA, or cytosine arabinoside (ARA-C. The mean age of patients was 32 years, with equal gender distribution. Twenty-nine patients had 1 lesion; 16, 2 lesions; 5, 3 lesions; and 8 had 4 or more. Most bone lesions were in the skull, spine, or jaw. Chemotherapy, surgery, curettage, or radiation, but not steroids alone, achieved improvement or resolution of lesions in a majority of patients. Comparison of the three chemotherapy regimens revealed 84% of patients treated with vinblastine/prednisone either did not respond or relapsed within a year, whereas 59% of patients treated with 2-CdA and 21% treated with ARA-C failed. Toxicity was worse with the vinblastine/prednisone group as 75% had grade 3-4 neuropathy. Grade 3-4 cytopenias occurred in 37% of the 2-CdA -treated patients and 20% of the ARA-C-treated patients. The major limitation of this study is it is retrospective and not a clinical trial. CONCLUSIONS: ARA-C is an effective and minimally toxic treatment for LCH bone lesions in adults. In contrast, vinblastine/prednisone results in poor overall responses and excessive toxicity.

  11. Orbital manifestations of Langerhans Cell Histiocytosis: A report of three cases

    Directory of Open Access Journals (Sweden)

    Jayanta K Das

    2009-01-01

    Full Text Available Langerhans Cell Histiocytosis (LCH is a spectrum of disorders characterized by accumulation of histiocytes in various tissues. It is rarely encountered in ophthalmic practice and has an affinity for the orbit. We report three patients with LCH involving the lateral orbital wall, each with a different form of the condition.

  12. Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings

    Energy Technology Data Exchange (ETDEWEB)

    Kilborn, T.N.; Teh, J.; Goodman, T.R

    2003-04-01

    Langerhans cell histiocytosis is a rare disease in children. However, its ability to present in many ways, to mimic other conditions, and to manifest itself in many organs makes it a fascinating disease for radiologists. This article reviews the history of the disease, the features that are most useful in determining prognosis, and the various radiological findings seen in paediatric patients.

  13. Imaging findings in Langerhans` cell histiocytosis of the liver and the spleen in an adult

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    Mampaey, S. [Dept. of Radiology, Aalsters Stedelijk Ziekenhuis, Aalst (Belgium)]|[Dept. of Radiology, University of Antwerp (Belgium); Warson, F. [Dept. of Anatomopathology, Aalsters Stedelijk Ziekenhuis, Aalst (Belgium); Hedent, E. van [Dept. of Radiology, Aalsters Stedelijk Ziekenhuis, Aalst (Belgium); Schepper, A.M. de [Dept. of Radiology, University of Antwerp (Belgium)

    1999-02-01

    We present a case of Langerhans` cell histiocytosis (LCH) of the liver and spleen in an adult. The imaging features are different from those in the few previously reported cases of individual organ involvement by LCH. (orig.) (orig.) With 2 figs., 5 refs.

  14. Recurrent adult-onset hypophyseal Langerhans cell histiocytosis after radiotherapy: a case report

    Directory of Open Access Journals (Sweden)

    Funk Ryan K

    2012-10-01

    Full Text Available Abstract Introduction Langerhans cell histiocytosis is a rare disease within the adult population, with very few cases reported as solitary hypophyseal lesions in adults. Of the reported cases, most have been treated successfully with surgery, radiotherapy, and/or chemotherapy. Radiotherapy has been thought to be curative at the relatively low dose of 20Gy. Here we report a case of recurrent hypophyseal Langerhans cell histiocytosis 9 months after radiotherapy with an interval period of symptomatic and radiographic response to therapy. Case presentation A 50-year-old Caucasian woman who had headaches, memory difficulties, and diabetes insipidus was found to have a 2.5cm suprasellar mass. Langerhans cell histiocytosis was diagnosed following stereotactic brain biopsy. Further workup revealed no other lesions. Initial radiation treatment succeeded in shrinking the tumor and relieving clinical symptoms temporarily; however, growth and recurrence of clinical symptoms was noted at 9 months. Re-irradiation was well tolerated and the patient had no acute side effects. Conclusion Isolated hypophyseal involvement by Langerhans cell histiocytosis in adults is a unique presentation of a rare disease. Although radiotherapy doses as low as 20Gy have been reported to offer control, this case demonstrates that higher doses may be warranted to ensure tumor control. With modern imaging and radiotherapy techniques higher doses should offer little increased more durable risk to surrounding critical structures.

  15. Central diabetes insipidus: Is it Langerhans cell histiocytosis of the pituitary stalk? A diagnostic pitfall.

    NARCIS (Netherlands)

    Prosch, H.; Grois, N.; Bökkerink, J.P.M.; Prayer, D.; Leuschner, I.; Minkov, M.; Gadner, H.

    2006-01-01

    Central diabetes insipidus (CDI) is a rare disorder that may be caused by a variety of diseases. In pediatric and adolescent patients the most common causes for CDI are Langerhans cell histiocytosis (LCH) and germinoma. To avoid a potentially hazardous biopsy of the hypothalamic pituitary region it

  16. Langerhans cell histiocytosis of the cervical spine: case report of an unusual location

    Energy Technology Data Exchange (ETDEWEB)

    Geusens, E.; Brys, P.; Ghekiere, J.; Baert, A.L. [Department of Radiology, University Hospital Gasthuisberg KU Leuven (Belgium); Samson, I. [Department of Orthopedic Surgery, University Hospitals, Leuven (Belgium); Sciot, R. [Department of Pathology II, University Hospitals, Leuven (Belgium); Brock, P. [Department of Pediatrics, University Hospitals, Leuven (Belgium)

    1998-09-01

    An unusual location for Langerhans cell histiocytosis of the cervical spine is presented. The osteolytic lesion, instead of being located in the vertebral body, was visualised in the left lateral mass of the fifth cervical vertebra, extending into the vertebral body and through the interapophyseal joint into the lateral mass of the fourth cervical vertebra. (orig.) With 3 figs., 7 refs.

  17. Cutaneous histiocytosis syndromes.

    Science.gov (United States)

    Roper, S S; Spraker, M K

    1985-11-01

    Cutaneous histiocytosis may take two principal forms. It is either a benign proliferative process or a relentless, progressive process with a poor prognosis. In histiocytic medullary reticulosis, histiocytes demonstrate nuclear atypia and the outcome is uniformly fatal. Benign cephalic histiocytosis X causes lesions similar to those of histiocytosis X, but Langerhans' cells are absent. In congenital self-healing histiocytosis X, the Letterer-Siwe-like cutaneous infiltrate contains Langerhans' cells, but the lesions heal spontaneously without treatment. The nodular cutaneous lesions of juvenile xanthogranuloma appear in infancy and resolve without treatment; however, the higher percentage (10%) of associated ocular lesions may lead to glaucoma and blindness. In histiocytosis X, the cutaneous lesions show a marked proliferation of Langerhans' cells, with prognosis dependent on the patient's age and the extent of organ dysfunction. Patients who survive the acute form of the disease may develop diabetes insipidus, growth retardation, pulmonary fibrosis, and biliary cirrhosis. A subtle immunologic defect has been identified in patients with histiocytosis X, yet the pathogenesis of the disease is still speculative. Familial disease occurring in early infancy should be differentiated from complete or partial immunodeficiency syndromes. Guidelines for evaluating patients with cutaneous histiocytosis are reviewed.

  18. Solitary cutaneous histiocytosis with granular cell changes: a morphological variant of reticulohistiocytoma?

    Science.gov (United States)

    Caltabiano, Rosario; Magro, Gaetano; Vecchio, Giada Maria; Lanzafame, Salvatore

    2010-02-01

    We first report a case of granular cell histiocytosis occurring as a solitary polypoid lesion of the nipple in a 15-year-old girl. Histologically, the lesion was composed of a dermal population of medium- to large-sized, short spindle- to round- to epithelioid-shaped cells with eosinophilic cytoplasm containing numerous and small diastase-resistant periodic acid-Schiff (PAS) positive granules. No associated inflammatory cells were observed. Immunohistochemical studies, revealing immunoreactivity exclusively to vimentin and CD68, were consistent with their histiocytic profile. Based on clinical, morphological and immunohistochemical features, the diagnosis of 'solitary cutaneous histiocytosis with granular cell changes' was proposed. The absence of an inflammatory cell component, such as lymphocytes and leucocytes, along with no history of a previous trauma or medical treatment, suggest that the present lesion could fit into the morphological spectrum of the so-called solitary epithelioid histiocytoma, also known as reticulohistiocytoma. Alternatively, the possibility of a histiocytic reaction to unknown stimuli cannot be completely ruled out. Nevertheless, awareness of solitary cutaneous histiocytosis with granular cell changes is useful to avoid confusion with other dermal tumors, especially 'granular cell tumor' and 'dermal non-neural granular cell tumor'.

  19. "Recurrent Bilateral Spontaneous Pneumothorax in Early Infancy: A Case of Langerhans Cell Histiocytosis"

    Directory of Open Access Journals (Sweden)

    S. Alavi

    2005-07-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disorder characterized by infiltration of either single or multiple organs by S100 and CD1a positive cells. Patients with pulmonary LCH are predisposed to pneumothorax due to destructive changes in the lung parenchyma. Here, we report a case of multisystem LCH who presented at 2 months of age with simultaneous bilateral spontaneous pneumothorax.

  20. Unusual cutaneous histiocytosis expressing an intermediate immunophenotype between Langerhans' cells and dermal macrophages.

    Science.gov (United States)

    Berti, E; Gianotti, R; Alessi, E

    1988-08-01

    Cutaneous histiocytosis was discovered in a 40-year-old man with a slow-growing nodule located on his right arm. Histologic findings showed an epidermotropic infiltrate of histiocytes with folded, irregular nuclei. Immunologically, the cells presented an intermediate phenotype between Langerhans' cells and dermal macrophages. After surgical removal of the lesion, neither a relapse nor visceral involvement was observed during two years of follow-up.

  1. Biliary wall calcification in Langerhans cell histiocytosis: report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Caruso, Settimo; Miraglia, Roberto; Maruzzelli, Luigi; Luca, Angelo; Gridelli, Bruno [Mediterranean Institute for Transplantation and Advanced Specialized Therapies (IsMeTT), Department of Diagnostic and Interventional Radiology, Palermo (Italy)

    2008-07-15

    Langerhans cell histiocytosis (LCH) is a disorder of unknown pathogenesis affecting one or more organs (unifocal or disseminated form) due to clonal proliferation of Langerhans cells. Liver involvement is more frequent in the disseminated form and the radiological findings of end-stage liver disease due to LCH are similar to those of sclerosing cholangitis. We present the multidetector CT findings in two children with LCH liver involvement and the unique finding of calcification of the biliary wall. (orig.)

  2. Langerhans cell histiocytosis with oral manifestations: a rare and unusual case report.

    Science.gov (United States)

    Yashoda-Devi, Bk; Rakesh, N; Agarwal, Manjushree

    2012-10-01

    Langerhans cell histiocytosis (LCH), is a rare, proliferative disorder in which the accumulation of pathologic Langerhans cells leads to local tissue infiltration and destruction. We present a case of a 32 years old, completely edentulous female patient who presented with erythema of hard palate, maxillary alveolar mucosa and mucosa over the distobuccal part of mandibular alveolar ridge with foci of ulcerations. Histopathologic features were suggestive of LCH which was confirmed by immunohistochemistry which was CD1a positive, confirmatory for LCH. Bone scan revealed multiple bone involvement. At this stage, disease had already progressed to multisystem involvement with endocrinal abnormalities (primary hypothyroidism and hyperprolactinemia), requiring aggressive treatment. Therefore, this case is a reminder of the possibility of occurrence of this rare disease in the oral cavity which might manifest itself in multiple presentations thus easily leading to the misdiagnosis and therefore, it could be easily overlooked by dentists. Key words:Langerhans cell histiocytosis, immunohistochemistry, bone scan.

  3. Langerhans cell histiocytosis in adults: a case report and review of the literature

    Science.gov (United States)

    2016-01-01

    Background Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocyte-like cells that generally affects children. Immunohistochemistry is essential to obtain the correct diagnosis, and treatment protocols are controversial. Objective Langerhans cell histiocytosis (LCH) is easy to be misdiagnosed because of its various clinic features and laboratory results. This research focused on the clinicopathological, histopathological, immunohistochemical and other features of LCH and aimed to analyze LCH clinical features for improving diagnosis and decreasing misdiagnosis rate. Case report A case of rare adult LCH was reported and the clinicopathological features were summarized by literature review. The multifocal form of this case includes diabetes insipidus, exophthalmos and mucocutaneous lesions in axillae and anogenital regions, such as infiltrated nodules, extensive coalescing, scaling, crusted papules and ulcerated plaques. The Langerhans cells diffusely infiltrated in the dermis and the tumor cells were positive for CD1a and S-100 expression. The diagnosis was Langerhans cell histiocytosis based on the pathological and immunohistochemical changes. Conclusion LCH has high rate of misdiagnosis and definitive diagnosis depends on pathological biopsy and X-ray examination. The prognosis is related to the onset age and the quantity of affected organs. Although specific therapeutic approach hasn't been well established, combined chemotherapy for multisystem lesions and surgical operation or radiotherapy for unifocal lesions may improve the therapy. PMID:26942568

  4. Langerhans cell histiocytosis arising from a BCC: a case report and review of the literature.

    Science.gov (United States)

    Patel, Payal; Talpur, Rakhshandra; Duvic, Madeleine

    2010-06-01

    Langerhans cell histiocytosis (LCH) is a rare disease characterized by a proliferation of Langerhans cells. Several organs may be involved, including the skin, bone, and central nervous system. Adult onset of LCH and solely localized cutaneous involvement are quite uncommon. Langerhans cell histiocytosis has been found in combination with other skin lesions and systemic conditions, but no definitive conclusion exists for this phenomenon. We present a case report of a 63-year-old woman who initially presented with 3 pink papules on her forehead that had developed sequentially within 1 month, all diagnosed by biopsy as basal cell carcinoma (BCC) and appropriately treated. Concurrent with the appearance of the third BCC, the patient began developing crusted ulcerative nodules on her scalp. Biopsy of 1 scalp nodule revealed a BCC, but a repeat biopsy of the same nodule weeks later revealed LCH. Langerhans cell histiocytosis arising from a BCC is extremely rare. No absolute explanation exists regarding the transformation of a BCC into LCH, but understanding the behavior of Langerhans cells may give us better insight into how this process could occur.

  5. B-RAF mutant alleles associated with Langerhans cell histiocytosis, a granulomatous pediatric disease.

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    Takeshi Satoh

    Full Text Available BACKGROUND: Langerhans cell histiocytosis (LCH features inflammatory granuloma characterised by the presence of CD1a+ dendritic cells or 'LCH cells'. Badalian-Very et al. recently reported the presence of a canonical (V600EB-RAF mutation in 57% of paraffin-embedded biopsies from LCH granuloma. Here we confirm their findings and report the identification of two novel B-RAF mutations detected in LCH patients. METHODS AND RESULTS: Mutations of B-RAF were observed in granuloma samples from 11 out of 16 patients using 'next generation' pyrosequencing. In 9 cases the mutation identified was (V600EB-RAF. In 2 cases novel polymorphisms were identified. A somatic (600DLATB-RAF insertion mimicked the structural and functional consequences of the (V600EB-RAF mutant. It destabilized the inactive conformation of the B-RAF kinase and resulted in increased ERK activation in 293 T cells. The (600DLATB-RAF and (V600EB-RAF mutations were found enriched in DNA and mRNA from the CD1a+ fraction of granuloma. They were absent from the blood and monocytes of 58 LCH patients, with a lower threshold of sequencing sensitivity of 1%-2% relative mutation abundance. A novel germ line (T599AB-RAF mutant allele was detected in one patient, at a relative mutation abundance close to 50% in the LCH granuloma, blood monocytes and lymphocytes. However, (T599AB-RAF did not destabilize the inactive conformation of the B-RAF kinase, and did not induce increased ERK phosphorylation or C-RAF transactivation. CONCLUSIONS: Our data confirmed presence of the (V600EB-RAF mutation in LCH granuloma of some patients, and identify two novel B-RAF mutations. They indicate that (V600EB-RAF and (600DLATB-RAF mutations are somatic mutants enriched in LCH CD1a(+ cells and absent from the patient blood. Further studies are needed to assess the functional consequences of the germ-line (T599AB-RAF allele.

  6. Intracranial non-Langerhans cell histiocytosis presenting as an isolated intraparenchymal lesion

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    Rajaram, Smitha; Shackley, Fiona; Raghavan, Ashok [Western Bank, Sheffield Children' s Hospital, Sheffield (United Kingdom); Wharton, Stephen B. [University of Sheffield, Department of Neurosciences, Sheffield (United Kingdom); Connolly, Daniel J.A. [Western Bank, Sheffield Children' s Hospital, Sheffield (United Kingdom); University of Sheffield, Academic Radiology, Sheffield (United Kingdom)

    2010-12-15

    Non-Langerhans cell histiocytosis in the absence of cutaneous or other organ involvement is very rare. A Caucasian boy age 3 years 11 months presented with episodes of recurrent right-side seizures over 2 weeks. Brain CT and MR imaging showed a single enhancing left frontal lobe lesion. Stereotactic biopsy was performed and histological examination showed diffuse infiltrate of macrophages with foamy cytoplasm. Four months later there was recurrence of seizure activity despite anti-epileptic medication and a repeat MR scan showed a persistent enhancing lesion in the left frontal lobe. Histological examination of the resection specimen resembled juvenile xanthogranuloma (JXG) involving the central nervous system. In the absence of skin lesions a diagnosis of non-Langerhans cell histiocytosis was made. The child made a full recovery following surgery with resolution of his symptoms. (orig.)

  7. B-RAF Mutant Alleles Associated with Langerhans Cell Histiocytosis, a Granulomatous Pediatric Disease

    Science.gov (United States)

    Lu, Hui-chun; Mian, Sophie; Trouillet, Celine; Mufti, Ghulam; Emile, Jean-Francois; Fraternali, Franca; Donadieu, Jean; Geissmann, Frederic

    2012-01-01

    Background Langerhans cell histiocytosis (LCH) features inflammatory granuloma characterised by the presence of CD1a+ dendritic cells or ‘LCH cells’. Badalian-Very et al. recently reported the presence of a canonical V600EB-RAF mutation in 57% of paraffin-embedded biopsies from LCH granuloma. Here we confirm their findings and report the identification of two novel B-RAF mutations detected in LCH patients. Methods and Results Mutations of B-RAF were observed in granuloma samples from 11 out of 16 patients using ‘next generation’ pyrosequencing. In 9 cases the mutation identified was V600EB-RAF. In 2 cases novel polymorphisms were identified. A somatic 600DLATB-RAF insertion mimicked the structural and functional consequences of the V600EB-RAF mutant. It destabilized the inactive conformation of the B-RAF kinase and resulted in increased ERK activation in 293 T cells. The 600DLATB-RAF and V600EB-RAF mutations were found enriched in DNA and mRNA from the CD1a+ fraction of granuloma. They were absent from the blood and monocytes of 58 LCH patients, with a lower threshold of sequencing sensitivity of 1%–2% relative mutation abundance. A novel germ line T599AB-RAF mutant allele was detected in one patient, at a relative mutation abundance close to 50% in the LCH granuloma, blood monocytes and lymphocytes. However, T599AB-RAF did not destabilize the inactive conformation of the B-RAF kinase, and did not induce increased ERK phosphorylation or C-RAF transactivation. Conclusions Our data confirmed presence of the V600EB-RAF mutation in LCH granuloma of some patients, and identify two novel B-RAF mutations. They indicate that V600EB-RAF and 600DLATB-RAF mutations are somatic mutants enriched in LCH CD1a+ cells and absent from the patient blood. Further studies are needed to assess the functional consequences of the germ-line T599AB-RAF allele. PMID:22506009

  8. Laryngeal Langerhans Cell Histiocytosis Presenting with Neck Mass in an Adult Woman

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    Hesam Jahandideh

    2016-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a very rare condition that commonly affects the head and neck region. There are very few cases of isolated laryngeal involvement by LCH, mostly reported in pediatric patients. Here, we report a case of laryngeal LCH in a 62-year-old woman presenting with a neck mass several weeks ago. The clinical and histopathological findings are reported with a brief discussion about the disease.

  9. Congenital solid neck mass: a unique presentation of Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Tantiwongkosi, Bundhit [Northeastern Ohio Universities College of Medicine and Canton Affiliated Hospitals, Department of Radiology, Canton, OH (United States); Goske, Marilyn J. [Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Steele, Mark [Akron Children' s Hospital, Department of Pathology, Akron, OH (United States)

    2008-05-15

    Langerhans cell histiocytosis (LCH) presenting in the neonatal period is very rare. In most cases, a self-limited cutaneous disease is the exclusive manifestation. We report an unusual case of neonatal LCH presenting with a large congenital solid neck mass without skin lesions. LCH should be considered in the differential diagnosis of solid masses in neonates and prompt physicians to search for visceral organ involvement. (orig.)

  10. Langerhans cell histiocytosis of long bones: MR imaging and complete follow up study

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    Mohammad A Hashmi

    2012-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a relatively rare disease affecting the reticuloendothelial system in the pediatric age group. It can affect bones, lung, liver, spleen, lymph nodes and skin. MR imaging is particularly informative in diagnosis and management of bone LCH. In this report, we present the initial and 23 months post-treatment MR images of a femoral LCH lesion in a 12-year-old child to describe the role of MRI in bone LCH.

  11. Primary cerebral non-Langerhans cell histiocytosis: MRI and differential diagnosis

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    Ernemann, U.; Skalej, M.; Voigt, K. [Department of Neuroradiology, University Hospital Tuebingen, Hoppe-Seyler-Strasse 3, 72076 Tuebingen (Germany); Hermisson, M.; Platten, M. [Department of Neurology, University Hospital Tuebingen, Hoppe-Seyler-Strasse 3, 72076 Tuebingen (Germany); Jaffe, R. [Pathology Department, Children' s Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213 (United States)

    2002-09-01

    We report a young woman with primary cerebral non-Langerhans cell histiocytosis of the juvenile xanthogranuloma family. The clinical course was complicated by extensive infiltration of cranial nerves and meninges and epi- and intramedullary spinal dissemination. Whereas the cutaneous form of juvenile xanthogranuloma is usually benign and self-limited, central nervous system involvement is associated with high morbidity and mortality and might therefore be considered a separate clinical entity. (orig.)

  12. Laryngeal Langerhans Cell Histiocytosis Presenting with Neck Mass in an Adult Woman

    Science.gov (United States)

    Jahandideh, Hesam; Nasoori, Yasser; Rostami, Sara; Safdarian, Mahdi

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a very rare condition that commonly affects the head and neck region. There are very few cases of isolated laryngeal involvement by LCH, mostly reported in pediatric patients. Here, we report a case of laryngeal LCH in a 62-year-old woman presenting with a neck mass several weeks ago. The clinical and histopathological findings are reported with a brief discussion about the disease. PMID:27127670

  13. Anesthesia Technique In A Patient With Langerhans Cell Histiocytosis For Diagnostic Biopsy

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    Abdi S

    2005-07-01

    Full Text Available Histiocytosis under general anesthesia. In these patients, depend on organs involvement and dysfunction, anesthesia technique and specific drugs should be selected.of langerhans cells consist of a range of clinical manifestations, including bone eosimophilic granuloma, Hand-Schuller-Chiristian syndrome and Letterer-Siwe disease. Definitive diagnosis is based on biopsy of involved tissues. This report describes anesthesia technique in a 2 years old girl for diagnostic biopsy

  14. Polyclonal T-Cells Express CD1a in Langerhans Cell Histiocytosis (LCH) Lesions

    Science.gov (United States)

    West, Jennifer A.; Olsen, Sharon L.; Mitchell, Jenée M.; Priddle, Ross E.; Luke, Jennifer M.; Åkefeldt, Selma Olsson; Henter, Jan-Inge; Turville, Christopher; Kannourakis, George

    2014-01-01

    Langerhans cell histiocytosis (LCH) is a complex and poorly understood disorder that has characteristics of both inflammatory and neoplastic disease. By using eight-colour flow cytometry, we have identified a previously unreported population of CD1a+/CD3+ T-cells in LCH lesions. The expression of CD1a is regarded as a hallmark of this disease; however, it has always been presumed that it was only expressed by pathogenic Langerhans cells (LCs). We have now detected CD1a expression by a range of T-cell subsets within all of the LCH lesions that were examined, establishing that CD1a expression in these lesions is no longer restricted to pathogenic LCs. The presence of CD1a+ T-cells in all of the LCH lesions that we have studied to date warrants further investigation into their biological function to determine whether these cells are important in the pathogenesis of LCH. PMID:25343480

  15. Polyclonal T-cells express CD1a in Langerhans cell histiocytosis (LCH lesions.

    Directory of Open Access Journals (Sweden)

    Jennifer A West

    Full Text Available Langerhans cell histiocytosis (LCH is a complex and poorly understood disorder that has characteristics of both inflammatory and neoplastic disease. By using eight-colour flow cytometry, we have identified a previously unreported population of CD1a(+/CD3(+ T-cells in LCH lesions. The expression of CD1a is regarded as a hallmark of this disease; however, it has always been presumed that it was only expressed by pathogenic Langerhans cells (LCs. We have now detected CD1a expression by a range of T-cell subsets within all of the LCH lesions that were examined, establishing that CD1a expression in these lesions is no longer restricted to pathogenic LCs. The presence of CD1a(+ T-cells in all of the LCH lesions that we have studied to date warrants further investigation into their biological function to determine whether these cells are important in the pathogenesis of LCH.

  16. Lacrimal gland and perioptic nerve lesions due to Langerhans cell histiocytosis (2007: 9b)

    Energy Technology Data Exchange (ETDEWEB)

    Herman, M.; Demaerel, P.; Wilms, G. [University Hospitals Leuven, Department of Radiology, Leuven (Belgium); Gool, S. van [University Hospitals Leuven, Department of Pediactrics, Leuven (Belgium); Casteels, I. [University Hospitals Leuven, Department of Ophthalmology, Leuven (Belgium)

    2007-12-15

    We report a patient presenting with bilateral lacrimal gland involvement and perioptic nerve sheath lesions due to Langerhans cell histiocytosis (LCH) invasion. LCH is a rare multisystemic disease characterized by a clonal proliferation of Langerhans cells. All organs may be involved with a clinical spectrum ranging from a solitary bone lesion to a severe life-threatening multisystem disease. Osteolytic orbital bone lesions with extension into the adjacent orbital soft tissues have been described. To our knowledge, lacrimal gland involvement has probably been described only once before. Perioptic nerve lesions are also very rare, having been described only three times before. (orig.)

  17. Langerhans Cell Histiocytosis Arising from the Mandible as Diagnosed by US-guided Core Biopsy

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Soo Jin [Center of Thyroid Cancer, National Cancer Center, Goyang (Korea, Republic of); Kim, Eun Kyung [Research Institute of Radiological Science, Yonsei University Heath System, Seoul (Korea, Republic of); Lee, Min Kyung [Eulji University College of Medicine, Eulji University Hospital, Daejeon (Korea, Republic of)

    2010-09-15

    Langerhans cell histiocytosis (LCH) is a clonal proliferative disorder of Langerhans cells. Although LCH is not considered a malignant disease, its appearance on radiographs may be similar to that of a malignant tumor. The diagnosis of LCH is usually made by a soft tissue biopsy, or by bone marrow aspiration or curettage. We present a patient with a mandibular mass confirmed to be LCH by US-guided core needle biopsy, and present a strategy for diagnosing localized LCH of the bone based on the usefulness and reliability of the percutaneous biopsy

  18. [The Langerhans cell histiocytosis with thymic localization as initial and exclusive place].

    Science.gov (United States)

    Hernández Pérez, J M; Franquet Casas, T; Rodríguez, S; Giménez, A

    2007-10-01

    The Langerhans' cell histiocytosis (LCH), also known as Histiocitosis X it is an illness not very frequent granulomatosus etiology not clarified yet, that it can have different manifestations and localizations, however the thymic localization as initial and exclusive place gives presentation HCL it is quite unusual. The present case is presented a patient that debuted with a clinical unspecific, where the tests give image they put she gives apparent a mass in previous mediastinum and that after the pathologic and immunohistochemical analysis they evidenced a proliferation Langerhans s cells and eosinophils it being positive for CD1a and S-100 confirming the diagnosis of the LCH.

  19. Niemann-Pick disease, type B with TRAP-positive storage cells and secondary sea blue histiocytosis

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    R. Saxena

    2009-09-01

    Full Text Available We present 2 cases of Niemann Pick disease, type B with secondary sea-blue histiocytosis. Strikingly, in both cases the Pick cells were positive for tartrate resistant acid phosphatase, a finding hitherto described only in Gaucher cells. This report highlights the importance of this finding as a potential cytochemical diagnostic pitfall in the diagnosis of Niemann Pick disease.

  20. Combined Cutaneous Rosai-Dorfman Disease and Localized Cutaneous Langerhans Cell Histiocytosis Within a Single Subcutaneous Nodule

    Science.gov (United States)

    Litzner, Brandon R.; Subtil, Antonio

    2015-01-01

    Abstract: Rosai-Dorfman disease (RDD) is a reactive multisystem histiocytosis that typically presents with cervical lymphadenopathy and systemic symptoms. Cutaneous involvement occurs in approximately 10% of cases, and 3% of cases are limited to the skin without nodal or other extranodal involvement. Langerhans cell histiocytosis (LCH) is a clonal histiocytosis with a wide spectrum of presentations ranging from isolated skin or bone disease to multisystem involvement. Rare case reports have identified concomitant presentation of RDD and LCH; however, most of these reports have involved LCH and RDD occurring concurrently but at separate sites. We present a rare case of concurrent RDD and LCH presenting within a single skin nodule. The patient did not have any evidence of systemic involvement and has remained stable without additional treatment. We also review the literature on this unusual co-presentation and suggest possible underlying mechanisms. Finally, we recommend baseline laboratory and imaging studies and discuss treatment options based on the available evidence. PMID:26588339

  1. Langerhans` cell histiocytosis of the spine: use of MRI in guiding biopsy

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    Kaplan, G.R.; Saifuddin, A. [Department of Diagnostic Imaging, Royal National Orthopaedic Hospital Trust, Brockley Hill (United Kingdom); Pringle, J.A.S. [Department of Morbid Anatomy, Royal National Orthopaedic Hospital Trust, Brockley Hill (United Kingdom); Noordeen, M.H.; Mehta, M.H. [Department of Spinal Deformities, Royal National Orthopaedic Hospital Trust, Brockley Hill (United Kingdom)

    1998-12-01

    The MRI features of two cases of spinal Langerhans` cell histiocytosis with multilevel involvement are presented in which MRI was of help in differentiating active from inactive healing lesions by the demonstration of signal changes in the vertebral body marrow of the active lesion, manifest as low signal intensity on T1-weighted sequences and high signal intensity on T2-weighted sequences. This distinction could not be made by plain radiography or bone scintigraphy. In cases where biopsy is required for diagnosis, MRI is recommended to guide the biopsy towards levels suggestive of active involvement. (orig.) With 7 figs., 13 refs.

  2. Langerhans' cell histiocytosis of the clivus: case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Hurley, M.E.; Hayes, R. [Dept. of Radiology, Our Lady' s Hospital for Sick Children, Crumlin, Dublin (Ireland); O' Meara, A. [Dept. of Oncology, Our Lady' s Hospital for Sick Children, Dublin (Ireland); Fogarty, E. [Dept. of Orthopaedics, Our Lady' s Hospital for Sick Children, Dublin (Ireland)

    2004-03-01

    This report describes a 5-year-old girl with Langerhans' cell histiocytosis (LCH) of the clivus. To date only five patients, including our patient, have been described with LCH at this site. Our patient differs from those previously reported by her atypical clinical presentation with torticollis, but without a sixth nerve palsy. In addition, she is the first patient to present with concomitant disease elsewhere at the time of diagnosis, i.e. both femoral necks and left proximal humerus. Our patient thus presents unique features and underlines the importance of including LCH in the differential diagnosis of erosive lesions of the clivus. (orig.)

  3. Langerhans Cell Histiocytosis of the Rib in an Adult: A Case Report

    Directory of Open Access Journals (Sweden)

    Sung Hyun Kim

    2016-01-01

    Full Text Available Single-site, single-system Langerhans cell histiocytosis (LCH of the rib is one of the rarest causes of bone tumor in adults. Herein, we report a case of a healthy 35-year-old male who presented with upper back pain that was attributed to a solitary osteolytic lesion at the posterolateral aspect of his sixth rib. For diagnostic confirmation and treatment, partial resection of the sixth rib was performed and pathologic finding was consistent with LCH. At the final follow-up after 2 years, no local recurrence or metastasis was observed.

  4. The rapid evolution of CT findings in pulmonary langerhans cell histiocytosis: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Tae Wook; Lee, Kyung Soo; Cho, Eun Yoon [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2007-07-15

    Imaging findings of pulmonary Langerhans cell histiocytosis (PLCH) demonstrate evolving changes over time, and the radiological transitions shown by imaging tools may allow a prediction of histopathological activity in PLCH. However, there are no reports describing how rapidly CT findings change with time. We describe a case of PLCH that showed a rapid evolutional change of the pulmonary lesions in a 48-year-old man, in which the nodular lesions showed cystic changes within two-month follow-up periods on chest CT scans.

  5. An unusual case of Erdheim-Chester disease with features of Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Furmanczyk, Paul S. [University of Washington Medical Center, Department of Pathology, 1959 NE Pacific, Room BB220, P.O. Box 356100, Seattle, WA (United States); Bruckner, James D. [University of Washington Medical Center, Department of Orthopaedics and Sports Medicine, Seattle, WA (United States); Gillespy, Thurman [Harborview Medical Center, Department of Radiology, Seattle, WA (United States); Rubin, Brian P. [The Cleveland Clinic, Department of Anatomic Pathology, Cleveland, OH (United States)

    2007-09-15

    Erdheim-Chester disease (ECD) and Langerhans cell histiocytosis (LCH) are both exceedingly rare histiocytic proliferations that can involve the skeletal system. We report on a case of ECD with some features suggestive of LCH. Radiographs demonstrated a large lytic lesion in the left femur, with multiple lesions of sclerosis involving both distal femurs and tibias. Both the lytic lesion and a sclerotic lesion were biopsied and demonstrated distinctive histologic features characteristic of ECD in the tibia and features of LCH in the femur. The clinical/radiologic and pathologic features that distinguish ECD and LCH as distinct entities are reviewed, and the underlying biological connection between them is discussed. (orig.)

  6. Langerhans cell histiocytosis following Hodgkin lymphoma: A case report from Iran

    Directory of Open Access Journals (Sweden)

    Nahid Reisi Dehkordi

    2010-01-01

    Full Text Available The occurrence of Langerhans Cell Histiocytosis (LCH in a patient with lymphoma is an indication of a probable relationship between them. The two conditions have similarities both clinically and histopathologically. Occurrence of these two conditions in the same patient, particularly not simultaneously, is rare. According to different management and treatment of these conditions, exact histopathologic evaluation and even using immunohistochemistery (IHC can prevent misdiagnosis. In this report, a 10 year old boy presented who afflicted with LCH 3 years after diagnosis and treatment of mixed cellularity Hodgkin lymphoma.

  7. A rare case of Langerhans cell histiocytosis of the skull in an adult: a systematic review

    Directory of Open Access Journals (Sweden)

    Corinna Chiong

    2013-07-01

    Full Text Available We report a 41-year old male who presented to the Emergency Department after falling while water-skiing. He had a previous medical history included chronic headaches, which had persisted for the last 2-3 months prior to presentation. Computed tomography of the head showed a small hypersensitivity with a small extra axial collection with a maximum thickness of 1 mm. Differential diagnoses included an arachnoid cyst, haemangioma, meningioma or a secondary lesion. A diagnosis of Langerhans Cell Histiocytosis was made based on the histopathology examination and the immunoperoxidase staining.

  8. Langerhans Cell Histiocytosis of the Rib in an Adult: A Case Report

    Science.gov (United States)

    Kim, Sung Hyun; Choi, Moon Young

    2016-01-01

    Single-site, single-system Langerhans cell histiocytosis (LCH) of the rib is one of the rarest causes of bone tumor in adults. Herein, we report a case of a healthy 35-year-old male who presented with upper back pain that was attributed to a solitary osteolytic lesion at the posterolateral aspect of his sixth rib. For diagnostic confirmation and treatment, partial resection of the sixth rib was performed and pathologic finding was consistent with LCH. At the final follow-up after 2 years, no local recurrence or metastasis was observed. PMID:26933424

  9. Extra-osseous involvement of Langerhans' cell histiocytosis in children

    Energy Technology Data Exchange (ETDEWEB)

    Schmidt, Sabine; Gudinchet, Francois [Departments of Radiology and Interventional Radiology, University Hospital Centre - CHUV, Lausanne (Switzerland); Eich, Georg [Department of Paediatric Radiology, Children' s Hospital, Zuerich (Switzerland); Hanquinet, Sylviane [Department of Paediatric Radiology, Hopital Cantonal, Geneva (Switzerland); Tschaeppeler, Heinz [Department of Paediatric Radiology, Children' s Hospital, Bern (Switzerland); Waibel, Peter [Department of Paediatric Radiology, Children' s Hospital, St. Gallen (Switzerland)

    2004-04-01

    The predominant clinical and radiological features of Langerhans' cell histiocytosis (LCH) in children are due to osseous involvement. Extra-osseous disease is far less common, occurring in association with bone disease or in isolation; nearly all anatomical sites may be affected and in very various combinations. The following article is based on a multicentre review of 31 children with extra-osseous LCH. The objective is to summarise the diverse possibilities of organ involvement. The radiological manifestations using different imaging modalities are rarely pathognomonic on their own. Nevertheless, familiarity with the imaging findings, especially in children with systemic disease, may be essential for early diagnosis. (orig.)

  10. Diabetes insipidus and Langerhans cell histiocytosis: a case report of reversibility with 2-chlorodeoxyadenosine.

    Science.gov (United States)

    Ottaviano, Fabio; Finlay, Jonathan L

    2003-07-01

    Diabetes insipidus (DI) is the most common manifestation of central nervous system involvement in Langerhans cell histiocytosis (LCH). Patients with LCH involving the head and neck region are reported to have about a 40% lifetime chance of developing DI. The clinical and biochemical diagnosis of DI is sometimes supported by the absence of the posterior pituitary bright signal on magnetic resonance images. Cladribine (2-chlorodeoxyadenosine, 2-CDA) has been reported as an active drug in children and adults with relapsed or refractory LCH. The authors report the successful reversal of DI in a 3-year-old child with established LCH using 2-CDA.

  11. MR cholangiography in the diagnosis of sclerosing cholangitis in Langerhans' cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Koenig, C.W.; Pfannenberg, C.; Truebenbach, J.; Remy, C.; Claussen, C.D. [Tuebingen Univ. (Germany). Abt. fuer Radiologische Diagnostik; Boehmer, G.M. [Dept. of Gastroenterology, Univ. of Tuebingen (Germany); Ruck, P. [Inst. of Pathology, Univ. of Tuebingen (Germany)

    2001-12-01

    Langerhans' cell histiocytosis (LCH) is a disorder of histiocytic proliferation that primarily affects infants. Imaging findings of a rare case of lung and liver involvement in an adult are presented. High-resolution computed tomography (HRCT) of the lungs showed confluent thin-walled cystic air spaces compatible with advanced LCH. Liver CT and MRI revealed unspecific signs of fatty infiltration. Irregular widening of peripheral bile ducts was displayed in breath-hold MR cholangiography. This pattern is considered characteristic for sclerosing cholangitis and should support the diagnosis of LCH in case of concomitant cystic pulmonary disease, even in adult patients. (orig.)

  12. Monostotic Langerhans' cell histiocytosis in a child with central diabetes insipidus.

    Science.gov (United States)

    Soares, Eduardo Costa Studart; Quidute, Ana Rosa Pinto; Costa, Fábio Wildson Gurgel; Gurgel, Maria Helane Costa; Alves, Ana Paula Negreiros Nunes; Fonteles, Cristiane Sá Roriz

    2012-01-01

    Langerhans'cell histiocytosis (LCH) comprises a rare group of reticuloendothelial system disorders that can produce focal or systemic manifestations. Diabetes insipidus is considered to be an important indicator of serious underlying diseases in children, including LCH. We report the case of a young patient with monostotic LCH confined to the mandibular ramus, who was diagnosed with the disease after presenting symptoms of central diabetes insipidus and was satisfactorily treated with multi-agent chemotherapy. Additionally, we discuss the clinical, radiographic, histological and immunohistochemical findings, as well as the multidisciplinary approach of this important disease, which should receive attention by dental practitioners, especially when it occurs in children.

  13. p53 expression in biopsies from children with Langerhans cell histiocytosis

    DEFF Research Database (Denmark)

    Bank, Micha I; Lundegaard, Pia Rengtved; Carstensen, Henrik;

    2002-01-01

    PURPOSE: Langerhans cell histiocytosis (LCH) is a rare pediatric and adult disease causing skin rashes, osteolytic bone lesions, tumorous growth in various organs, and in some patients, organ dysfunction. The cause of the disease is obscure, and it is not yet understood why some patients develop...... single-system lesions only without relapse, whereas others develop fatal multiorgan disease. The expression of p53 tumor suppressor gene product detected immunohistochemically can be used as a guideline to alterations in DNA repair control and apoptosis. The authors have chosen to analyze p53 expression...

  14. BRAF V600E-Positive Multisite Langerhans Cell Histiocytosis in a Preterm Neonate

    Directory of Open Access Journals (Sweden)

    Sara V. Bates

    2013-10-01

    Full Text Available Hemorrhagic pustules with a “blueberry muffin” appearance accompanied by respiratory failure in a neonate present a challenging differential diagnosis that includes infections and neoplasms. We present a case of multiorgan, multisite Langerhans cell histiocytosis (LCH, positive for the oncogenic BRAF V600E mutation, in a preterm neonate. Infants with LCH pose a diagnostic challenge due to their heterogeneous presentations. This case is unusual in that the newborn presented with severe multiorgan involvement. Due to the rare incidence, wide spectrum of clinical manifestations, and high mortality rate, clinicians must maintain a high index of suspicion for LCH.

  15. Multisystem Langerhans cell histiocytosis coexisting with metastasizing adenocarcinoma of the lung: A case report

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    Lovrenski Aleksandra

    2013-01-01

    Full Text Available Introduction. Langerhans cell histiocytosis (LCH is an uncommon disease of unknown etiology characterized by uncontrolled proliferation and infiltration of various organs by Langerhans cells. Case report. We presented a 54-year-old man, heavy smoker, with dyspnea, cough, hemoptysis, headache and ataxia, who died shortly after admission to our hospital. On the autopsy, tumor was found in the posterior segment of the right upper pulmonary lobe as well as a right-sided occipitoparietal lesion which penetrated into the right ventricle resulting in internal and external hematocephalus. Histologically and immunohistohemically, the diagnosis of primary lung adenocarcinoma with brain metastasis was made (tumor cells showed positivity for CK7 and TTF-1 which confirmed the diagnosis. In the lung parenchyma around the tumor, as well as in brain tissue around the metastatic adenocarcinoma histiocytic lesions were found. Light microscopic examination of the other organs also showed histiocytic lesions involving the pituitary gland, hypothalamus, spleen and mediastinal lymph nodes. Immunohistochemical studies revealed CD68, S-100 and CD1a immunoreactivity within the histiocytes upon which the diagnosis of Langerhans' cells histiocytosis was made. Conclusion. The multisystem form of LCH with extensive organ involvement was an incidental finding, while metastatic lung adenocarcinoma to the brain that led to hematocephalus was the cause of death.

  16. Langerhans Cell Histiocytosis of the Thyroid with Multiple Cervical Lymph Node Involvement Accompanying Metastatic Thyroid Papillary Carcinoma

    Science.gov (United States)

    Ceyran, A. Bahar; Şenol, Serkan; Bayraktar, Barış; Özkanlı, Şeyma; Cinel, Z. Leyla; Aydın, Abdullah

    2014-01-01

    A 37-year-old male case was admitted with goiter. Ultrasonography of thyroid showed a 5 cm cystic nodule in the left lobe with a 1.5 cm solid component. Fine needle aspiration biopsy revealed atypia of undetermined significance or follicular lesion. The patient was operated on. The pathological diagnosis was reported as papillary thyroid carcinoma. The immunohistochemical examination showed multiple foci of Langerhans cell histiocytosis involving both lobes. The patient died due to cardiac arrest with respiratory causes in the early postoperative period. Langerhans cell histiocytosis is a rare primary condition which involves abnormal clonal proliferation of Langerhans cells in various tissues and organs. Thyroid involvement is infrequently seen. Although the etiology is unknown, genetic components may be linked to the disease. It is also associated with a family history of thyroid disease. Papillary thyroid carcinoma is the most common malignant epithelial tumor of the thyroid gland. Langerhans cell histiocytosis presenting with papillary thyroid carcinoma is rare. The privilege of our case is langerhans cell histiocytosis of the thyroid with multiple cervical lymph node involvement accompanying cervical lymph node metastatic thyroid papillary carcinoma. PMID:25349760

  17. Langerhans cell histiocytosis of skin: A clinicopathologic analysis of five cases

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    Punia Rajpal

    2006-01-01

    Full Text Available Background and Aims: Langerhans cell histiocytosis (LCH is a rare proliferative disorder of histiocytes characterized by a proliferation of abnormal and clonal Langerhans cells. We retrospectively studied clinicopathologic features of this disorder in five cases. Methods: Clinical and histopathological findings of five cases of cutaneous LCH were reviewed based on the hospital records. Results: The age of patients ranged from 28 days to 5 years and M: F ratio was 1:1.5. Clinically, the diagnoses suggested were histiocytosis, varicella, transient neonatal pustular melanosis, keloid, sarcoidosis, seborrheic keratosis and LCH. The most common type of skin lesion was a generalized papular lesion. Histologically, all cases showed aggregates of large mononuclear histiocytes (Langerhans cells with reniform, irregular, cleaved nuclei and abundant eosinophilic cytoplasm. There was multi-systemic involvement in two patients and single-system involvement in three patients. Conclusion: Cutaneous lesions may be the sole presenting feature of LCH. Diagnosis is based on demonstration of S-100 positive histiocytes.

  18. Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation

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    B. Routy

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare myeloid neoplasm characterized by the proliferation and dissemination of histiocytes. These in turn may cause symptoms ranging from isolated, infiltrative lesions to severe multisystem disease. Pulmonary Langerhans cell histiocytosis (PLCH presents as a localized polyclonal proliferation of Langerhans cells in the lungs causing bilateral cysts and fibrosis. In adults, this rare condition is considered a reactive process associated with cigarette smoking. Recently, clonal proliferation has been reported with the presence of BRAF V600E oncogenic mutation in a subset of PLCH patients. Spontaneous resolution was described; however, based on case series, smoking cessation remains the most effective way to achieve complete remission and prevent long term complications related to tobacco. Herein, we report the case of an adult woman with biopsy-proven PLCH presenting with thoracic (T8 vertebral bone destruction. Both the lung and the bone diseases regressed following smoking cessation, representing a rare case of synchronous disseminated PCLH with bone localization. This observation underscores the contribution of cigarette smoking as a systemic trigger of both pulmonary and extrapulmonary bone lesions. A review of similar cases in the literature is also presented.

  19. Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation

    Science.gov (United States)

    Routy, B.; Hoang, J.; Gruber, J.

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm characterized by the proliferation and dissemination of histiocytes. These in turn may cause symptoms ranging from isolated, infiltrative lesions to severe multisystem disease. Pulmonary Langerhans cell histiocytosis (PLCH) presents as a localized polyclonal proliferation of Langerhans cells in the lungs causing bilateral cysts and fibrosis. In adults, this rare condition is considered a reactive process associated with cigarette smoking. Recently, clonal proliferation has been reported with the presence of BRAF V600E oncogenic mutation in a subset of PLCH patients. Spontaneous resolution was described; however, based on case series, smoking cessation remains the most effective way to achieve complete remission and prevent long term complications related to tobacco. Herein, we report the case of an adult woman with biopsy-proven PLCH presenting with thoracic (T8) vertebral bone destruction. Both the lung and the bone diseases regressed following smoking cessation, representing a rare case of synchronous disseminated PCLH with bone localization. This observation underscores the contribution of cigarette smoking as a systemic trigger of both pulmonary and extrapulmonary bone lesions. A review of similar cases in the literature is also presented. PMID:25789184

  20. Blood Patch Pleurodesis in the Treatment of Persistant Air Leakage in Langerhans Cell Histiocytosis; A Case Report

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    Ayse Baccioglu

    2013-08-01

    Full Text Available Pulmonary langerhans cell histiocytosis is a rare reactive disorder with unclear pathogenesis. 16-year-old male patient complained about shortness of breath induced with exercise, non-productive cough, and intermittent chest pain for one year. Pulmonary function tests were in restrictive nature. There were multiple air cysts in lung parenchyma smaller than one cm and minimal pneumothorax on the left hemithorax in high resolution computed tomography of thorax. There was no hypoxemia in arterial blood gas analysis, and no pulmonary hypertension in echocardiography. Pulmonary langerhans cell histiocytosis was diagnosed with clinical features and typical radiographic appearance. Existence of multisystem langerhans cell histiocytosis was excluded thorough a detailed history, comprehensive physical examination, and baseline radiographic, blood and urine tests. He was recommended to quit smoking and close follow-up was planned. A tube thoracostomy was performed for left sided total pneumothorax one month later, and %u201Cblood patch%u201D pleurodesis was done due to persistent air leakage with a successful outcome. As far as we know this is the first case report of pulmonary langerhans cell histiocytosis who was treated with autologous blood pleurodesis in the literature.

  1. A rare case of "switch on and off" multi-system Langerhans cell histiocytosis in an adult patient

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    Froudarakis Marios E

    2011-07-01

    Full Text Available Abstract Introduction We report the case of a 24-year-old Greek woman with histologically proven osseous and pulmonary Langerhans cell histiocytosis whose lesions had progressively regressed with a "switch on and off" mode. This is the first report in the literature of this mode of presentation of Langerhans cell histiocytosis. Case presentation The patient had first presented at the age of 20 years with a solitary lesion of the humerus which spontaneously regressed. At that time, no therapy or smoking cessation was indicated. Four years later she presented with bilateral pneumothorax and pulmonary lesions of Langerhans cell histiocytosis. She had pleurodesis for this disease-related complication, and no further systemic treatment was applied, except with regard to smoking cessation. During the follow-up period, her pulmonary lesions regressed without recurrence six years after the initial lung involvement. Conclusion This uncommon case of remission of multi-system Langerhans cell histiocytosis indicates the unpredictable evolution of the disease, raising the question of conservative management in such a patient.

  2. Hepatic involvement of Langerhans cell histiocytosis in children - imaging findings of computed tomography, magnetic resonance imaging and magnetic resonance cholangiopancreatography

    Energy Technology Data Exchange (ETDEWEB)

    Shi, Yingyan; Qiao, Zhongwei; Gong, Ying; Yang, Haowei; Li, Guoping; Pa, Mier [Children' s Hospital of Fudan University, Department of Radiology, Shanghai (China); Xia, Chunmei [Shanghai Medical College of Fudan University, Physiology and Pathophysiology Department, Shanghai (China)

    2014-06-15

    Langerhans cell histiocytosis is a rare disease that occurs mainly in children, and hepatic involvement is generally a poor prognostic factor. To describe CT and MRI findings of hepatic involvement of Langerhans cell histiocytosis in children, especially the abnormal bile duct manifestation on magnetic resonance cholangiopancreatography (MRCP). Thirteen children (seven boys, six girls; mean age 28.9 months) were diagnosed with disseminated Langerhans cell histiocytosis. They underwent CT (n = 5) or MRI (n = 4), or CT and MRI examinations (n = 4) to evaluate the liver involvement. Periportal abnormalities presented as band-like or nodular lesions on CT and MRI in all 13 children. The hepatic parenchymal lesions were found in the peripheral regions of the liver in seven children, including multiple nodules on MRI (n = 6), and cystic-like lesions on CT and MRI (n = 3). In 11 of the 13 children the dilatations of the bile ducts were observed on CT and MRI. Eight of the 13 children underwent MR cholangiopancreatography, which demonstrated stenoses or segmental stenoses with slight dilatation of the central bile ducts, including the common hepatic duct and its first-order branches. The peripheral bile ducts in these children showed segmental dilatations and stenoses. Stenosis of the central bile ducts revealed by MR cholangiopancreatography was the most significant finding of liver involvement in Langerhans cell histiocytosis in children. (orig.)

  3. Langerhans cell histiocytosis: unusual skeletal manifestations observed in thirty-four cases

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    Hindman, B.W.; Thomas, R.D.; Young, Lionel W. [Loma Linda University Medical Center, 11234 Anderson Street, Loma Linda, CA 92354 (United States); Yu, Leisure [Department of Orthopedic Surgery, Loma Linda University Medical Center, Loma Linda, CA 92354 (United States)

    1998-04-01

    Objective. Unusual manifestations are occasionally encountered in Langerhans cell histiocytosis and may be a source of confusion. The objective of this study was to determine the frequency of occurrence of the unusual manifestations in our case material. Design and patients. Thirty-four children, average age 4.4 years (range 3 months to 16 years) with 262 skeletal lesions of biopsy-proven Langerhans cell histiocytosis (LCH) were retrospectively reviewed to determine the frequency of occurrence of unusual manifestations defined either as an atypical location of a skeletal lesion or an atypical radiographic appearance of the lesion. Results. Twenty-four unusual lesions were found in this retrospective review. Among these were epiphyseal lesions, transphyseal lesions, extracranial `button` sequestra, posterior vertebral arch lesions, dural extension of vertebral lesions, and fluid-fluid levels. The finding of fluid-fluid levels has not previously been described in the radiologic literature. Involvement of unusual sites included clavicles and small bones of the hands and feet. Conclusions. Radiographic, computed tomographic, and magnetic resonance imaging of LCH yield a variety of unusual manifestations. Recognition of these varied appearances of LCH may prevent confusion of such appearances with other pathologic processes. When the unusual manifestation occurs as the initial finding of the disease, LCH should be included in the differential diagnosis. (orig.) With 7 figs., 10 refs.

  4. High-resolution computed tomography findings in pulmonary Langerhans cell histiocytosis

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    Rodrigues, Rosana Souza [Universidade Federal do Rio de Janeiro (HUCFF/UFRJ), RJ (Brazil). Hospital Universitario Clementino Fraga Filho. Unit of Radiology; Capone, Domenico; Ferreira Neto, Armando Leao [Universidade do Estado do Rio de Janeiro (UERJ), Rio de Janeiro, RJ (Brazil)

    2011-07-15

    Objective: The present study was aimed at characterizing main lung changes observed in pulmonary Langerhans cell histiocytosis by means of high-resolution computed tomography. Materials and Methods: High-resolution computed tomography findings in eight patients with proven disease diagnosed by open lung biopsy, immunohistochemistry studies and/or extrapulmonary manifestations were retrospectively evaluated. Results: Small rounded, thin-walled cystic lesions were observed in the lung of all the patients. Nodules with predominantly peripheral distribution over the lung parenchyma were observed in 75% of the patients. The lesions were diffusely distributed, predominantly in the upper and middle lung fields in all of the cases, but involvement of costophrenic angles was observed in 25% of the patients. Conclusion: Comparative analysis of high-resolution computed tomography and chest radiography findings demonstrated that thinwalled cysts and small nodules cannot be satisfactorily evaluated by conventional radiography. Because of its capacity to detect and characterize lung cysts and nodules, high-resolution computed tomography increases the probability of diagnosing pulmonary Langerhans cell histiocytosis. (author)

  5. Isolated thymic Langerhans cell histiocytosis discovered on F-18 fluorodeoxyglucose positron emission tomography/computed tomography (F-18 FDG PET/CT)

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    Turpin, Sophie [CHU Sainte-Justine, Nuclear Medicine, Montreal (Canada); Carret, Anne-Sophie [CHU Sainte-Justine, Hemato-Oncology, Montreal (Canada); Dubois, Josee [CHU Sainte-Justine, Radiology, Montreal (Canada); Buteau, Chantal [CHU Sainte-Justine, Infectious Diseases, Montreal (Canada); Patey, Natalie [CHU Sainte-Justine, Pathology, Montreal (Canada)

    2015-11-15

    The thymic infiltration in young patients with multisystemic Langerhans cell histiocytosis and its radiologic features are well known. However, isolated thymic disease has seldom been reported in the literature. We report the case of a 10-month-old child admitted for fever of unknown origin. Whole-body F-18 fluorodeoxyglucose positron emission tomography/computed tomography (F-18 FDG PET/CT) was performed to identify a focus of infection. It demonstrated an unusual aspect of the thymus, which led to further investigation and revealed isolated infiltration of the thymus by Langerhans cell histiocytosis. The patient was treated accordingly and is now disease free. As evaluation of Langerhans cell histiocytosis patients with F-18 FDG PET/CT is becoming more frequent, it is important to be aware of the scintigraphical characteristics of thymic Langerhans cell histiocytosis. (orig.)

  6. Langerhans cell histiocytosis: A case report of a three year old boy with primary oral manifestations

    Directory of Open Access Journals (Sweden)

    Seraj B

    2006-06-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disease with formation of granulomatous infiltrations consisting of Langerhans cells, histiocytes, lymphocytes and eosinophilic granulocytes. The ethiopathogenesis of the disease has not been fully clarified yet. It can occur as focal or disseminated form - acute or chronic. Oral manifestations may be the first signs. This article reports a case of a 3 year old boy with LCH suffering from severe dental mobility and foul breathe. Radiographically, the lesions appeared as well-defined radiolucent defects in maxilla and mandible. The microscopic findings consisted of sheet like arrangements of histiocytes with a mixture of eosinophils and other inflammatory cells. Biochemical tests were within normal limits.

  7. A case of coexisting Warthin tumor and langerhans cell histiocytosis associated with necrosis, eosinophilic abscesses and a granulomatous reaction in intraparotid lymph nodes

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    Fredrik Petersson

    2011-04-01

    Full Text Available We present a patient (50-year-old male with coexisting Warthin tumor and involvement of two intraparotid lymph nodes by Langerhans cell histiocytosis associated with necrosis, eosinophilic abscesses and a granulomatous reaction. This is the second documented case of this unusual combination of histological changes in nodal Langerhans cell histiocytosis and the first case involving intraparotid lymph nodes occurring together with an ipsilateral Warthin tumor.

  8. Haematopoietic Stem Cell Transplantation for Refractory Langerhans Cell Histiocytosis: Outcome by Intensity of Conditioning

    Science.gov (United States)

    Veys, Paul A.; Nanduri, Vasanta; Baker, K. Scott; He, Wensheng; Bandini, Giuseppe; Biondi, Andrea; Dalissier, Arnaud; Davis, Jeffrey H.; Eames, Gretchen M.; Egeler, R. Maarten; Filipovich, Alexandra H.; Fischer, Alain; Jürgens, Herbert; Krance, Robert; Lanino, Edoardo; Leung, Wing H.; Matthes, Susanne; Michel, Gérard; Orchard, Paul J.; Pieczonka, Anna; Ringdén, Olle; Schlegel, Paul G.; Sirvent, Anne; Vettenranta, Kim; Eapen, Mary

    2015-01-01

    Summary Patients with Langerhans cell histiocytosis (LCH) refractory to conventional chemotherapy have a poor outcome. There are currently two promising treatment strategies for high-risk patients: the first involves the combination of 2-chlorodeoxyadenosine and cytarbine; the other approach is allogeneic haematopoietic stem cell transplantation (HSCT). Here we evaluated 87 patients with high-risk LCH who were transplanted between 1990–2013. Prior to the year 2000, most patients underwent HSCT following myeloablative conditioning (MAC): only 5 of 20 patients (25%) survived with a high rate (55%) of transplant-related mortality (TRM). After the year 2000 an increasing number of patients underwent HSCT with reduced intensity conditioning (RIC): 49/67 (73%) patients survived, however, the improved survival was not overtly achieved by the introduction of RIC regimens with similar 3-year probability of survival after MAC (77%) and RIC transplantation (71%). There was no significant difference in TRM by conditioning regimen intensity but relapse rates were higher after RIC compared to MAC regimens (28% vs. 8%, p=0.02), although most patients relapsing after RIC transplantation could be salvaged with further chemotherapy. HSCT may be a curative approach in 3 out of 4 patients with high risk LCH refractory to chemotherapy: the optimal choice of HSCT conditioning remains uncertain. PMID:25817915

  9. FNAC of Bacillus- Calmette- Guerin lymphadenitis masquerading as Langerhans cell histiocytosis.

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    Gupta Nalini

    2004-12-01

    Full Text Available Abstract Bacillus Calmette Guerin (BCG lymphadenitis is a well known entity. Disseminated BCG infection usually presents as generalized lymphadenopathy, skin rash and hepatosplenomegaly and at times, can pose a diagnostic challenge to clinicians. There are only a few published studies on the cytological findings of BCG lymphadenitis. In this letter we report the fine needle aspiration cytology (FNAC of BCG lymphadenitis clinically masquerading as Langerhans cell histiocytosis (LCH. FNA smears showed sheets of foamy macrophages and many polymorphs in a dirty necrotic background with many macrophages as well as polymorphs showing negatively stained rod like structures within their cytoplasm. Zeihl Neelson stain revealed that these cells were heavily loaded with acid fast bacilli (AFB. In the index case, AFB were also seen within the cytoplasm of polymorphs, which has not been documented earlier in the literature.

  10. Langerhans cell histiocytosis with multisystem involvement in an infant: A case report

    Science.gov (United States)

    BI, LINTAO; SUN, BUTONG; LU, ZHENXIA; SHI, ZHANGZHEN; WANG, DAN; ZHU, ZHENXING

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocyte-like cells, with a wide range of clinical presentations that vary from a solitary lesion to more severe multifocal or disseminated lesions. The disease can affect any age group; however, the peak incidence rate is in infants aged between 1 and 3 years-old. Diagnosis of LCH should be based on the synthetical analysis of clinical presentations, in addition to features of imaging and histopathology. Although certain cases regress spontaneously, other patients require systemic chemotherapy together with the administration of steroids. The present study reports the case of an infant with LDH with multisystem involvement, including that of the bone, skin, orbit, spleen and lungs. The patient received chemotherapy and obtained rapid improvement in the involved systems. A total of 2.5 years after completion of the therapy, the patient still remains in follow-up and no evidence of active disease has been noted. PMID:26136948

  11. Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy

    Science.gov (United States)

    May, Adam; Kane, Garvan; Yi, Eunhee; Frantz, Robert; Vassallo, Robert

    2014-01-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments. Although pulmonary hypertension (PH) is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established. We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement over a 10 year follow-up period. This case indicates that PLCH-associated PH may, in certain instances, be highly responsive to advanced PH therapies and emphasizes the importance of trialing these therapies among patients with PLCH-related PH. PMID:26029568

  12. Case of Langerhans Cell Histiocytosis That Mimics Meningioma in CT and MRI

    Science.gov (United States)

    Zhu, Ming; Yu, Bing-Bing; Zhai, Ji-Liang

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder histologically characterized by the proliferation of Langerhans cells. Here we present the case of a 13-year-old girl with LCH wherein CT and MRI results led us to an initially incorrect diagnosis of meningioma. The diagnosis was corrected to LCH based on pathology findings. An intracranial mass was found mainly in the dura mater, with thickening of the surrounding dura. It appeared to be growing downward from the calvaria, pressing on underlying brain tissue, and had infiltrated the inner skull, causing a bone defect. The lesion was calcified with the typical dural tail sign. The dural origin of the lesion was verified upon surgical dissection. There are no previous reports in the literature describing LCH of dural origin presenting in young patients with typical dural tail signs and meningioma-like imaging findings. The current case report underscores the need for thorough histological and immunocytochemical examinations in LCH differential diagnosis. PMID:26962425

  13. A Fatal Case of Congenital Langerhans Cell Histiocytosis with Disseminated Cutaneous Lesions in a Premature Neonate

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    Michio Inoue

    2016-01-01

    Full Text Available Background. The outcome of neonates with congenital cutaneous Langerhans cell histiocytosis (LCH is variable. Observations. We report a case of LCH in a female premature neonate born at 33-week gestation. She had disseminated cutaneous lesions, which consisted of hemorrhagic papules and vesicles, with sparse healthy skin areas, and the hands and feet were contracted with scarring and blackened. She was in respiratory failure although no apparent pulmonary or bone lesions on X-rays were noted. Skin biopsy confirmed a diagnosis of LCH due to observation of CD1a+ Langerhans cells, which lacked expression of E-cadherin and CD56. The patient died 57 hours after birth. Conclusions. Based on this case and the literature survey, the outcome of premature babies with congenital cutaneous LCH lesions is noted to be unfavorable, with the majority of such cases suffering from multisystem disease.

  14. Solitary Langerhans cell histiocytosis of the hard palate: a diagnostic pitfall

    Science.gov (United States)

    Varsha, Dalal; Kaur, Manveen; Chaudhary, Neena; Siraj, Fouzia

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a relatively rare and unique disease characterized by an abnormal proliferation of immature dendritic cells. It is predominantly seen in children with adults showing less than ten times the incidence compared to childhood. The clinical presentation and organ involvement is highly variable. Oral manifestations generally consist of mucosal ulceration associated with lesions of the underlying bone. Lesions limited to the oral mucosa are rare. We present a case of a 45-year-old male who presented with an ulcer on the hard palate showing histopathologic features of LCH. The present case is a reminder of the possibility of occurrence of this unusual entity in the oral cavity. Appropriate use of immunohistochemistry is advocated to avoid diagnostic pitfalls. PMID:27703428

  15. Solitary Langerhans cell histiocytosis of the hard palate: a diagnostic pitfall

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    Varsha, Dalal

    2016-09-01

    Full Text Available Langerhans cell histiocytosis (LCH is a relatively rare and unique disease characterized by an abnormal proliferation of immature dendritic cells. It is predominantly seen in children with adults showing less than ten times the incidence compared to childhood. The clinical presentation and organ involvement is highly variable. Oral manifestations generally consist of mucosal ulceration associated with lesions of the underlying bone. Lesions limited to the oral mucosa are rare. We present a case of a 45-year-old male who presented with an ulcer on the hard palate showing histopathologic features of LCH. The present case is a reminder of the possibility of occurrence of this unusual entity in the oral cavity. Appropriate use of immunohistochemistry is advocated to avoid diagnostic pitfalls.

  16. Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker

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    K. Earlam

    2016-01-01

    Full Text Available Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT of the thorax findings includes centrilobular nodules and cysts that are bizarre in shape, variable in size, and thin-walled. Often the diagnosis can be made based on the appropriate clinical presentation and typical imaging findings. Treatment includes smoking cessation and the potential use of glucocorticoids or cytotoxic agents depending on the severity of disease and multisystem involvement.

  17. Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker

    Science.gov (United States)

    Earlam, K.; Souza, C. A.; Glikstein, R.; Gomes, M. M.; Pakhalé, S.

    2016-01-01

    Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT) of the thorax findings includes centrilobular nodules and cysts that are bizarre in shape, variable in size, and thin-walled. Often the diagnosis can be made based on the appropriate clinical presentation and typical imaging findings. Treatment includes smoking cessation and the potential use of glucocorticoids or cytotoxic agents depending on the severity of disease and multisystem involvement. PMID:27445532

  18. PULMONARY LANGERHANS CELL HISTIOCYTOSIS PRESENTING AS SIMULTANEOUS BILATERAL SPONTANEOUS PNEUMOTHORAX IN A NON-SMOKER PATIENT

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    M. Vaziri

    2008-06-01

    Full Text Available Pulmonary Langerhans Cell Histiocytosis (PLCH is a rare idiopathic disorder that primarily affects young adult cigarette smokers. Affected patients often present with cough and dyspnea and about 20% of patients present with or later develop pneumothorax. It is striking that more than 90% of patients are smokers. We report a very unusual case of PLCH in a 20-year- old male patient with no smoking history in whom a life- threatening complication such as simultaneous bilateral pneumothorax was the presenting feature. The final diagnosis was made by open surgical biopsy and recurrent pneumothoraces necessitated surgical management with pleurodesis. We emphasize the early use of pleurodesis in managing patients with PLCH and spontaneous pneumothorax.

  19. A Rare Case of Erdheim-Chester Disease and Langerhans Cell Histiocytosis Overlap Syndrome

    Science.gov (United States)

    Nabi, Shahzaib; Arshad, Adeel; Jain, Tarun; Virk, Fawad; Gulati, Rohit; Awdish, Rana

    2015-01-01

    A 48-year-old woman with a past medical history of seizures and end-stage renal disease secondary to obstructive uropathy from retroperitoneal fibrosis presented to the emergency department with seizures and altered mental status. A Glasgow Coma Scale of 4 prompted intubation, and she was subsequently admitted to the intensive care unit. Magnetic resonance imaging of the brain performed to elucidate the aetiology of her seizure showed a dural-based mass within the left temporoparietal lobe as well as mass lesions within the orbits. Further imaging showed extensive retroperitoneal fibrosis extending to the mediastinum with involvement of aorta and posterior pleural space. Imaging of the long bones showed bilateral sclerosis and cortical thickening of the diaphyses. Imaging of the maxillofacial structures showed osseous destructive lesions involving the mandible. These clinical and radiological features were consistent with a diagnosis of Erdheim-Chester disease; however, the patient's skin biopsy was consistent with Langerhans cell histiocytosis. PMID:26579323

  20. Localized Langerhans` cell histiocytosis of bone: treatment and follow-up in children

    Energy Technology Data Exchange (ETDEWEB)

    Libicher, M. [Dept. of Diagnostic Radiology, Univ. Heidelberg (Germany); Roeren, T. [Dept. of Diagnostic Radiology, Univ. Heidelberg (Germany); Troeger, J. [Dept. of Pediatric Radiology, Univ. Heidelberg (Germany)

    1995-11-01

    We report a case of Langerhans` cell histiocytosis (LCH) involving the right scapula in a 4-year-old child. Because of progressive shoulder pain and immobility methylprednisolone was injected directly into the lesion under computed tomography (CT) guidance. Follow-up studies with magnetic resonance imaging (MRI) and ultrasound (US) revealed continuous ossification of the osteolytic lesion with healing after 6 months. Plain films and CT confirmed the healing process. We consider intralesional corticoid instillation a safe method when performed under CT guidance. For initial evaluation and follow-up, MRI and US yielded reliable results in comparison to plain films and CT, thus helping to reduce the radiation dosage in children. (orig.)

  1. Pulmonary Langerhans cell histiocytosis in children: A spectrum of radiologic findings

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    Bano, Shahina, E-mail: dr_shahinaindia@yahoo.com [Department of Radiodiagnosis, Lady Hardinge Medical College and Associated Hospitals, New Delhi 110001 (India); Chaudhary, Vikas, E-mail: dr_vikaschaudhary@yahoo.com [Department of Radiodiagnosis, Employees’ State Insurance Corporation (ESIC) Model Hospital, Gurgaon 122001, Haryana (India); Narula, Mahender Kaur, E-mail: narulamk@gmail.com [Department of Radiodiagnosis, Lady Hardinge Medical College and Associated Hospitals, New Delhi 110001 (India); Anand, Rama, E-mail: rama_home@yahoo.co.in [Department of Radiodiagnosis, Lady Hardinge Medical College and Associated Hospitals, New Delhi 110001 (India); Venkatesan, Bhuvaneswari, E-mail: venkatesanbhuvana@yahoo.com [Department of Radiodiagnosis, Lady Hardinge Medical College and Associated Hospitals, New Delhi 110001 (India); Mandal, Shramana, E-mail: shramana@hotmail.co.in [Department of Pathology, Govind Ballabh Pant Hospital, New Delhi 110002 (India); Majumdar, Kaushik, E-mail: drkaushik.m@gmail.com [Department of Pathology, Govind Ballabh Pant Hospital, New Delhi 110002 (India)

    2014-01-15

    Pulmonary Langerhans cell histiocytosis (PLCH) is a well known entity in adults but is exceedingly rare in children. It is better described in adults than in children. We describe the current understanding of PLCH in children and a spectrum of radiological findings of PLCH in the paediatric population. On high resolution computed tomography (HRCT), PLCH may have variable appearance depending on the stage of disease, ranging from small interstitial nodular opacities to multiple thin/thick walled cysts (often bizarre in shape), eventually leading to marked parenchymal fibrosis and honeycomb pattern. CT finding of PLCH is similar in adult and paediatric populations with the exception that lung base near the costophrenic angle is spared in adults but almost always involved in children.

  2. A Rare Case of Erdheim-Chester Disease and Langerhans Cell Histiocytosis Overlap Syndrome

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    Shahzaib Nabi

    2015-01-01

    Full Text Available A 48-year-old woman with a past medical history of seizures and end-stage renal disease secondary to obstructive uropathy from retroperitoneal fibrosis presented to the emergency department with seizures and altered mental status. A Glasgow Coma Scale of 4 prompted intubation, and she was subsequently admitted to the intensive care unit. Magnetic resonance imaging of the brain performed to elucidate the aetiology of her seizure showed a dural-based mass within the left temporoparietal lobe as well as mass lesions within the orbits. Further imaging showed extensive retroperitoneal fibrosis extending to the mediastinum with involvement of aorta and posterior pleural space. Imaging of the long bones showed bilateral sclerosis and cortical thickening of the diaphyses. Imaging of the maxillofacial structures showed osseous destructive lesions involving the mandible. These clinical and radiological features were consistent with a diagnosis of Erdheim-Chester disease; however, the patient’s skin biopsy was consistent with Langerhans cell histiocytosis.

  3. Results of Treatment with 2-Chlorodeoxyadenosine (2-CDA in Multiple Reactivations or Refractory Langerhans Cell Histiocytosis

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    Ghasem Miri-Aliabad

    2014-01-01

    Full Text Available Background: Langerhans cell histiocytosis (LCH is the most common type of histiocytosis and characterized by abnormal proliferation and excess accumulation of inflammatory and langerhans cells at various tissue sites. Clinical manifestations are variable, ranging from spontaneously regressing single bone lesion to multisystem disease, life-threatening and refractory to treatment. Conventional chemotherapy has been shown to be effective in treatment of majority of patients with LCH. However, treatment of refractory disease or multiple reactivations is difficult. The aim of this study is to assess the efficacy of 2-CDA in relapsed or refractory LCH. Materials and methods: Four patients with relapsed or refractory LCH that were treated with 2-chlorodeoxyadenosin (2-CDA enrolled in this study. All patients had received at least one prior chemotherapy regimen. The dose and schedule of 2-CDA was 6 mg/m²/day for 5 days every 3-4 weeks. Results: Median age at the time of treatment with 2-CDA was 9.7 years. Three patients had multisystem disease and one had multifocal bone lesions. All patients had multifocal bone lesions. None of them had risk organ involvement. Mean course of treatment with 2-CDA was 9.5. Radiologic evaluations revealed complete resolution of bone lesions in two (50% patients. In one (25% patient lesions regressed (partial response and in another (25% the disease remained stable. Drug related side effects were minimal. At the present time all patients are alive. Conclusion: Our study demonstrates that 2-CDA as a single agent is efficacious in treatment of multiple reactivations or refractory LCH and well-tolerated in children.

  4. Physiologic Determinants of Exercise Capacity in Pulmonary Langerhans Cell Histiocytosis: A Multidimensional Analysis

    Science.gov (United States)

    Fry, Stephanie; Giovannelli, Jonathan; Langlois, Carole; Bricout, Nicolas; Aguilaniu, Bernard; Bellocq, Agnes; Le Rouzic, Olivier; Dominique, Stephane; Delobbe, Alain; François, Geraldine; Tazi, Abdellatif; Wallaert, Benoit; Chenivesse, Cecile

    2017-01-01

    Background Reduced exercise capacity severely impacts quality of life in pulmonary Langerhans cell histiocytosis. Ascertaining mechanisms that impair exercise capacity is necessary to identify targets for symptomatic treatments. Methods Dyspnea, pulmonary function tests and cardiopulmonary exercise test were analysed in 62 study participants. Data were compared between subjects with impaired and normal aerobic capacity (V’O2 peak less than 84% versus 84% predicted or more). Data were reduced using a principal component analysis. Multivariate analysis included V’O2 peak as the dependent variable and principal components as covariates. Results V’O2 peak was reduced in 44 subjects (71%). Subjects with impaired aerobic capacity presented: (i) decreased FEV1, FVC, FEV1/FVC, DLCO and DLCO/VA and increased AaDO2, (ii) increased ventilatory equivalents at ventilatory threshold, VD/VT peak, AaDO2 peak and PaCO2 peak and decreased ventilatory reserve and PaO2 peak. There was no difference between groups in dyspnea scores. Principal component analysis extracted 4 principal components interpreted as follows: PC1: gas exchange; PC2: “pseudorestriction”; PC3: exercise-induced hyperpnea; PC4: air trapping. Multivariate analysis explained 65% of V’O2 peak. The 4 principal components were independently associated with V’O2 peak (βcoefficients: PC1: 9.3 [4.6; 14], PC2: 7.5 [3; 11.9], PC3: -5.3 [-9.6;-1.], PC4: -9.8 [-14,9;-4.7]). Conclusion Impaired exercise capacity is frequent in pulmonary Langerhans cell histiocytosis. It is mainly caused by pulmonary changes but is not associated with increased dyspnea intensity. Therefore, treating the lung represents a relevant approach for improving exercise capacity, even in patients experiencing mild dyspnea. PMID:28072848

  5. Severe Periodontal Disease Manifested in Chronic Disseminated Type of Langerhans Cell Histiocytosis in a 3-Year Old Child

    Science.gov (United States)

    Srivastava, Vinay Kumar; Bansal, Rajesh; Gupta, Vineeta; Bansal, Manish; Patne, Shashikant

    2014-01-01

    ABSTRACT% Langerhans cell histiocytosis (LCH), previously known as histio-cytosis X, is a rare idiopathic disorder of reticulo-endothelial system with abnormal proliferation of bone marrow derived Langerhans cells along with a variable number of leukocytes, such as eosinophils, neutrophils, lymphocytes and plasma cells. Three years old male child presented with multifocal osteolytic lesions and papulosquamous skin lesions. Clinical and radio-graphic features, such as severe alveolar bone loss, mobility of teeth, precocious eruption of teeth, foating appearance of teeth in orthopantomogram (OPG), osteolytic lesion in skull and cutaneous lesions were highly suggestive of LCH disease. Skin biopsy confirmed a diagnosis of LCH. Induction chemotherapy with oral prednisolone and intravenous vinblastine was started. Child responded well to chemotherapy. The clinical significance of the presented case is to diagnose the case of LCH on the basis of the manifestation of severe periodontal disease as this can be first or only manifestation of LCH. A dentist plays a major role in the multidisciplinary treatment of LCH through routine examination and periodic follow-up. How to cite this article: Bansal M, Srivastava VK, Bansal R, Gupta V, Bansal M, Patne S. Severe Periodontal Disease Manifested in Chronic Disseminated Type of Langerhans Cell Histiocytosis in a 3-Year Old Child. Int J Clin Pediatr Dent 2014;7(3):217-219. PMID:25709306

  6. Langerhans Cell Histiocytosis in Childhood: Review, Symptoms in the Oral Cavity, Differential Diagnosis and Report of One Case

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    Mohammad Shooriabi

    2016-08-01

    Full Text Available Background Langerhans cell histiocytosis (LCH is a rare disease in which monoclonal migration and proliferation of specific dendritic cells is seen. The disease primarily affects the bones and skin, but there is a possibility that involves other organs or appears as a multi-systemic disease. Case Report In oral examination of a nine-month girl, two deep wounds with a yellow membrane with approximate size of 1 × 1 cm on both sides of mandibular alveolar ridge were seen. The edges of the wounds were swollen and proliferated and redder than the surrounding mucosa. At the touch the edges of the wound were not indurated. The wound were created from the third-month and the size of wounds had become slightly larger within 6 months. According to the chronic wound and being non-responsive to various systemic and local treatments, incisional biopsy was taken from the wounds. Langerhans cell histiocytosis was confirmedhistologically and immune histochemically. Conclusion Mouth ulcers may be the only symptoms of Langerhans cell histiocytosis. Therefore, the role of dentist could be important in diagnosis of this disease.

  7. Diagnosis of Langerhans Cell Histiocytosis on Fine Needle Aspiration Cytology: A Case Report and Review of the Cytology Literature

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    Neeta Kumar

    2011-01-01

    Full Text Available A case of multifocal Langerhans cell histiocytosis in a two-year-old child is presented where fine needle aspiration was helpful in achieving a rapid and accurate diagnosis in an appropriate clinical and radiological setting. This can avoid unnecessary biopsy and guide the management especially where access to histopathology is limited. The highly characteristic common and rare cytological features are highlighted with focus on differential diagnoses and causes of pitfalls.

  8. Diagnosis and Intralesional Corticotherapy in Oral Ulcers Occurring as the Sole Manifestation of Langerhans Cell Histiocytosis. A Case Report

    Science.gov (United States)

    Gambirazi, Liane; Libório, Tatiana; Nunes, Fábio; Sugaya, Norberto; Migliari, Dante

    2016-01-01

    This article reports a case of oral mucosa lesions as the sole manifestation in Langerhans cell histiocytosis (LCH). This is a very uncommon manifestation of LCH since this disease preferably affects the bones with frequent involvement of the jaws. LCH may also involve other organs, particularly the lungs, liver, lymph nodes, and skin. The highlights of this report are the differential diagnosis, immunohistochemical analysis and, mostly, the therapeutic approach. PMID:27398106

  9. Langerhans Cell Histiocytosis of the Clavicle in an Adult: A Case Report and Review of the Literature

    Science.gov (United States)

    Udaka, Toru; Susa, Michiro; Kikuta, Kazutaka; Nishimoto, Kazumasa; Horiuchi, Keisuke; Sasaki, Aya; Kameyama, Kaori; Nakamura, Masaya; Matsumoto, Morio; Chiba, Kazuhiro; Morioka, Hideo

    2015-01-01

    Langerhans cell histiocytosis (LCH) usually occurs in children under the age of 10 years with a predilection for the skull, spine, rib and humerus. Solitary LCH occurring in an adult clavicle is uncommon with limited reports to date. The lesion in our patient was curetted with the intent to make a diagnosis, which subsequently lead to the remission of the symptom and the disease. At the final follow-up after 1 year, no local recurrence or metastasis is observed. PMID:26600774

  10. Unifocal Granuloma of Femur due to Langerhans' Cell Histiocytosis: A Case Report and Review of the Literature

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    Harpreet Singh

    2010-01-01

    Full Text Available The radiological diagnosis of osteolytic lesions of the long bones in pediatric population constitutes a challenge when the case history and clinical data are uncharacteristic. We believe that the description of few clinically and histologically proven cases to verify the existence of radiological signs useful for diagnosis may be of interest. Here, we describe a case of Langerhans' cell histiocytosis (LCH presenting as unifocal eosinophilic granuloma of femur along with a brief review of the literature.

  11. Primary Langerhans cell histiocytosis (LCH in the adult cervical spine: A case report and review of the literature

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    Sang-Deok Kim, M.D.

    2017-03-01

    Full Text Available Langerhans cell histiocytosis (LCH of the spine is a common benign disease in children and adolescents that rarely affects adults. Main management of single lesion (unifocal vertebral LCH is conservative method, unless there is neurological deficit due to mass effect, surgery must be considered. This is an interesting and rare case report of the patient with LCH at C5 vertebral body who underwent fusion surgery.

  12. Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report

    Science.gov (United States)

    Choi, Yeun Seoung; Lim, Jung Soo; Kwon, Woocheol; Jung, Soon-Hee; Park, Il Hwan; Lee, Myoung Kyu; Lee, Won Yeon; Yong, Suk Joong; Lee, Seok Jeong; Jung, Ye-Ryung; Choi, Jiwon; Choi, Ji Sun; Jeong, Joon Taek; Yoo, Jin Sae

    2015-01-01

    Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus. PMID:26508947

  13. A rare case of langerhans cell histiocytosis of the gastrointestinal tract

    Institute of Scientific and Technical Information of China (English)

    Uday Shankar; Monika Prasad; Om P Chaurasia

    2012-01-01

    Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized,bone marrow-derived langerhans cells and mature eosinophils.The clinical spectrum ranges from an acute,fulminant,disseminated disease called LettererSiwe disease to solitary or few,indolent and chronic lesions of the bone or other organs called eosinophilic granuloma.Involvement of the gastrointestinal tract is very rare in LCH.We present the case of a 53-year-old woman referred by her primary care physician for a screening colonoscopy.A single sessile polyp,measuring 4 mm in size,was found in the rectum.Histopathological examination revealed that the lesion was relatively well circumscribed and comprised mainly a mixture of polygonal cells with moderate-to-abundant pink slightly granular cytoplasm.The nuclei within these cells had frequent grooves and were occasionally folded.Immunohistochemical staining was positive for CD-1a which confirmed the diagnosis of LCH.On further workup,there was no evidence of involvement of any other organ.On follow up colonoscopy one year later,there was no evidence of disease recurrence.Review of the published literature revealed that LCH presenting as solitary colonic polyp is rare.However,with the increasing rates of screening colonoscopy,more colonic polyps may be identified as LCH on histopathology.This underscores the importance of recognizing this rare condition and ensuring proper follow-up to rule out systemic disease.

  14. Langerhans cell histiocytosis in children diagnosed by fine-needle aspiration

    Science.gov (United States)

    Handa, Uma; Kundu, Reetu; Punia, Rajpal Singh; Mohan, Harsh

    2015-01-01

    Background: Langerhans cell histiocytosis (LCH) is a rare intricate pediatric neoplasm with varied clinical manifestations and multiple treatment modalities. Aim: To study the cytological features of LCH and the differential diagnoses on fine-needle aspiration (FNA). Materials and Methods: FNA was performed using a 23-gauge needle fitted to a 10 mL syringe mounted on syringe holder. LCH was diagnosed on FNA smears in seven cases confined to the head and neck region, which included three cases of lymphadenopathy, three cases of scalp swelling, and one case of orbital swelling. Results: The age of the patients ranged from 25 days to 11 years and male-to-female ratio was 1:1.3. Clinically, the diagnoses suggested were tuberculosis, inflammatory lesion, abscess, and malignancy. The cytologic findings included high cellularity, isolated Langerhans cells (LCs) with prominent nuclear indentation, grooves and abundant vacuolated cytoplasm, multinucleated giant cells, eosinophils, and lymphocytes. Areas of necrosis were noted in one case. Histopathology, along with positive S-100 immunohistochemistry, confirmed the diagnosis of LCH. Conclusions: LCH is a rare disease occurring predominantly in children and can be diagnosed with ease on FNA cytology by the presence of characteristic Langerhans cells. The S-100 positivity aids in suggesting a diagnosis of LCH. PMID:26811572

  15. Hypopituitarism and goitre as endocrine manifestation of Langerhans cell histiocytosis (LCH). Case Report.

    Science.gov (United States)

    Skowronska-Jozwiak, Elzbieta; Sporny, Stanislaw; Szymanska-Duda, Joanna; Baranska, Dobromila; Lewinski, Andrzej

    2016-07-01

    Langerhans cell histiocytosis (LCH) in adults is a rare disorder of unknown etiology characterized by monoclonal proliferation of Langerhans cells. It belongs to dendritic cell disorders and occurs in 1-2 adults per million. The most common endocrine manifestation of classical LCH is associated with the posterior pituitary, with clinical symptoms of diabetes insipidus. Less than 80 reported cases of LCH involving the thyroid gland have been published so far. We present the case of a 39 years old woman with 10 years history of diabetes insipidus and secondary amenorrhoea, which appeared after second delivery. She was suspected for lymphocytic inflammation of pituitary and she was administered steroid treatment. She was also treated symptomatically with desmopressin, L-thyroxine, estrogen and progestagen replacement therapy due to diabetes insipidus, secondary hypothyroidism and hypogonadotropic hypogonadism. In September 2014, she noticed a painless, firm tumour of the neck. Ultrasound (US) examination demonstrated bilateral, solid, hypoechogenic thyroid nodules. The result of fine-needle aspiration biopsy (FNAB) was not diagnostic. Due to rapid progression and US image of the tumour, she was referred for surgery. In postoperative histopathology tumour cells were positive for CD1a and S-100 protein, therefore diagnosis of LCH was established. Postoperatively, the results of thoracic computed tomography scan, abdominal US and bone scintigraphy revealed no evidence of multifocal disease. We have not observed any disease recurrence in the patient after a year of follow-up in postoperative course. This case illustrates diagnostic and therapeutic difficulties in patient with LCH.

  16. A Case of Langerhans Cell Histiocytosis Manifested as a Suprasellar Mass

    Science.gov (United States)

    Yoon, Ju Young; Park, Byung-Kiu; Yoo, Heon; Lee, Sang Hyun; Hong, Eun Kyung; Park, Weon Seo; Kwon, Young Joo; Yoon, Jong Hyung

    2016-01-01

    Langerhans cell histiocytosis (LCH) has diverse clinical manifestations, including intracranial mass lesions. We report a case of LCH that manifested as a suprasellar mass, and initially misdiagnosed as a germ cell tumor. A 29-year-old woman presented with polyuria, polydipsia and amenorrhea. Laboratory findings revealed hypopituitarism with central diabetes insipidus, and a suprasellar mass and a pineal mass were observed on magnetic resonance imaging. Under the clinical impression of a germ cell tumor, the patient was treated with germ cell tumor chemotherapy (cisplatin and etoposide) and radiation therapy without biopsy. After initial shrinkage of the lesions, further growth of the tumor was observed and a biopsy was performed. The histopathology revealed LCH. After chemotherapy according to the LCH III protocol, the tumor disappeared. She is on regular follow up for 5 years without relapse. The present findings indicate that LCH should be included in the differential diagnosis of a suprasellar mass, even in adults, especially when it manifests with diabetes insipidus. This case also underscores the importance of a histopathologic diagnosis in patients with suprasellar tumors before the initiation of a specific therapy, even if the clinical findings are highly suggestive of a specific diagnosis. PMID:27195259

  17. [Possibilities and limits of paraffin-embedded cell markers in diagnosis of primary cutaneous histiocytosis].

    Science.gov (United States)

    Fartasch, M; Goerdt, S; Hornstein, O P

    1995-03-01

    To date, the rare primary histiocytoses of the skin are diagnosed definitively on the basis of the clinical symptoms, H&E-stained sections, and demonstration of CD1 positivity in frozen sections and of Birbeck granules on electron microscopy. The improvement and analysis of antibodies with the ability to react in paraffin tissue allow retrospective evaluation and classification of these disorders. The antibodies for S-100-protein, peanut agglutinin (PNA) and PCNA (proliferating cell nuclear antigen) have been advocated for differentiation of the specific cells of Langerhans cell histiocytosis (LCH) from other histiocytic cell systems. To date the non-Langerhans cell histiocytoses (non-LCH) have no common ultrastructural and immunohistochemical characteristics. The infiltrate is made up of multiple cell populations, which are of significance for the cellular pathobiology (subtypes of monocytes/macrophages and dendritic cells). The number and distribution of the different monocyte/macrophages and dendritic cells and their ability to react with immunohistochemical markers in paraffin tissue can be completely different in different clinical entities. The antibodies against factor XIIIa (shown on xanthoma disseminatum) and the monoclonal antibody Ki-M1P (shown on juvenile xanthogranuloma) seem to be valuable in discrimination between LCH and non-LCH. Both markers show a positive staining pattern with the characteristic large macrophages. In juvenile xanthogranuloma, the foam cells and giant cells express Ki-M1P, KP1 and anti-cathepsin B. Other monocyte/macrophage markers with the ability to react in paraffin tissue, such as Mac387, lysozyme, alpha 1-antitrypsin and Leu-M1 (Anti-CD 15), in contrast, did not show a typical staining pattern with the characteristic large macrophages dominating the histological picture.

  18. Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement

    Energy Technology Data Exchange (ETDEWEB)

    Azouz, E. Michel [University of Miami, Pediatric Radiology Section, Department of Radiology, Miami, FL (United States); Saigal, Gaurav [McGill University, Department of Medical Imaging, Quebec (Canada); Rodriguez, Maria M. [University of Miami, Department of Pathology, Miami, FL (United States); Podda, Antonello [University of Miami, Division of Pediatric Hematology/Oncology, Miami, FL (United States)

    2005-02-01

    Langerhans' cell histiocytosis (LCH) is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often solitary bone lesion that occurs predominantly in the pediatric age group. The hallmark of LCH is the proliferation and accumulation of a specific histiocyte: the Langerhans' cell. In bone this may cause pain and adjacent soft-tissue swelling, but some lesions are asymptomatic. LCH can involve any bone, but most lesions occur in the skull (especially the calvarium and temporal bones), the pelvis, spine, mandible, ribs, and tubular bones. Imaging diagnosis of the disease in bone is first based on the plain radiographic appearance, which is usually a central destructive, aggressive-looking lesion. In the skull, the lesions develop in the diploic space, are lytic, and their edges may be beveled, scalloped or confluent (geographic), or show a ''button sequestrum.'' Vertebral body involvement usually causes collapse, resulting in vertebra plana. With significant recent improvements in the quality of gamma cameras, imaging techniques, and in studying children, bone scintigraphy at diagnosis and on follow-up usually reveals the sites of active disease, especially when the involvement is polyostotic. CT and MR imaging are very useful in providing detailed cross-sectional anatomic detail of the involved bone, including the bone marrow and the adjacent soft tissues. CT is better suited for demonstrating bone detail and MR imaging for bone marrow and soft-tissue involvement. (orig.)

  19. A solitary pulmonary ground-glass nodule in adult systemic langerhans’ cell histiocytosis

    Science.gov (United States)

    Zhou, Ying; Li, Yunyuan; Fan, Li; Liu, Shiyuan

    2015-01-01

    During a thoracic computed tomography (CT) scan, a 36-year-old male was diagnosed with a solitary oval pulmonary mixed ground-glass nodule in the right upper lobe of the lung. The edge of the nodule was well-defined, and its largest axial size was approximately 1.1×0.9 cm2. This nodule was slightly lobulated, but not obviously speculated. Solid components, micro-cystic lucency shadow, small high-density rings and tiny vascular branches were all visible in the nodule. During hospitalization, a technetium 99 m methylene diphosphonate (Tc-99 m MDP) bone scan was performed, which showed a skeletal foci with abnormal uptake in the left iliac. A pulmonary lobectomy of the right upper lobe of the lung by video-assisted thoracoscopy was performed. In post-operative pathological photomicrographs, proliferative Langerhans’ cells, eosinophils and lymphocytes were found. Immunohistochemistry showed that the expression of S-100 protein, CD1a, and CD68 antigen all stained positive. Since Langerhans’ cell histiocytosis (LCH) that is also associated with isolated mixed ground-glass nodules is relatively rare, such a multi-systemic LCH case as identified herein, is reported. PMID:26722574

  20. Intracranial manifestations of Langerhans cell histiocytosis. MR imaging; Intrakranielle Manifestationen der Langerhanszell-Histiozytose. Kernspintomographische Befunde

    Energy Technology Data Exchange (ETDEWEB)

    Kujat, C. [Universitaetskliniken des Saarlandes, Homburg/Saar (Germany). Inst. fuer Neuroradiologie; Hermes, M. [Universitaetskliniken des Saarlandes, Homburg/Saar (Germany). Inst. fuer Neuroradiologie; Heide, G. [Universitaetskliniken des Saarlandes, Homburg/Saar (Germany). Neurologische Klinik; Baeumler, S. [Universitaetskliniken des Saarlandes, Homburg/Saar (Germany). Klinik fuer Kinder- und Jugenmedizin; Feiden, W. [Universitaetskliniken des Saarlandes, Homburg/Saar (Germany). Abt. fuer Neuropathologie

    1994-11-01

    We report on 3 patients with intracranial manifestations on Langerhans cell histiocytosis (LH). The results are correlated with histological, clinical and radiological reports on some 70 patients described in the literature as suffering from intracranial LH. Two different morphological pictures can be differentiated. First, typical infiltrates can be seen by microscopy; these can be located in every part of the brain, but are seen mainly in the region of te hypothalamus. These infiltrations are shown as space-occupying lesions with Gd-DTPA enhancement. The other manifestation is a demyelinized lesion with a sparse infiltration of Langerhans cells. These lesions, which are located mainly in the region of the nucleus dentatus of the cerebellum and the brain stem, show increased signal intensity in the T2 sequence and no Gd-DTPA enhancement on MRI. (orig.) [Deutsch] Es werden die Befunde von 3 Patienten mit intrakranieller Manifestation einer Langerhanszell-Histiozytose (LH) dargestellt. Die Korrelation mit den histologischen, radiologischen und/oder klinischen Befunden bei etwa 70 in der Literatur erfassten Patienten mit intrakranieller LH laesst 2 morphologische Bilder bei dieser Krankheit erkennen: In der Regel sind histologisch definierte LH-Infiltrate zu erkennen, die bevorzugt in der Hypothalamusregion - jedoch letztlich in jedem Teil des Gehirns - auftreten koennen und sich im MRT als gadoliniumaffine Raumforderung darstellen. Die andere Manifestationsform dominiert im Bereich des Nucleus dentatus des Kleinhirns und im Hirnstamm. Es handelt sich um Demyelinisierungsherde mit spaerlicher Histiozyteninfiltration, die im MRT als nicht gadoliniumaffine Bezirke zu erkennen sind. (orig.)

  1. Isolated Langerhans cell histiocytosis of the sublingual gland in an adult

    Science.gov (United States)

    Yang, Shaodong; Chen, Xinming; Zhang, Jiali; Fang, Qiong

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the proliferation of pathologic Langerhans cells. Its clinical presentation is highly variable, that range from single-system, limited disease to severe, multi-organ disease with high mortality. LCH usually affects children and young adults. The most frequent sites for LCH are the bone, skin, lung, pituitary gland, and lymph nodes. Salivary gland involvement by LCH is extremely rare, and only a few cases of LHC involving the parotid glands have been reported in the English literature. To our knowledge, the involvement of the sublingual gland as a part of single or multisystem LCH has not been previously described. Herein we reported the first case of primary LCH of the sublingual gland. A 40-year-old woman presented with a 2-month history of a painless mass on the right sublingual area. Excision of the lesion including the right sublingual gland was performed. Histopathological diagnosis of LCH was rendered. The patient remains free of symptoms 17 months after surgery. PMID:26722591

  2. Role of multidisciplinary approach in a case of Langerhans cell histiocytosis with initial periodontal manifestations

    Science.gov (United States)

    Cisternino, Angelo; Asa’ad, Farah; Fusco, Nicola; Ferrero, Stefano; Rasperini, Giulio

    2015-01-01

    Introduction: Langerhans cell histiocytosis (LCH) is a rare inflammatory myeloid neoplasia of unknown etiology occurring in both children and adults. This condition is characterized by an abnormal proliferation of Langerhans cells that may virtually affect all sites in the human body. Oral manifestations of LCH could be the first clinical sign of disease and its periodontal localization could be easily mistaken for other more common entities, such as chronic periodontitis, aggressive periodontitis, and necrotizing ulcerative periodontitis. Case presentation: A 32-years old female visited a private dental practice with a chief complaint of sensitivity in the mandibular left first molar. Clinical and radiographic examination revealed deep periodontal pocket, recession, furcation involvement, mobility, severe alveolar bone destruction and a diagnosis of aggressive periodontitis was rendered. Multiple tooth extractions were carried out due to progressive periodontal destruction with impaired healing and development of ulcerative lesions. Multidisciplinary investigation demonstrated that the periodontal involvement was a manifestation of an underlying systemic disease. A biopsy of a bone lesion was therefore performed, revealing the presence of multifocal single system LCH. Conclusion: The identification of periodontal LCH is not trivial given that it may clinically resemble other periodontal disease entities. The dentist can be the first health care personnel to unravel the presence of an underlying systemic LCH. PMID:26722570

  3. Local Langerhans cell histiocytosis (eosinophilic granuloma in a six-month baby: a case report

    Directory of Open Access Journals (Sweden)

    Bahador M

    2008-12-01

    Full Text Available "nBackground: Langerhans cell histiocytosis (LCH is a group of idiopathic disorders characterized by the proliferation of specialized bone marrow-derived Langerhans cells and mature eosinophils. The estimated annual incidence ranges from 0.5-2 cases per 100,000 persons per year. The pathogenesis of LCH is unknown. The prevalence of LCH seems to be higher among whites and males. The most common complaints at presentation are those related to bone lesions. Treatment consists of surgery, chemotherapy and radiotherapy alone or in combination. The age of onset varies according to the variety of LCH. Solitary lesions may occur in bones or skin. Cutaneous lesions present with firm, painless papulonodules or vesicles. "nCase report: This six-month-old baby presented with firm papulonodules on her temporal skin, but fortunately her other organs were healthy. She underwent two surgeries, separated by a one-month interval. Due to local recurrence after a short period of time, she underwent a 10-Gy dose of radiation. Her response proved good during follow-up. "nConclusion: Radiotherapy is good for controlling local recurrence in LCH, with few sequelae related to treatment.

  4. [Xanthomas in a patient with Langerhans cell histiocytosis and liver cirrhosis].

    Science.gov (United States)

    Calzado, Leticia; Postigo, Concepción; Prado Sánchez-Caminero, M; Sanz, Henar; Guerra, Aurora; Vanaclocha, Francisco; Rodríguez-Peralto, José L

    2005-10-01

    Skin involvement in acute forms of Langerhans cell histiocytosis (LCH) is in the form of erythematous papules, although rare forms of xanthomatous lesions have been described. We present the case of a boy with acute disseminated LCH who, at the age of 16 months, began to experience outbreaks of seborrheic dermatitis-like skin lesions and progressive hepatic dysfunction. The symptoms were complicated by partial central diabetes insipidus and specific pulmonary infiltration by Langerhans cells, which led to fibrosis. During the course of the disease, the patient developed liver cirrhosis, alterations in the lipid profile and disseminated xanthomatous skin lesions, concomitant with the lesions specific to the LCH. Despite successive cycles of chemotherapy, the outcome was the death of the patient after five years, due to his liver disease. Xanthomatous lesions in LCH are typical of the late stages of chronic progressive forms, such as Hand-Schüller-Christian disease. When they appear in acute disseminated forms, there is some controversy over whether they correspond to a progression of the disease towards more chronic forms, or whether they are associated independent lesions, such as in this case.

  5. Langerhans cell histiocytosis: literature review and descriptive analysis of oral manifestations.

    Science.gov (United States)

    Madrigal-Martínez-Pereda, Cristina; Guerrero-Rodríguez, Vanesa; Guisado-Moya, Blanca; Meniz-García, Cristina

    2009-05-01

    Langerhans cell histiocytosis (LCH) is a rare disease, of unknown pathogenesis, characterized by intense and abnormal proliferation of bone marrow-derived histiocytes (Langerhans cells). It can present both local and systemic manifestations involving bone, skin and mucosal tissue, and internal organs. Three basic clinical forms develop: Letterer-Siwe disease (subacute or acute disseminated form), Hand-Schüller-Christian disease (disseminated chronic form) and eosinophilic granuloma (localized chronic form). LCH may manifest orally with single or multiple lesions of the alveolar or basal bone, ulcerated mucosal lesions accompanied by adenopathies and/or periodontal lesions, presenting gingival inflammation, bleeding, recession, necrosis, odontalgia, dental hypermobility and premature loss of teeth. The principal differential diagnoses include advanced periodontal disease or a periapical process of dental or periodontal origin. The odontologist plays a vital role in the diagnosis and multidisciplinary treatment of such patients, by performing routine examinations for periodic follow-up of the disease and its possible oral manifestations, bearing in mind that these may be the first or only signs of LCH.

  6. The role of bone scintigraphy in Langerhans' cell histiocytosis; a case report; Interet de la scintigraphie osseuse dans l'histiocytose langerhansienne: a propos d'un cas

    Energy Technology Data Exchange (ETDEWEB)

    Razzouk, M.; Carrier, P.; Darcourt, J. [Centre TEP, Hopital de l' Archet-1, Service Universitaire de Medecine Nucleaire, 06 - Nice (France); Razzouk, M.; Carrier, P.; Darcourt, J. [Federation interhospitaliere, CHU, CAL universitaire de Nice, 06 - Nice (France); Deville, A. [Hopital de l' Archet, Service d' Hemato-Oncologie Pediatrique, 06 - Nice (France); Cadet, G. [Centre Hospitalier intercommunal de Grasse, Service de Pediatrie, 06 - Grasse (France)

    2008-09-15

    We present one case of bone-Langerhans cell histiocytosis in a three-year-old male child presenting osseous lesions in the skull and the femur, which are very frequent localizations in histiocytosis. Bone scintigraphy is useful for both initial staging and follow-up associated with other imaging modalities. (authors)

  7. Button sequestrum in a case of localized Langerhans' cell histiocytosis of the ilium: case report

    Energy Technology Data Exchange (ETDEWEB)

    Tordeur, M. [Pitie-Salpetriere Teaching Hospital, Radiology Dept., Paris (France); Wybier, M. [Lariboisiere Teaching Hospital, Paris (France); Laporte, J.L. [Pitie-Salpetriere Teaching Hospital, Pathology Dept., Paris (France); Grenier, P. [Pitie-Salpetriere Teaching Hospital, Radiology Dept., Paris (France); Laredo, J.D. [Lariboisiere Teaching Hospital, Paris (France)

    2000-04-15

    Langerhans' cell histiocytosis (LCH) is characterized by a proliferation of cells exhibiting the same immunohistochemical and ultra-structural characteristics as Langerhans' cells of the epidermis. Eosinophilic granuloma, chronic polyostotic disease (Hand-Schuller-Christian disease) and multisystemic disease (Letterer-Siwe disease) all belong to the spectrum LCH. Osseous lesions are the most common findings. the radiological appearance of skeletal LCH depends on the site and on disease activity. Button sequestrum has been described as uncommon in LCH of the skull and exceedingly rare in LCH at other sites. We report a case of localized LCH of the ilium with a button sequestrum. (author)

  8. Clinicopathological pattern of cranial unifocal Langerhans cell histiocytosis: A study at medical college hospital

    Directory of Open Access Journals (Sweden)

    Salma Bhat

    2015-01-01

    Full Text Available Background: Eosinophilic granuloma (EG of bone refers to a generally benign form of Langerhans cell histiocytosis localized to the bone. Patients may present with a solitary lesion (monostotic or multiple sites of involvement (polyostotic. Materials and Methods: This study was done to evaluate the clinicopathological pattern of 6 cases of EGs of the skull diagnosed at a tertiary care hospital. All patients of EG were included with the help of medical records over a 5-year period that is, November 2009 to November 2014. They all had been preoperatively evaluated by skull X-ray and computed tomography. To rule out a multifocal disease scintigraphy was performed in all cases preoperatively. Surgical excision was performed, and EG was diagnosed on histopathology and immunohistochemistry. Results: There was a male predominance. Parietal bone was the most common affected bone. Total excision of the lesion was performed in all cases. No patient received postoperative radiotherapy. The follow-up period ranged from 6 months to 3 years. No tumor recurrence was noted. Conclusion: With an unknown etiology, nonspecific clinical and radiological findings with diagnosis possible only on histopathological examination, EG needs to be considered in the differential diagnosis as a skull mass, especially in children.

  9. Coincidence FDG-PET in the evaluation of Langerhans' cell histiocytosis: preliminary findings

    Energy Technology Data Exchange (ETDEWEB)

    Binkovitz, Larry A.; Adler, Brent H. [Department of Radiology, Columbus Children' s Hospital, OH (United States); Olshefski, Randal S. [Department of Hematology and Oncology, Columbus Children' s Hospital, OH (United States)

    2003-09-01

    Bone involvement in Langerhans' cell histiocytosis (LCH) is common. Both bone scintigraphy and plain films are used to identify osseous lesions, but lack specificity for disease activity and response to therapy. FDG-PET is a sensitive technique for identifying bone lesions when histiocytes are present. To describe the potential of coincidence FDG-PET (cFDG-PET) for identification of active bone lesions in LCH and to determine whether it can provide more specific information regarding lesional response to therapy than bone scintigraphy or radiography. The clinical data and imaging findings of three patients with osseous lesions of LCH were retrospectively reviewed. cFDG-PET identified all active LCH osseous lesions in these patients, differentiated active from healed lesions, and demonstrated normalization of uptake in a treated lesion earlier than bone scintigraphy and radiography. cFDG-PET appears to have greater specificity than bone scintigraphy and radiography for the identification of active osseous lesions in LCH. It also may predict response to treatment earlier than conventional techniques. Its use in the evaluation of LCH warrants further study. (orig.)

  10. Adult Multisystem Langerhans Cell Histiocytosis Presenting with Central Diabetes Insipidus Successfully Treated with Chemotherapy

    Science.gov (United States)

    Choi, Jung-Eun; Lee, Hae Ri; Ohn, Jung Hun; Moon, Min Kyong; Park, Juri; Lee, Seong Jin; Choi, Moon-Gi; Yoo, Hyung Joon; Kim, Jung Han

    2014-01-01

    We report the rare case of an adult who was diagnosed with recurrent multisystem Langerhans cell histiocytosis (LCH) involving the pituitary stalk and lung who present with central diabetes insipidus and was successfully treated with systemic steroids and chemotherapy. A 49-year-old man visited our hospital due to symptoms of polydipsia and polyuria that started 1 month prior. Two years prior to presentation, he underwent excision of right 6th and 7th rib lesions for the osteolytic lesion and chest pain, which were later confirmed to be LCH on pathology. After admission, the water deprivation test was done and the result indicated that he had central diabetes insipidus. Sella magnetic resonance imaging showed a mass on the pituitary stalk with loss of normal bright spot at the posterior lobe of the pituitary. Multiple patchy infiltrations were detected in both lung fields by computed tomography (CT). He was diagnosed with recurrent LCH and was subsequently treated with inhaled desmopressin, systemic steroids, vinblastine, and mercaptopurine. The pituitary mass disappeared after two months and both lungs were clear on chest CT after 11 months. Although clinical remission in multisystem LCH in adults is reportedly rare, our case of adult-onset multisystem LCH was treated successfully with systemic chemotherapy using prednisolone, vinblastine, and 6-mercaptopurine, which was well tolerated. PMID:25309800

  11. Cladribine and cytarabine in refractory multisystem Langerhans cell histiocytosis: results of an international phase 2 study

    Science.gov (United States)

    Bernard, Frederic; van Noesel, Max; Barkaoui, Mohamed; Bardet, Odile; Mura, Rosella; Arico, Maurizio; Piguet, Christophe; Gandemer, Virginie; Armari Alla, Corinne; Clausen, Niels; Jeziorski, Eric; Lambilliote, Anne; Weitzman, Sheila; Henter, Jan Inge; Van Den Bos, Cor

    2015-01-01

    An international phase 2 study combining cladribine and cytarabine (Ara-C) was initiated for patients with refractory, risk-organ–positive Langerhans cell histiocytosis (LCH) in 2005. The protocol, comprising at least two 5-day courses of Ara-C (1 g/m2 per day) plus cladribine (9 mg/m2 per day) followed by maintenance therapy, was administered to 27 patients (median age at diagnosis, 0.7 years; median follow-up, 5.3 years). At inclusion, all patients were refractory after at least 1 course of vinblastine (VBL) plus corticosteroid, all had liver and spleen involvement, and 25 patients had hematologic cytopenia. After 2 courses, disease status was nonactive (n = 2), better (n = 23), or stable (n = 2), with an overall response rate of 92%. Median disease activity scores decreased from 12 at the start of therapy to 3 after 2 courses (P < .0001). During maintenance therapy, 4 patients experienced reactivation in risk organs. There were 4 deaths; 2 were related to therapy toxicity and 2 were related to reactivation. All patients experienced severe toxicity, with World Health Organization grade 4 hematologic toxicity and 6 documented severe infections. The overall 5-year survival rate was 85% (95% confidence interval, 65.2%-94.2%). Thus, the combination of cladribine/Ara-C is effective therapy for refractory multisystem LCH but is associated with high toxicity. PMID:26194764

  12. Whole-body MRI of Langerhans cell histiocytosis: comparison with radiography and bone scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Goo, Hyun Woo; Yang, Dong Hyun [Asan Medical Center, Department of Radiology, University of Ulsan College of Medicine, Seoul (Korea); Ra, Young Shin [Asan Medical Center, Department of Neurosurgery, University of Ulsan College of Medicine, Seoul (Korea); Song, Joon Sup; Im, Ho Joon; Seo, Jong Jin; Ghim, Thad; Moon, Hyung Nam [Asan Medical Center, Department of Pediatric Oncology, University of Ulsan College of Medicine, Seoul (Korea)

    2006-10-15

    In Langerhans cell histiocytosis (LCH) evaluation of the extent of disease is one of the major predictors of patient outcome. Historically this is undertaken using plain radiography and bone scintigraphy. Recently, whole-body (WB) MRI has been reported to be useful in detecting skeletal and extraskeletal metastases in both adults and children. To evaluate the usefulness of WB MRI in patients with LCH in comparison with plain radiography and bone scintigraphy. In nine children (1-7 years of age; mean 3.3 years) who had a pathological diagnosis of LCH and had either plain radiography or bone scintigraphy for comparison, 43 WB MR examinations were performed. Skeletal and extraskeletal lesions of the disease on WB MRI were compared with those on plain radiography and bone scintigraphy. LCH showed unifocal single-system involvement in one patient, multifocal single-system involvement in three, and multifocal multisystem disease in five. WB MRI identified additional skeletal lesions in three (38%) of eight patients, compared with plain radiography, and in two (25%) of eight, compared with bone scintigraphy. WB MRI detected extraskeletal lesions of the disease in five (56%) of the nine patients exclusively, except for one patient whose lung lesions were also detected on plain radiography. In two patients, treatment was changed according to WB MRI findings. (orig.)

  13. Clinicopathological pattern of cranial unifocal Langerhans cell histiocytosis: A study at medical college hospital

    Science.gov (United States)

    Bhat, Salma; Nazir, Parvez; Bashir, Humaira; Reshi, Ruby; Sheikh, Sheema; Wani, Rohi

    2015-01-01

    Background: Eosinophilic granuloma (EG) of bone refers to a generally benign form of Langerhans cell histiocytosis localized to the bone. Patients may present with a solitary lesion (monostotic) or multiple sites of involvement (polyostotic). Materials and Methods: This study was done to evaluate the clinicopathological pattern of 6 cases of EGs of the skull diagnosed at a tertiary care hospital. All patients of EG were included with the help of medical records over a 5-year period that is, November 2009 to November 2014. They all had been preoperatively evaluated by skull X-ray and computed tomography. To rule out a multifocal disease scintigraphy was performed in all cases preoperatively. Surgical excision was performed, and EG was diagnosed on histopathology and immunohistochemistry. Results: There was a male predominance. Parietal bone was the most common affected bone. Total excision of the lesion was performed in all cases. No patient received postoperative radiotherapy. The follow-up period ranged from 6 months to 3 years. No tumor recurrence was noted. Conclusion: With an unknown etiology, nonspecific clinical and radiological findings with diagnosis possible only on histopathological examination, EG needs to be considered in the differential diagnosis as a skull mass, especially in children. PMID:26855527

  14. Radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis in nine patients

    Energy Technology Data Exchange (ETDEWEB)

    Song, You Seon; Lee, In Sook; Kim, Do Kyung [Pusan National University Hospital, Medical Research Institute, Department of Radiology, Busan (Korea, Republic of); Pusan National University, School of Medicine, Department of Radiology, Busan (Korea, Republic of); Yi, Jae Hyuck [Kyungpook National University Hospital, Department of Radiology, Daegu (Korea, Republic of); Cho, Kil Ho [Yeungnam University College of Medicine, Department of Radiology, Daegu (Korea, Republic of); Song, Jong Woon [Inje University Haeundae Paik Hospital, Department of Radiology, Busan (Korea, Republic of)

    2011-11-15

    The purpose of this article was to evaluate the radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis (LCH), emphasizing the CT and MR findings. The images of nine patients with pathologically proven LCH (five men and four women; mean age, 37.11 years) were retrospectively reviewed. Imaging analysis was confined to the long and flat bones. CT scans were performed in five patients and MR imaging was performed in eight. Images were assessed for the following features on CT and MRI: the location and number of lesions; the presence of cortical destruction, endosteal scalloping, and a periosteal reaction on CT or MRI; the margin of soft tissue masses, the presence of bone marrow edema, and a ''budding'' appearance on MRI; and the presence of sclerotic margins or septations on CT. The involved skeletal sites were the pelvis (seven), femurs (five), humeri (two), tibias (two), fibula (one), clavicle (one), scapula (one), and sternum (one). Endosteal scalloping, a periosteal reaction, and a budding appearance were common on MRI or CT images. Although cortical destruction and the soft tissue lesion formation were rare, soft tissue masses had well-defined margins. Endosteal scalloping and a budding appearance with a periosteal reaction on CT and MRI may be helpful signs for differentiation of LCH from malignant tumors in adults. (orig.)

  15. A Rare Case of Vulvar Langerhans Cell Histiocytosis and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Didem Didar Balcı

    2009-12-01

    Full Text Available Langerhans cell histiocytosis (LCH of the vulva is rare. A 32 year-old woman presented with a two year history of non-healing vulvar ulceration and a nine month history of ulcerative nodular lesion in the left inguinal region. Biopsy results of the vulva and inguinal lesions were consistent with LCH. Screening of the body systems revealed hepatosplenomegaly, iliac, inguinal, paraaortic lymph nodes and lung involvement. She was treated with systemic steroid, resulting in complete improvement of vulvar lesion. She also underwent a local excision of left inguinal lesion. Three months later, we found a lesion on her left inguinal region that was consistent with a recurrence and enlargement in the lymph nodes. Therefore, high dose systemic steroid therapy and then a 12-session radyotherapy were given. After the treatment, a complete improvement was obtained. A 1-year of systemic methotrexate therapy was recommended to prevent recurrence of the disease. In women with vulvar ulcer, LCH should be considered in the differential diagnosis.

  16. {sup 18}F-FDG PET in neuro-degenerative Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Ribeiro, M.Jo. [CEA, DSV, DRM, Serv Hosp Frederic Joliot, F-91406 Orsay (France); Idbaih, A.; Hoang-Xuan, K. [UPMC, Grp Hosp Pitie Salpetriere, ServNeurol Mazarin, AP-HP, Paris (France); Thomas, C. [CHU Nantes, Unite Hematol et Oncol Pediat, F-44035 Nantes 01 (France); Remy, P. [CEA, Serv Hosp Frederic Joliot, CNRS, URA 2210, F-91406 Orsay (France); Remy, P. [CHU Henri Mondor, Fac Med Paris 12, Dept Neurosci, AP-HP, F-94010 Creteil (France)

    2008-07-01

    Introduction: The so called 'neuro-degenerative Langerhans cell histiocytosis' (ND-LCH) is a rare and severe complication of LCH presenting as a progressive cerebellar ataxia associated with pyramidal tract signs, and cognitive impairment. MRI is the gold standard to investigate CNS lesions of ND-LCH but little is known about functional changes observed in this disease. Objectives: To search for CNS metabolic changes in NDLCH. Methods: Seven patients suffering from ND-LCH were investigated by {sup 18}F-FDG PET in this prospective study and compared with 21 healthy controls. Results: ND-LCH patients demonstrated recurrent abnormalities including bilateral hypo-metabolism in the cerebellum, the basal ganglia (caudate nuclei), frontal cortex and, bilateral, a relatively increased metabolism in the amygdalae (p {<=} 0.001). Functional changes in these anatomical regions may be detected in the absence of any apparent lesion on MRI. Conclusions: ND-LCH demonstrates a recurrent {sup 18}F-FDG PET metabolic signature. Our results suggest that {sup 18}F-FDG PET might be a useful tool for an early diagnosis of ND-LCH before neuro-radiologic abnormalities appear. (authors)

  17. Radiotherapy for bony manifestations of Langerhans Cell Histiocytosis. Review and proposal for an international registry

    Energy Technology Data Exchange (ETDEWEB)

    Olschewski, T.; Seegenschmiedt, M.H. [Dept. of Radiotherapy and Radiation Oncology, Alfried Krupp Krankenhaus, Essen (Germany)

    2006-02-01

    Purpose: to examine the role of radiotherapy (RT) in adult Langerhans Cell Histiocytosis (LCH) for osseous manifestations, to define open questions regarding RT, and to develop recommendations for the clinical decision-making and problem-solving process. Material and methods: a literature review using different medical databases was conducted including the last 3 decades, and resulting questions regarding the use of ionizing radiation were systematically compiled. Results: the literature review revealed a local control rate of 96% (93% complete remissions) in patients with osseous single-system disease and of 92% (76% complete remissions) in patients with bony involvement in multi-system disease. To increase our knowledge, a prospective registry has been developed to allow a differentiated analysis of RT outcome and definition of potential prognostic factors. Conclusion: ionizing radiation can be successfully applied as a single treatment or in combination with other therapies for osseous manifestations of LCH. It leads to high remission and local control rates. Nevertheless, many open questions still exist. A prospective clinical registry is proposed to define the exact role of RT in this disease and to develop future interdisciplinary treatment guidelines. (orig.)

  18. Radiographic skeletal survey and radionuclide bone scan in Langerhans cell histiocytosis of bone

    Energy Technology Data Exchange (ETDEWEB)

    Nieuwenhuyse, J.P. van [Department of Radiology and Medical Imaging, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Clapuyt, P. [Department of Radiology and Medical Imaging, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Malghem, J. [Department of Radiology and Medical Imaging, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Everarts, P. [Department of Radiology and Medical Imaging, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Melin, J. [Department of Nuclear Medicine, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Pauwels, S. [Department of Nuclear Medicine, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Brichard, B. [Department of Pediatric Hematology, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Ninane, J. [Department of Pediatric Hematology, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Vermylen, C. [Department of Pediatric Hematology, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Cornu, G. [Department of Pediatric Hematology, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium)

    1996-10-01

    Background. The lack of a consensus in the literature on the imaging strategy in Langerhans cell histiocytosis (LCH) bone lesions in childhood. Objective. To evaluate the relative value of radionuclide bone scan (RBS) and radiographic skeletal survey (RSS) in the detection of LCH bone lesions, both in the initial work-up of the disease and during the follow-up period. Materials and methods. Ten children with bone lesions evaluated by means of RSS and RBS in a retrospective study (1984-1993). Results. Fifty radiologically and/or scintigraphically abnormal foci were detected: 27 anomalies in the initial work-up (12 by both RSS and RBS, 8 by RSS only and 7 by RBS only) and 23 additional anomalies during follow-up (10 by both RSS and RBS, 10 by RSS only and 3 by RBS only). RSS+/RBS- lesions (n = 18) are more frequently encountered in the skull (P = 0.038), and more frequently lack radiologic signs of osteoblastic activity (P = 0.020), than RSS+/RBS+ lesions (n = 22). RSS-/RBS+ abnormalities (n = 10) were most frequently insignificant. Conclusion. In the initial work-up both RSS and RBS should be carried out, while in the follow-up only RSS should be performed. (orig.). With 2 figs., 4 tabs.

  19. Langerhans cell histiocytosis: a retrospective analysis in a Korean tertiary hospital from 2003 to 2012.

    Science.gov (United States)

    Kwon, Soon Hyo; Choi, Jae Woo; Kim, Hyo Jin; Youn, Sang Woong

    2013-10-01

    Epidemiological study of Langerhans cell histiocytosis (LCH) has been limited due to its rarity and multisystemic involvement. The aim of this study was to investigate the epidemiological features of LCH via the clinical data warehouse (CDW). Clinical data of 30 LCH patients from the all departments of a tertiary referral hospital between 2003 and 2012 were analyzed retrospectively by searching the CDW. The male-to-female ratio was 2.8:1. The age of onset ranged 7 days to 57 years with a median of 13 years. Of the patients, 36.7% presented initial symptoms before the age of 10 years. The involved organs at diagnosis were: bone (66.7%), skin (16.7%), lungs (13.3%) and lymph node (3.3%). For all of the 30 cases, there were 31 disease sites because of a single case of multisystemic disease involving both skin and bone. Of the 96.7% of patients with single-system disease, 69.0% had bony involvement. This study elucidated the clinical features of LCH from all the departments of a tertiary hospital via the CDW, which suggests a potential role of the CDW as a new epidemiological approach for rare diseases.

  20. Occult Langerhans Cell Histiocytosis Presenting with Papillary Thyroid Carcinoma, a Thickened Pituitary Stalk and Diabetes Insipidus

    Science.gov (United States)

    2016-01-01

    Etiologies of a thickened stalk include inflammatory, neoplastic, and idiopathic origins, and the underlying diagnosis may remain occult. We report a patient with a thickened pituitary stalk (TPS) and papillary thyroid carcinoma (PTC) whose diagnosis remained obscure until a skin lesion appeared. The patient presented with PTC, status postthyroidectomy, and I131 therapy. PTC molecular testing revealed BRAF mutant (V600E, GTC>GAG). She had a 5-year history of polyuria/polydipsia. Overnight dehydration study confirmed diabetes insipidus (DI). MRI revealed TPS with loss of the posterior pituitary bright spot. Evaluation showed hypogonadotropic hypogonadism and low IGF-1. Chest X-ray and ACE levels were normal. Radiographs to evaluate for extrapituitary sites of Langerhans Cell Histiocytosis (LCH) were unremarkable. Germinoma studies were negative: normal serum and CSF beta-hCG, alpha-fetoprotein, and CEA. Three years later, the patient developed vulvar labial lesions followed by inguinal region skin lesions, biopsy of which revealed LCH. Reanalysis of thyroid pathology was consistent with concurrent LCH, PTC, and Hashimoto's thyroiditis within the thyroid. This case illustrates that one must be vigilant for extrapituitary manifestations of systemic diseases to diagnose the etiology of TPS. An activating mutation of the protooncogene BRAF is a potential unifying etiology of both PTC and LCH. PMID:27656301

  1. A confusing case report of pulmonary langerhans cell histiocytosis and literature review

    Directory of Open Access Journals (Sweden)

    Li Yang

    2016-01-01

    Full Text Available Pulmonary Langerhans Cell Histiocytosis (PLCH is a rare disease. From the insidious onset and nonspecific manifestations, it is difficult to diagnose PLCH. To help improve the diagnosis and therapy options of adult PLCH, we present this case report and literature review about a confusing case of PLCH. In this report, we present a 37-year-old male PLCH case that was negative for CD1a and S100 expression. Smoking cessation and use of prescribed Spiriva appeared to improve the patient’s symptoms. To the best of our knowledge, this is the first reported case of PLCH in which improved symptoms were seen with the use of Spiriva alone.The mechanism is not clear, but potentially has some relationship with dilating the airway, decreasing the mucous hypersecretion and promoting anti-inflammatory pathways. From this patient’s case, we may be able to find more cases to then find other first line therapies for PLCH patients.

  2. Langerhans Cell Histiocytosis of the Cranial Base: Is Low-Dose Radiotherapy Effective?

    Directory of Open Access Journals (Sweden)

    Andreas Meyer

    2012-01-01

    Full Text Available Introduction. Langerhans cell histiocytosis (LCH is a rare disease of unknown etiology with different clinical features. A standardised treatment has not been established so far. Case Report. We report a case of a 28-year-old patient who initially presented with hypesthesia of the fifth cranial nerve and pain of the left ear. Diagnosis showed a tumour localised in the cranial base with a maximum diameter of 4.1 cm. The diagnosis of LCH was confirmed histologically by biopsy. Diagnostic workup verified the cranial lesion as the sole manifestation of LCH. A total dose of 9 Gy (single dose 1.8 Gy was delivered. The symptoms dissolved completely within 6 months after radiation; repeated CT and MRI scans revealed a reduction in size of the lesion and a remineralisation of the bone. After a followup of 13 years the patient remains free of symptoms without relapse or any side effects from therapy. Discussion. Due to the indolent course of the disease with a high rate of spontaneous remissions the choice of treatment strongly depends on the individual clinical situation. In the presented case low-dose radiotherapy was sufficient to obtain long-term local control in a region with critical structures and tissues.

  3. Purely cutaneous Langerhans cell histiocytosis presenting as an ulcer on the chin in an elderly man successfully treated with thalidomide

    Science.gov (United States)

    Subramaniyan, Radhakrishnan; Ramachandran, Rajagopal; Rajangam, Gnanasekaran; Donaparthi, Navya

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare, clonal proliferative disorder of Langerhans’ cells of unknown etiology. Although the clinical presentation and therapeutic approach to the disease in children have been well established; limited data is available about the disease in adults. Purely cutaneous involvement of LCH in a man older than 70 years has rarely been described. Herein we report the case of a 71-year-old man with cutaneous LCH confined to the perioral region, scalp, and flexures successfully treated with thalidomide. PMID:26753141

  4. Radiotherapy of Langerhans' cell histiocytosis. Results and Implications of a national patterns-of-care study

    Energy Technology Data Exchange (ETDEWEB)

    Seegenschmiedt, M.H. [Alfried Krupp Krankenhaus, Essen (Germany). Dept. of Radiation Oncology and Radiotherapy; Olschewski, T.

    2006-11-15

    Purpose: This patterns-of-care study was performed to define the current clinical experience with radiotherapy of Langerhans' cell histiocytosis in adults in Germany and to define open questions resulting from this study. Material and Methods: A standardized questionnaire was sent to 198 German radiotherapy institutions. Data about patient characteristics, stage of disease, practice and fractionation of radiotherapy, outcome of therapy, etc. were systematically evaluated. 123 of 198 institutions answered the complete questionnaire (62.1%). Results: Only 23 of the 123 institutions (18.7%) reported experience with radiotherapy of Langerhans' cell histiocytosis of adults. 18 institutions with 98 patients were evaluable. The majority of patients (72 of 98) was treated on a linear accelerator. The median single dose of radiotherapy was 2 Gy, while the median total dose was 24 Gy. 81 of 89 evaluable patients (91%) reached a local control of the treated lesion(s), 69 of those had a complete remission. Eight of 89 patients (9%) developed an in-field recurrence. 87.8% of patients experienced no acute and 97% of patients no late side effects of radiotherapy. Conclusion: Clinical experience with radiotherapy of Langerhans' cell histiocytosis in adults in Germany is still very limited. Nevertheless, the clinical results - with high remission and local control rates - confirm the effectiveness of radiotherapy in the multidisciplinary treatment of this disease. Due to the small number of patients in this study despite higher incidence rates, the knowledge of this disease has to be multiplied in Germany. Future patients should be systematically included into a prospective radiotherapy registry. (orig.)

  5. Primary Langerhans cell histiocytosis of the vulva: Report of a case and brief review of the literature

    Directory of Open Access Journals (Sweden)

    Pan Zenggang

    2009-01-01

    Full Text Available Primary Langerhans cell histiocytosis (LCH of the vulva is rare. Fifteen cases of primary cutaneous vulvar LCH have been reported in English literature. We report an additional case of LCH confined to the vulva. In this article, we describe the clinical presentation, histopathology and immunohistochemistry findings of vulvar LCH that are helpful to both gynecologists and pathologists in the diagnosis of this entity. We briefly discuss the pathogenesis of LCH. The debate whether LCH is a reactive or neoplastic entity is still ongoing.

  6. Detection of Epstein-Barr Virus DNA in Langerhans Cell Histiocytosis

    Science.gov (United States)

    Khoddami, Maliheh; Nadji, Seyed Alireza; Dehghanian, Paria; Vahdatinia, Mahsa; Shamshiri, Ahmad Reza

    2015-01-01

    Background: Langerhans cell histiocytosis (LCH) is a rare histiocytic proliferation of unknown etiology. It is characterized by granuloma-like proliferation of Langerhans-type dendritic cells and mainly affects young children. Although multiple investigators have suggested the possible role of viruses, such as Epstein-Barr virus (EBV), human herpesvirus-6 (HHV-6), Herpes simplex virus (HSV) types 1 and 2, and Cytomegalovirus (CMV) in the pathogenesis of LCH, it remains, however, debated. Objectives: The EBV infection is reported to be associated with LCH. Nevertheless, no report could be found about involved Iranian children in English medical literature. In this study, we investigated the presence of EBV in Iranian children with LCH. Patients and Methods: In this retrospective study, in which we investigated the prevalence of presence of EBV DNA in LCH, using paraffin-embedded tissue samples of 30 patients with LCH and 30 age and tissue-matched controls, who were operated for reasons other than infectious diseases (between the years 2002 and 2012), by real-time polymerase chain reaction (RT-PCR) method, in the department of pediatric pathology. No ethical issues arose in the study, because only the pathology reports were reviewed, retrospectively, and the patients were anonymous. Results: There was a significant difference in prevalence of EBV presence between patients and controls. The EBV was found by RT-PCR in 19 (63.33%) out of 30 patients and only in eight (26.7%) of 30 control samples. The P = 0.004, was calculated using chi-square test (OR: 4.75; 95% CI: 1.58 ‒ 14.25). Conclusions: Our study is the first investigation performed on patients with LCH and its possible association with EBV in Iran. Considering the P = 0.004, which is statistically significant, the findings do support the hypothesis of a possible role for EBV in the pathogenesis of LCH. These results are in accordance with several previous investigations, with positive findings. PMID:26870310

  7. Activity and toxicity of 2-CDA in Langerhans cell histiocytosis: A single institutional experience

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    Biswas G

    2007-01-01

    Full Text Available Background : Langerhans cell histiocytosis (LCH is a rare disorder characterized by clonal proliferation of immature and abnormal bone marrow derived langerhans cells. Treatment is usually multimodal. Potent anti-monocyte as well as immunomodulatory activity of 2-CDA and its proven efficacy in many lymphoproliferative disorders has made 2-CDA a rational choice in treatment of LCH. Aim : To evaluate the efficacy and toxicity profile of 2-CDA in children with relapsed or refractory LCH. Setting and Design : This is a pilot study and we present the initial data of the first seven patients treated at our institution. Materials and Methods : Seven patients of relapsed and refractory LCH were enrolled from July 2000 to June 2004. The cohort of seven patients included six males and one female with a median age at initiation of cladribine was 2.25 years (range, 1.67 to 7.0 years. Three patients had received one prior chemotherapy regimen while the rest were heavily pretreated. Cladribine was administered over two hours IV daily for five days and repeated every four weeks. Results : After a median of six courses of cladribine (range, 2 to 9, two (33% patients achieved PR and two (33% patients have SD on imaging but are clinically better. None experienced grade 3 or 4 hematologic toxicity. At a median follow-up of 19 months (range, 8 to 52 months, five patients remain alive and one patient has died. Conclusion : Our study shows that single agent 2-CDA is active and well-tolerated in children with relapsed or refractory LCH.

  8. Pulmonary Langerhans cell histiocytosis: analysis of 14 patients and literature review

    Science.gov (United States)

    Li, Cheng-Wei; Li, Man-Hui; Li, Jiang-Xiong; Tao, Ru-Jia; Xu, Jin-Fu

    2016-01-01

    Background Pulmonary Langerhans cell histiocytosis (PLCH) is an orphan disease in respiratory medicine, which most affects adult smokers. The purpose of this article was to discuss the clinical features, especially the radiologic features of PLCH patients during their hospitalization through a retrospective analysis on clinical data. Furthermore, the current literature was also reviewed. Methods Between December 2008 and June 2012, 14 patients with PLCH were assessed at Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China. Among these patients, seven patients were diagnosed through tissue biopsy from the lung and one patient from enlarged cervical lymph nodes; the rest of six patients were diagnosed based on the clinical-radiological data. The data consisting of demographics, clinical presentation, smoking habits, pulmonary function tests (PFTs) and radiographic image from the medical records was analyzed retrospectively. Results The average age of patients (11 males and 3 females) was 42.79 (±13.71) years old. All male patients and one female patient had a long smoking history. The common manifestations were cough and exertional dyspnea. Spontaneous pneumothorax was found in three patients. Varieties of pulmonary shadows such as nodular, cystic, patch-like and cord-like were revealed by chest computed tomography (CT) examination. Large Langerhans cells (LCs) were discovered in biopsy tissue by immunohistochemical stains. Conclusions PLCH is still an orphan disease and maybe related to smoking. Clinical symptoms such as cough and exertional dyspnea are non-specific. We shall pay attention to recurrent pneumothorax as clinically it is associated with PLCH. The characteristic radiological manifestation is cystic or nodular shadow in the lungs, which plays crucial roles in diagnosing PLCH. PMID:27293848

  9. Evolving radiological features of hypothalamo-pituitary lesions in adult patients with Langerhans cell histiocytosis (LCH)

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    Makras, P. [Athens General Hospital, Department of Endocrinology and Diabetes, Athens (Greece); Athens General Hospital, Department of Radiology, Athens (Greece); Samara, C.; Antoniou, M.; Nikolakopoulou, Z. [Athens Hospital, 9. Pulmonary Department, Athens (Greece); Zetos, A. [General Hospital, Department of Pathology, Athens (Greece); Papadogias, D.; Piaditis, G.; Kaltsas, G.A. [Athens General Hospital, Department of Endocrinology and Diabetes, Athens (Greece); Toloumis, G. [Athens General Hospital, Department of Radiology, Athens (Greece); Andreakos, E.; Kontogeorgos, G.

    2006-01-01

    Langerhans cell histiocytosis (LCH) is a rare, systemic disease caused by monoclonal expansion of dendritic cells that shows a particular predilection for the hypothalamic-pituitary system (HPS). We studied the function (anterior and posterior pituitary hormonal secretion) and morphology using magnetic resonance imaging (MRI) of the HPS in 17 adult patients (seven males, median age 35 years, range 18-59 years) with multisystem LCH. We also evaluated the evolution of structural HPS abnormalities in relation to pituitary function and response to treatment in 12 of these patients during a median follow-up period of 3.75 years (range 1.5-10 years). Of the 17 patients, 14 (82%) had abnormal HPS imaging, and 12 (70%) had more than one area involved. Lack of the bright spot of the posterior pituitary lobe was typically found in all patients with the diagnosis of diabetes insipidus (DI). Eight patients (47%) had infundibular enlargement, six (35%) pituitary infiltration, four (24%) partially or completely empty sella, three (18%) hypothalamic involvement, and two (12%) infundibular atrophy. DI was found in 16 patients (94%) and anterior pituitary hormonal deficiency (APHD) in 10 patients (59%); two patients had single (12%) and 8 (47%) multiple APHD. During the follow-up period there was improvement of the initially demonstrated HPS pathology in seven (47%) patients, and five (33%) of them had received at least one form of treatment. APHD and DI persisted in all patients except in one in whom established gonadotrophin deficiency recovered. In summary, DI and APHD are very common in patients with multisystem LCH and are almost always associated with abnormal HPS imaging. (orig.)

  10. Clinico-biologic profile of Langerhans cell histiocytosis: A single institutional study

    Directory of Open Access Journals (Sweden)

    Narula G

    2007-01-01

    Full Text Available Context: Langerhans cell histiocytosis (LCH is a rare atypical cellular disorder characterized by clonal proliferation of Langerhans cells leading to myriad clinical presentations and highly variable outcomes. There is a paucity of Indian studies on this subject. Aim: To present the experience of management of LCH at a single institution. Settings and Design: This is a retrospective observational study of patients with LCH who presented at the Tata Memorial Hospital between January 1987 and December 2002. Materials and Methods: Fifty-two patients with LCH were treated in the study period. Due to the long observation period and variability in diagnostic and therapeutic protocols, the patients were risk-stratified based on present criteria. The disease pattern, management approaches and treatment outcomes of patients were recorded. Statistical Analysis Used: Statistical analyses were done using Student′s ′t′ test, test for proportion and survival estimates based on the Kaplan-Meier method. Results: The median age at presentation was 3 years and more than 48% of the patients had Group I disease. Skeleton, skin and lymphoreticular system were the commonly involved organs. Majority (80% required some form of therapy. The projected overall survival is 63% at 10 years and mean survival is 118 months. Seventeen percent of surviving patients developed long-term sequelae. Conclusions: The clinico-biologic profile of LCH patients in India is largely similar to international patterns except a higher incidence of lymphoreticular involvement. Majority of the patients respond favorably to therapy and have a good outcome, except a subset of Group I patients who warrant enrolment in clinical trials with innovative therapeutic strategies to improve outcome.

  11. Tertiary lymphoid structures are confined to patients presenting with unifocal Langerhans Cell Histiocytosis.

    Science.gov (United States)

    Quispel, Willemijn T; Steenwijk, Eline C; van Unen, Vincent; Santos, Susy J; Koens, Lianne; Mebius, Reina; Egeler, R Maarten; van Halteren, Astrid G S

    2016-08-01

    Langerhans cell histiocytosis (LCH) is a neoplastic myeloid disorder with a thus far poorly understood immune component. Tertiary lymphoid structures (TLS) are lymph node-like entities which create an immune-promoting microenvironment at tumor sites. We analyzed the presence and clinical relevance of TLS in n = 104 H&E-stained, therapy-naive LCH lesions of non-lymphoid origin and applied immunohistochemistry to a smaller series. Lymphoid-follicular aggregates were detected in 34/104 (33%) lesions. In line with the lymphocyte recruitment capacity of MECA-79(+) high endothelial venules (HEVs), MECA-79(+)-expressing-LCH lesions (37/77, 48%) contained the most CD3(+) T-lymphocytes (p = 0.003). TLS were identified in 8/15 lesions and contained T-and B-lymphocytes, Follicular Dendritic Cells (FDC), HEVs and the chemokines CXCL13 and CCL21 representing key cellular components and TLS-inducing factors in conventional lymph nodes (LN). Lymphoid-follicular aggregates were most frequently detected in patients presenting with unifocal LCH (24/70, 34%) as compared to patients with poly-ostotic or multi-system LCH (7/30, 23%, p = 0.03). In addition, patients with lymphoid-follicular aggregates-containing lesions had the lowest risk to develop new LCH lesions (p = 0.04). The identification of various stages of TLS formation within LCH lesions may indicate a key role for the immune system in controlling aberrant histiocytes which arise in peripheral tissues.

  12. Selective CT for PET/CT: dose reduction in Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Gelfand, Michael J.; Sharp, Susan E. [Cincinnati Children' s Hospital Medical Center, Section of Nuclear Medicine, Department of Radiology, Cincinnati, OH (United States); Palumbo, Joseph S. [Cincinnati Children' s Hospital Medical Center, Cancer and Blood Diseases Institute, Department of Pediatrics, Cincinnati, OH (United States)

    2014-08-23

    In Langerhans cell histiocytosis (LCH), FDG PET demonstrates active disease in bone. Other imaging modalities show the effects of bone destruction by LCH. To evaluate a selective CT method for reducing effective dose from FDG PET/CT in LCH, using whole-body modified attenuation correction CT at extremely low exposure settings, with repeat selective limited-volume CT at typical localization settings. Fifty-one PET/CT scans were performed in 23 LCH patients, median patient age 8.5 years (range: 1-25 years). Thirty-four were performed with modified attenuation correction CT settings, with bed positions (excluding head and neck) repeated at localization CT settings in regions with abnormal or difficult to interpret PET findings. Of 34 modified attenuation correction PET/CT scans, 10 required repeat localization CT of 1 to 3 bed positions (total: 17 bed positions). Lytic bone lesions were easily recognized at modified attenuation correction settings. Calculated average effective dose for the 34 whole-body CT scans at modified attenuation correction settings was 1.65 mSv. Average effective dose per patient for repeat imaging of 17 bed positions at localization settings was 1.19 mSv. Average total effective dose from CT for all 34 scans performed at the modified attenuation correction CT settings, including the 10 repeat localization CT scans, was 2.0 mSv. High-quality PET scans were consistently obtained with reduced FDG-administered activities of 3.7 MBq/kg (0.10 mCi/kg). In active LCH, abnormal FDG uptake was seen in all lytic bone lesions ≥9 mm, including cranial vault lesions. Substantial reduction in effective dose is possible using selective CT techniques for FDG PET/CT. (orig.)

  13. Central nervous system imaging in childhood Langerhans cell histiocytosis – a reference center analysis

    Science.gov (United States)

    Porto, Luciana; Schöning, Stefan; Hattingen, Elke; Sörensen, Jan; Jurcoane, Alina; Lehrnbecher, Thomas

    2015-01-01

    Background The aim of our study was (1) to describe central nervous system (CNS) manifestations in children with Langerhans cell histiocytosis (LCH) based on images sent to a reference center and meeting minimum requirements and (2) to assess the inter-rater agreement of CNS-MRI results, which represents the overall reproducibility of this investigation. Methods We retrospectively reviewed brain MRI examinations in children with LCH, for which MRI minimum requirements were met. Abnormalities were rated by two experienced neuroradiologists, and the inter-rater agreement was assessed. Results Out of a total of 94 imaging studies, only 31 MRIs met the minimum criteria, which included T2w, FLAIR, T1w images before/after contrast in at least two different section planes, and thin post contrast sagittal slices T1w through the sella. The most common changes were osseous abnormalities, followed by solid enlargement of the pineal gland, thickened enhancing stalk and signal changes of the dentate nucleus. Whereas inter-rater agreement in assessing most of the CNS lesions was relatively high (κ > 0.61), the application of minimum criteria often did not allow to evaluate the posterior pituitary. Conclusions The diversity of radiological protocols from different institutions leads to difficulties in the diagnosis of CNS abnormalities in children with LCH. Although the inter-rater agreement between neuroradiologists was high, not all the LCH manifestations could be completely ruled out when using the minimum criteria. Brain MRIs should therefore follow LCH guideline protocols and include T1 pre-gadolinium sagittal images, and be centrally reviewed in order to improve the comparison of clinical trials. PMID:26401129

  14. A NEW LOOK AT LANGERHANS CELL HISTIOCYTOSIS: REVIEW OF A SERIES OF 55 CASES

    Institute of Scientific and Technical Information of China (English)

    LIU Yue-ping; ZHU Yun-ping; QU Yuan; GAO Li; LIU Xin-fan; XU Guo-zhen; LI Ye-xiong

    2005-01-01

    Objective: Langerhans cell histiocytosis (LCH) has been well described only in children. We analyzed the characteristics, reactivation, and outcome of LCH in a cohort of 55 patients across all ages. Methods: We reviewed the records of all patients with LCH treated at a single institute between Jan. 1974 and May 1998. Results: The 55 patients were 2 to 67 years of age (median, 31 years) at the time of diagnosis, and 85.5% were male. Forty patients (72.7%) had single-system LCH; Fifteen (27.3%) had multisystem disease. The head and neck was the most frequent tumor site (63.6%). LCH was not found in organs at risk of involvement (liver, spleen, bone marrow, and lungs). The frequency of bony invasion (23.6% overall) differed significantly according to age (15 years (66.7%) vs. Age >15 years (11.6%) (P=0.0005). At a median follow-up of 12 years, no patient died of LCH. The 5, 10-year survival estimates were 100%. The 5, 10-year disease-free survival estimates were 70.9% and 58.4%. The 5-year disease-free survival estimate was 58.3% for age ( 15 years vs. 74.4% for age >15 years (P=0.83) and 75% for single-system disease vs. 60% for multisystem disease (P=0.13). LCH was reactivated in 43.6% of patients, with a median of 14 months (range, 2-180 months). Three patients with recurrent disease experienced spontaneous remission. At the time of the most recent follow-up, 23.6% of survivors had active disease. Conclusion: LCH is not found exclusively in children and adolescents. The frequency of bone invasion is inversely related to age. Reactivation is very common regardless of the type of treatment, but the prognosis is generally good.

  15. Diffuse Large B Cell Lymphoma Mimicking Granulomatosis with Polyangiitis

    Directory of Open Access Journals (Sweden)

    Mohammad E. Naffaa

    2016-01-01

    Full Text Available In a patient with systemic multiorgan disease with overlapping features, the differential diagnosis included infectious diseases, malignancies, and systemic autoimmune or inflammatory diseases. We present an unusual case of a young male with B cell lymphoma who presented with symptoms mimicking systemic vasculitis and review the existing literature.

  16. Diffuse Large B Cell Lymphoma Mimicking Granulomatosis with Polyangiitis

    Science.gov (United States)

    Horowitz, Netanel; Ben-Itzhak, Ofer; Braun-Moscovici, Yolanda

    2016-01-01

    In a patient with systemic multiorgan disease with overlapping features, the differential diagnosis included infectious diseases, malignancies, and systemic autoimmune or inflammatory diseases. We present an unusual case of a young male with B cell lymphoma who presented with symptoms mimicking systemic vasculitis and review the existing literature. PMID:27293945

  17. Langerhans cell histiocytosis with multifocal sites. A case report; Histiocitose de celulas de Langerhans com acometimento multifocal. Relato de um caso

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    Albuquerque, Silvio Cavalcanti; Silveira, Andre; Ribeiro Neto, Joao Vicente [Instituto Materno Infantil de Pernambuco (IMIP), Recife (Brazil)

    1998-05-01

    The authors report a case of Langerhans cell histiocytosis in which the patient, at the time of the diagnosis, had lesions at the central nervous system, mediastinum and long bones, all detectable in simple roentgenogram. The recent reclassification of this disease is discussed together with it`s radiological findings. (author) 4 refs., 6 figs.

  18. Multifocal Langerhans cell histiocytosis in an adult with a pathological fracture of the mandible and spontaneous malunion: A case report

    Science.gov (United States)

    SHI, SAILANG; LIU, YANMING; FU, TAO; LI, XIUZHEN; ZHAO, SHIFANG

    2014-01-01

    Langerhans cell histiocytosis (LCH) is rare in the adult population and even rarer with jaw involvement. The current study presents the case of a 39-year-old male who complained of recurrent pain, swelling of the gingiva and an occasional pus-like discharge in the right mandible for one year. The patient was previously prescribed antibiotics, but this did not resolve the problem. An initial panoramic radiograph showed an osteolytic lesion and bone fracture in the right mandible. Eight months later, a new radiograph showed the spontaneous malunion of the fractured mandible. The patient was eventually diagnosed with Langerhans cell histiocytosis by histopathology and immunohistochemistry. Further lesions were found in the ribs and ilium by nuclear bone scanning. The patient was subsequently treated with systemic chemotherapy, and the lesions are currently effectively being controlled. This study is the first to show that spontaneous intralesional bone regeneration may lead to reunification of the mandible fracture caused by LCH in an adult. PMID:25120660

  19. Multifocal Langerhans cell histiocytosis of bone: Indications for radiotherapy; Histiocytose langerhansienne osseuse multifocale: place de la radiotherapie

    Energy Technology Data Exchange (ETDEWEB)

    Gaundong Mbethe, G.L.; Dejean, C.; Henriques de Figueiredo, B.; Sargos, P.; Italiano, A.; Kantor, G. [Service de radiotherapie, centre regional de lutte contre le cancer, institut Bergonie, 33 - Bordeaux (France)

    2010-12-15

    Langerhans cell histiocytosis is a non-malignant proliferative disease of unknown etiology that can affect one or more organs. This is a rare disease, 1 to 2/100, 000, affecting mainly children with a male predominance. The osseous lesions are the most frequent (60 to 90%). There is however no consensus treatment for the management of these sites. We report the cases of two patients successfully treated with radiotherapy after primary chemotherapy, at doses of 15 Gy in ten sessions of 1.5 Gy for one patient and 18 Gy in ten fractions of 1.8 Gy for the other. Single or multifocal bone Langerhans cell histiocytosis without visceral involvement is a benign, self-limiting affection in most cases. Some bone lesions could be treated by radiotherapy alone. But the high variability of doses currently given in the literature does not allow determining the lowest effective dose limiting the risk of secondary neoplasia or impaired growth in children, in whom lower doses of 6 to 8 Gy are recommended. The decision of radiotherapy must be weighed against the risk of the disease. Caution should be the rule in this non-malignant tumour pathology. (authors)

  20. Localized Langerhans cell histiocytosis masquerading as Brodie's abscess in a 2-year-old child: a case report

    Science.gov (United States)

    Chang, Wei-Fang; Hsu, Yi-Chih; Wu, Yi-Der; Kuo, Chun-Lang; Huang, Guo-Shu

    2016-01-01

    Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, refers to a spectrum of diseases characterized by idiopathic proliferation of histiocytes that produce either focal (localized LCH) or systemic manifestations (Hand-Schüller-Christian disease and Letterer-Siwe disease). Localized LCH accounts for approximately 60-70 % of all LCH cases. Osseous involvement is the most common manifestation and typically involves the flat bones, along with lesions of the skull, pelvis, and ribs. Localized LCH in bone shows a wide spectrum of clinical manifestations and radiologic features that may mimic those of infections as well as benign and malignant tumors. The diagnostic imaging findings of localized LCH are also diverse and challenging. The penumbra sign is a common and characteristic magnetic resonance imaging (MRI) feature of Brodie's abscess, but is rarely seen in localized LCH. In this report, we describe a case of localized LCH misdiagnosed as Brodie's abscess in a 2-year-old child based on clinical symptoms, laboratory findings, and pre-diagnostic MRI findings (penumbra sign). Therefore, the penumbra sign is not sufficient to clearly establish the diagnosis of Brodie's abscess, and the differential diagnosis of localized LCH should be considered when a child with an osteolytic lesion presents with a penumbra sign. PMID:27065773

  1. Localized Langerhans cell histiocytosis masquerading as Brodie's abscess in a 2-year-old child: a case report.

    Science.gov (United States)

    Chang, Wei-Fang; Hsu, Yi-Chih; Wu, Yi-Der; Kuo, Chun-Lang; Huang, Guo-Shu

    2016-01-01

    Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, refers to a spectrum of diseases characterized by idiopathic proliferation of histiocytes that produce either focal (localized LCH) or systemic manifestations (Hand-Schüller-Christian disease and Letterer-Siwe disease). Localized LCH accounts for approximately 60-70 % of all LCH cases. Osseous involvement is the most common manifestation and typically involves the flat bones, along with lesions of the skull, pelvis, and ribs. Localized LCH in bone shows a wide spectrum of clinical manifestations and radiologic features that may mimic those of infections as well as benign and malignant tumors. The diagnostic imaging findings of localized LCH are also diverse and challenging. The penumbra sign is a common and characteristic magnetic resonance imaging (MRI) feature of Brodie's abscess, but is rarely seen in localized LCH. In this report, we describe a case of localized LCH misdiagnosed as Brodie's abscess in a 2-year-old child based on clinical symptoms, laboratory findings, and pre-diagnostic MRI findings (penumbra sign). Therefore, the penumbra sign is not sufficient to clearly establish the diagnosis of Brodie's abscess, and the differential diagnosis of localized LCH should be considered when a child with an osteolytic lesion presents with a penumbra sign.

  2. A case of invasive Langerhans cell histiocytosis localizing only in the lung and diagnosed as pneumothorax in an adolescent female

    Science.gov (United States)

    Dejima, Hitoshi; Morita, Shigeki; Takahashi, Yusuke; Matsutani, Noriyuki; Iinuma, Hisae; Kondo, Fukuo; Kawamura, Masafumi

    2015-01-01

    In infants, Langerhans cell histiocytosis (LCH) is associated with poor clinical outcomes as Langerhans cells invade and damage multiple organs, a presentation that is different from that in adults. Here, we present a case of a 15-year-old female who visited ourclinic complaining of right chest pain and dyspnea. She was diagnosed with right pneumothorax by chest X-ray. Chest computed tomography showed multiple cystic changes in the bilateral lung. Additionally, bullous lesions occupying the upper lobe and multiple white tiny nodules on the surface of the lung were observed by thoracoscopy. These nodules comprised proliferating atypical CD1a/S-100-positive cells invading the pulmonary parenchyma, leading to the diagnosis of LCH. Because of the extensive invasion into the pulmonary parenchyma, chemotherapy was administered. This case of LCH was unique in that the age of onset was atypical and the tumor cells occupied a single organ, despite their malignant behavior. PMID:26045867

  3. From normal to abnormal MR findings within three weeks in a solitary pelvic Langerhans histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Hung, Po-Cheng [Division of Pediatric Neurology, Chang Gung Children' s Hospital, College of Medicine Chang Gung University, Taoyuan (Taiwan); Division of Pediatric Neurology, Chang Gung Children' s Hospital, 5 Fu-Hsing Street, Kwei-Shan, Taoyuan (Taiwan); Wang, Huei-Shyong [Division of Pediatric Neurology, Chang Gung Children' s Hospital, College of Medicine Chang Gung University, Taoyuan (Taiwan); Jaing, Tang-Her [Division of Pediatric Hematology and Oncology, Chang Gung Children' s Hospital, College of Medicine Chang Gung University, Taoyuan (Taiwan); Huang, Tsung-Jen [Division of Orthopediatrics, Chang Gung Children' s Hospital, College of Medicine Chang Gung University, Taoyuan (Taiwan); Pang, Leou-Chuan [Division of Pathology, Chang Gung Children' s Hospital, College of Medicine Chang Gung University, Taoyuan (Taiwan)

    2003-08-01

    We present a histologically proven case of pelvic Langerhans histiocytosis (eosinophilic granuloma) which showed abnormality on MR imaging 3 weeks after a normal MR examination mimicking an infection. (orig.)

  4. Coincident expression of the chemokine receptors CCR6 and CCR7 by pathologic Langerhans cells in Langerhans cell histiocytosis.

    Science.gov (United States)

    Fleming, Mark D; Pinkus, Jack L; Fournier, Marcia V; Alexander, Sarah W; Tam, Carmen; Loda, Massimo; Sallan, Stephen E; Nichols, Kim E; Carpentieri, David F; Pinkus, Geraldine S; Rollins, Barrett J

    2003-04-01

    It has been suggested that a switch in chemokine receptor expression underlies Langerhans cell migration from skin to lymphoid tissue. Activated cells are thought to down-regulate CCR6, whose ligand macrophage inflammatory protein-3 alpha (MIP-3 alpha)/CCL20 is expressed in skin, and up-regulate CCR7, whose ligands are in lymphoid tissues. In Langerhans cell histiocytosis (LCH), pathologic Langerhans cells (LCs) accumulate in several tissues, including skin, bone, and lymphoid organs. We have examined 24 LCH cases and find that pathologic LCs expressed CCR6 and CCR7 coincidentally in all cases. Furthermore, MIP-3 alpha/CCL20 is expressed by keratinocytes in involved skin and by macrophages and osteoblasts in involved bone. Expression of CCR6 by pathologic LCs may contribute to their accumulation in nonlymphoid organs such as skin and bone, whereas CCR7 expression may direct them to lymphoid tissue. Histiocytes in Rosai-Dorfman disease and hemophagocytic syndrome also coexpressed CCR6 and CCR7, suggesting that this may be a general attribute of abnormal histiocytes.

  5. A rare case of solitary brain Langerhans cell histiocytosis with intratumoral hemorrhage in a patient affected by Turner syndrome

    Science.gov (United States)

    Granata, Francesca; Morabito, Rosa; Grasso, Giovanni; Alafaci, Elisabetta; Salpietro, Francesco M.; Alafaci, Concetta

    2016-01-01

    Background: Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of cells with characteristics similar to bone marrow-derived Langerhans cells. The case of a young woman, affected by Turner syndrome and a solitary intraparenchymal LCH associated with an osteolytic lesion of the overlying skull, is presented. Case Description: The patient, with an insidious history of headache and a growing soft mass in the left frontal region, presented with a sudden generalized tonic-clonic epileptic seizure. Neuroradiological investigations showed an osteolytic lesion of the left frontal bone and an underlying brain lesion associated with recent signs of bleeding. The patient was operated on with a complete removal of the lesion. The postoperative course was uneventful. Conclusions: The clinical, neuroradiological, and intraoperative findings are presented, along with a review of the literature. Although rare, LCH should be considered in the differential diagnosis when a scalp lesion occurs with a progressive growing. PMID:27127696

  6. Eosinophilic granuloma of bone and biochemical demonstration of 49-kDa CD1a molecule expression by Langerhans-cell histiocytosis.

    Science.gov (United States)

    Cambazard, F; Dezutter-Dambuyant, C; Staquet, M J; Schmitt, D; Thivolet, J

    1991-09-01

    Histiocytic cells infiltrating the lesions in eosinophilic granuloma of bone as well as in cutaneous histiocytosis X were studied using a murine monoclonal antibody (MA) produced with proliferating cells from an eosinophilic granuloma of bone. This MA reacts with Langerhans cells (LC) of normal human skin or mucous membranes and with proliferating cells of eosinophilic granuloma of bone and skin lesions of Letter-Siwe disease, as shown by immunohistochemistry and immunogold labelling. As other murine MA's obtained after immunization with human cortical thymocytes, this MA immunoprecipitates the 49-kDa CD1a antigen found on human LC and thymic-cell surfaces but not its breakdown product after treatment with trypsin, as demonstrated by analysis of immunoelectron labelling, cytofluorometry and gel electrophoresis. This first production of a CD1a MA from an eosinophilic granuloma supports the concept of Langerhans-cell histiocytosis.

  7. Adult Onset of BRAFV600E-Mutated Langerhans Cell Histiocytosis with Cutaneous Involvement Successfully Diagnosed by Immunohistochemical Staining

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    Tono, Hisayuki; Fujimura, Taku; Kakizaki, Aya; Furudate, Sadanori; Ishibashi, Masaya; Aiba, Setsuya

    2015-01-01

    Langerhans cell histiocytosis (LCH) is characterized by the clonal proliferation of Langerhans cells; it is categorized as a single-system disease with single or multifocal lesions, and as a multi-system disease with or without the risk of organ involvement. Although the skin is not categorized as a risk organ, the precise diagnosis of skin lesions is necessary to determine the protocol for the treatment of LCH. In this report, we describe a 28-year-old Japanese man with adult onset of BRAFV600E-mutated LCH with cutaneous involvement successfully diagnosed by immunohistochemical staining. Our report suggests that immunohistochemical staining for the BRAFV600E gene could be a diagnostic tool to determine the clinical type of LCH. PMID:26500535

  8. Adult Onset of BRAFV600E-Mutated Langerhans Cell Histiocytosis with Cutaneous Involvement Successfully Diagnosed by Immunohistochemical Staining

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    Hisayuki Tono

    2015-09-01

    Full Text Available Langerhans cell histiocytosis (LCH is characterized by the clonal proliferation of Langerhans cells; it is categorized as a single-system disease with single or multifocal lesions, and as a multi-system disease with or without the risk of organ involvement. Although the skin is not categorized as a risk organ, the precise diagnosis of skin lesions is necessary to determine the protocol for the treatment of LCH. In this report, we describe a 28-year-old Japanese man with adult onset of BRAFV600E-mutated LCH with cutaneous involvement successfully diagnosed by immunohistochemical staining. Our report suggests that immunohistochemical staining for the BRAFV600E gene could be a diagnostic tool to determine the clinical type of LCH.

  9. Langerhans′ cell histiocytosis involving posterior elements of the dorsal spine: An unusual cause of extradural spinal mass in an adult

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    Devendra K Tyagi

    2011-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a clonal proliferation of Langerhans cells occurring as an isolated lesion or as part of a systemic proliferation. It is commoner in children younger than 10 years of age with sparing of the posterior elements in more than 95% of cases. We describe a case of LCH in an adult female presenting with paraplegia. MRI revealed a well-defined extradural contrast enhancing mass at D2-D4 vertebral level involving the posterior elements of spine. D2-5 laminectomy with excision of lesion was performed which lead to marked improvement of patients neurological status. Histopathology was suggestive of eosinophilic granuloma. We describe the case, discuss its uniqueness and review the literature on this rare tumor presentation.

  10. Langerhans' cell histiocytosis involving posterior elements of the dorsal spine: An unusual cause of extradural spinal mass in an adult.

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    Tyagi, Devendra K; Balasubramaniam, Srikant; Savant, Hemant V

    2011-07-01

    Langerhans cell histiocytosis (LCH) is a clonal proliferation of Langerhans cells occurring as an isolated lesion or as part of a systemic proliferation. It is commoner in children younger than 10 years of age with sparing of the posterior elements in more than 95% of cases. We describe a case of LCH in an adult female presenting with paraplegia. MRI revealed a well-defined extradural contrast enhancing mass at D2-D4 vertebral level involving the posterior elements of spine. D2-5 laminectomy with excision of lesion was performed which lead to marked improvement of patients neurological status. Histopathology was suggestive of eosinophilic granuloma. We describe the case, discuss its uniqueness and review the literature on this rare tumor presentation.

  11. A case of adult Langerhans cell histiocytosis showing successfully regenerated osseous tissue of the skull after chemotherapy.

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    Suzuki, Takahiro; Izutsu, Koji; Kako, Shinichi; Ohta, Satoshi; Hangaishi, Akira; Kanda, Yoshinobu; Motokura, Toru; Chiba, Shigeru; Kurokawa, Mineo

    2008-04-01

    Langerhans cell histiocytosis (LCH) is a proliferative disorder of Langerhans cells and extremely rare in adults. Adult LCH is often associated with osteolytic bone lesions, but large bone-defective lesions have been rarely reported. We report an adult case of LCH accompanied by large osteolytic lesions in the skull that successfully responded to chemotherapy. A 47-year-old woman with LCH who had multiple, large osteolytic areas of more than 3 cm in diameter in the skull was admitted to our hospital. She was treated with systemic chemotherapy consisting of prednisolone, vinblastine, and 6-mercaptopurine. Twelve months later, when she completed the treatment, osteolytic areas were covered with hard osseous tissue, and X-ray examination confirmed regeneration of the bone. This case indicates that chemotherapy can be effective even for the treatment of large osteolytic lesions in adult LCH patients.

  12. Angioimmunoblastic T Cell Lymphoma Mimicking Chronic Urticaria

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    Mohleen Kang

    2016-01-01

    Full Text Available Angioimmunoblastic T cell lymphoma (AITL is a rare but distinct type of T cell lymphoma with an aggressive course and high mortality. Most patients are diagnosed late in the disease and usually present with generalized lymphadenopathy. A minority have skin lesions at the time of diagnosis, more commonly in the form of nonspecific maculopapular rash with or without pruritus. We report a rare case of AITL presenting with chronic, recurrent angioedema and urticaria-like lesions and no palpable peripheral adenopathy. Primary Care physicians, dermatologists, and allergists must maintain a high index of suspicion for cutaneous manifestations of lymphoma, especially if the skin lesions are refractory to standard treatment. Timely diagnosis is essential to improve survival.

  13. 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

  14. 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.

  15. Lyme carditis mimicking giant cell arteritis

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    Krati Chauhan

    2015-10-01

    Full Text Available Presenting an interesting case of a patient who complained of myalgias, fatigue, headache, jaw claudication and scalp tenderness. Patient’s physical examination was unremarkable. Laboratory findings showed elevated erythrocyte sedimentation rate and C-reactive protein, bilateral temporal artery biopsy results were negative and first degree atrioventricular block was seen on electrocardiogram. Serology for Borrelia burgdorferi was positive; patient was diagnosed with Lyme carditis and treated with doxycycline. Lyme is a tick-borne, multi-system disease and occasionally its presentation may mimic giant cell arteritis. On follow-up there was complete resolution of symptoms and electrocardiogram findings.

  16. Intracranial germ cell tumor mimicking anorexia nervosa.

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    Andreu Martínez, F J; Martínez Mateu, J M

    2006-12-01

    We report on a case of a 23 year-old female diagnosed as having a germ-cell tumour located in the sellar region. The patient referred anorexia, psychic disorders, weight loss of 15 kilograms and secondary amenorrhea during the previous three years. This is the reason why the patient was diagnosed as having anorexia nervosa. Subsequently, the patient presented some endocrine dysfunction. MRI revealed the existence of a lesion located in suprasellar and hypothalamic regions. This case shows that the presence of intracranial tumours next to the hypothalamus must be borne in mind as a rare but real possibility in cases of anorexia nervosa, specially in those non-typical cases.

  17. Granuloma inguinale mimicking as squamous cell carcinoma of penis

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    Abhishek Pilani

    2014-01-01

    Full Text Available Granuloma inguinale (GI is an acquired chronic, slowly progressive, mildly contagious disease of venereal origin, characterized by granulomatous ulceration of the genitalia and neighboring sites, with little or no tendency to spontaneous healing caused by Klebsiella (Calymmatobacterium granulomatis. A 55-year-old male presented with fissured, foul smelling, fungating growth over prepuce with phimosis mimicking squamous cell carcinoma (SCC without lymphadenopathy. It started with painless papulonodular showed pseudoepitheliomatous hyperplasia, infiltration in dermis, acanthosis and vacuolated macrophages suggestive of GI and not showing any histopathological features of SCC. Patient was successfully treated by giving cotrimoxazole twice a day for 21 days. Here, we presented a case of GI mimicking SCC of penis, which was diagnosed on basis of histopathology and treated with excision followed by medical therapy with cotrimoxazole.

  18. Spontaneous extradural hemorrhage due to Langerhans cell histiocytosis of the skull in a child: A rare presentation

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    Bakhaidar, Mohamad G.; Alghamdi, Fahad A.; Baeesa, Saleh S.

    2016-01-01

    Eosinophilic granuloma (EG) represents a local form of Langerhans cell histiocytosis that occurs mostly in children. It usually presents with a gradually enlarging painless skull mass, and rarely presents a rapid clinical deterioration. This 7-year-old boy who was diagnosed with EG, based on a magnetic resonance imaging scan, after presenting with a painless right parietal swelling of 7-week duration. Three weeks prior his scheduled surgery, he presented to the emergency department with a 2-day history of sudden increased of the subcutaneous swelling associated with a headache, vomiting, and decreased the level of consciousness; there was no history of trauma. Brain computed tomography revealed a right parietal bone defect with large subgaleal and extradural hematoma. He underwent emergent surgical excision of the skull lesion and evacuation of the hematoma. Histopathological examination confirmed the diagnosis of EG. We aim to raise the awareness of physicians of this rare spontaneous hemorrhagic complication of EG and review the literature. PMID:27195034

  19. A Case Report: The Diagnosis and Therapeutic Evaluation for a Rare Disease of Langerhans Cell Histiocytosis Involving Thyroid

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    Cai, Ye-Feng; Wang, Qing-Xuan; Ni, Chun-Jue; Dong, Si-Yang; Lv, Lin; Li, Quan; Chen, En-Dong; Zhang, Xiao-Hua

    2015-01-01

    Abstract Langerhans cell histiocytosis (LCH) involving the thyroid gland is extremely rare. Currently, the diagnosis and therapeutic evaluation for LCH involving thyroid is a challenge. We reported a rare case of LCH involving thyroid, presenting as painless thyroid goiters, and successfully performed positron emission tomography/computed tomography (PET/CT) to make an accurate diagnosis and therapeutic evaluation for LCH. Although the histology or cytology is the golden standard for the diagnosis of LCH involving thyroid, the PET/CT should be keep in mind when LCH involving thyroid with inconclusive cytologic results. During the treatment of LCH, PET/CT can be performed to assess the therapeutic effect and select the most effective and reliable treatment for LCH. PMID:26554785

  20. 朗格汉斯细胞组织细胞增多症研究进展%RESEARCH PROGRESS OF LANGERHANS CELL HISTIOCYTOSIS

    Institute of Scientific and Technical Information of China (English)

    王海燕; 侯梅

    2011-01-01

    Langerhans cell histiocytosis is a rare histiocytic disorder, the pathogenesis of which is not clear. It has various biological behaviors and the clinical course is complex, as well as non-specific, so the diagnosis is often delayed or mistakened. We present a review of Langerhans cell histiocytosis , highlighting new insights into pathogenesis, diagnosis and management of the disease. '%朗格汉斯细胞组织细胞增多症是一种少见的组织细胞异常性疾病,其发病机制不清,生物学行为多样,临床表现相对复杂,并且缺乏特异性,极易延迟诊断及误诊.本文就朗格汉斯细胞组织细胞增多症的发病机制、诊断与治疗作一综述.

  1. Langerhans cell histiocytosis or tuberculosis on a medieval child (Oppidum de la Granède, Millau, France - 10th-11th centuries AD).

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    Colombo, Antony; Saint-Pierre, Christophe; Naji, Stephan; Panuel, Michel; Coqueugniot, Hélène; Dutour, Olivier

    2015-06-01

    In 2008, a skeleton of a 1 - 2.5-year-old child radiocarbon dated from the 10th - 11th century AD was discovered on the oppidum of La Granède (Millau, France). It presents multiple cranial osteolytic lesions having punched-out or geographical map-like aspects associated with sequestrum and costal osteitis. A multi 3D digital approach (CT, μCT and virtual reconstruction) enabled us to refine the description and identify the diploic origin of the lytic process. Furthermore, precise observation of the extent of the lesions and associated reorganization of the skeletal micro-structure were possible. From these convergent pieces of evidence, the differential diagnosis led to three possibilities: Langerhans cell histiocytosis, tuberculosis, or Langerhans cell histiocytosis and tuberculosis.

  2. Insufficiency of Bone Scintigraphy in Vertebral Lesions of Langerhans Cell Histiocytosis Compared to F-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography and Diagnostic Computed Tomography

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    Koç, Zehra Pınar; Şimşek, Selçuk; Akarsu, Saadet; Balcı, Tansel Ansal; Onur, Mehmet Ruhi; Kepenek, Ferat

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a benign disorder related to the histiocytes which can infiltrate bone tissue. The most effective method for demonstrating severity of this disease is PET/CT and bone scintigraphy might show bone lesions. We present a seventeen year old male patient with disseminated LCH presented with exophtalmos and having multiple vertebral lesions which were identified by F-18 FDG PET/CT scan and diagnostic CT but not in the bone scintigraphy. PMID:25800594

  3. Insufficiency of Bone Scintigraphy in Vertebral Lesions of Langerhans Cell Histiocytosis Compared to F-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography and Diagnostic Computed Tomography

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    Zehra Pınar Koç

    2015-02-01

    Full Text Available Langerhans cell histiocytosis (LCH is a benign disorder related to the histiocytes which can infiltrate bone tissue. The most effective method for demonstrating severity of this disease is PET/CT and bone scintigraphy might show bone lesions. We present a seventeen year old male patient with disseminated LCH presented with exophtalmos and having multiple vertebral lesions which were identified by F-18 FDG PET/CT scan and diagnostic CT but not in the bone scintigraphy

  4. Langerhans cell histiocytosis is a neoplasm and consequently its recurrence is a relapse: In memory of Bob Arceci.

    Science.gov (United States)

    Egeler, R Maarten; Katewa, Satyendra; Leenen, Pieter J M; Beverley, Peter; Collin, Matthew; Ginhoux, Florent; Arceci, Robert J; Rollins, Barrett J

    2016-10-01

    Langerhans cell histiocytosis (LCH) remains a poorly understood disorder with heterogeneous clinical presentations characterized by focal or disseminated lesions that contain excessive CD1a+ langerin+ cells with dendritic cell features known as "LCH cells." Two of the major questions investigated over the past century have been (i) the origin of LCH cells and (ii) whether LCH is primarily an immune dysregulatory disorder or a neoplasm. Current opinion is that LCH cells are likely to arise from hematopoietic precursor cells, although the stage of derailment and site of transformation remain unclear and may vary in patients with different extent of disease. Over the years, evidence has provided the view that LCH is a neoplasm. The demonstration of clonality of LCH cells, insufficient evidence alone for neoplasia, is now bolstered by finding driver somatic mutations in BRAF in up to 55% of patients with LCH, and activation of the RAS-RAF-MEK-ERK (where MEK and ERK are mitogen-activated protein kinase and extracellular signal-regulated kinase, respectively) pathway in nearly 100% of patients with LCH. Herein, we review the evidence that recurrent genetic abnormalities characterized by activating oncogenic mutations should satisfy prerequisites for LCH to be called a neoplasm. As a consequence, recurrent episodes of LCH should be considered relapsed disease rather than disease reactivation. Mapping the complete genetic landscape of this intriguing disease will provide additional support for the conclusion that LCH is a neoplasm and is likely to provide more potential opportunities for molecularly targeted therapies.

  5. Dentinogenic Ghost Cell Tumor of the Peripheral Variant Mimicking Epulis

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    Uddipan Kumar

    2010-01-01

    Full Text Available Dentinogenic ghost cell tumor (DGCT is an uncommon locally invasive odontogenic tumor regarded by many as a variant of calcifying odontogenic cyst. The peripheral variant of this clinical rarity appears as a well-circumscribed mass mimicking a nonspecific gingival enlargement. Microscopic appearance of odontogenic epithelium admixed with focal areas of dentinoid formation and sheets of ghost cells giving the definitive diagnosis of dentinogenic ghost cell tumor imply that microscopic examination is compulsory for any gingival mass. Van Gieson histochemical stain further confirmed the nature of dentinoid-like material. A complete workup of a case of peripheral dentinogenic ghost cell tumor is presented in this paper and the current concept as well as the appraisal of literature is presented.

  6. Pulmonary langerhans cell histiocytosis in adults: high-resolution CT - pathology comparisons and evolutional changes at CT

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    Kim, Hyo Jin; Lee, Ho Yun; Kim, Tae Sung [Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Samsung Medical Center, Seoul (Korea, Republic of); Lee, Kyung Soo [Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Samsung Medical Center, Seoul (Korea, Republic of); Samsung Medical Center, Department of Radiology, Seoul (Korea, Republic of); Johkoh, Takeshi [Kinki Central Hospital of Mutual Aid Association of Public School Teachers, Department of Radiology, Hyoko (Japan); Tomiyama, Noriyuki [Osaka University Graduate School of Medicine, Department of Radiology, Osaka (Japan); Han, Joungho [Sungkyunkwan University School of Medicine, Department of Pathology, Samsung Medical Center, Seoul (Korea, Republic of)

    2011-07-15

    To compare high-resolution (HR) CT and histopathological findings and to evaluate serial CT findings in pulmonary Langerhans cell histiocytosis (PLCH). We reviewed CT of lung lesions in 27 adults (M:F = 20:7, mean age, 41 {+-} 12.3 years) with PLCH. After evaluating lung abnormalities including nodules, micronodules, thick-walled, thin-walled, and bizarre-shaped cysts and reticulation, observers compared CT findings obtained at lung biopsy sites with histopathological findings. The final CT was compared with the initial CT to determine disease extent changes. The most frequently observed patterns of lung abnormalities were micronodules (n = 24, 89%), thick-walled (n = 22, 82%), and thin-walled (n = 22, 82%) cysts. Even thin-walled and bizarre cysts harboured active inflammatory Langerhans cell sheets and eosinophils in their walls. In thin-walled cysts, we noted pericystic inflammatory cell infiltrations along the alveolar walls, as well as pericystic emphysema. Thin-walled or bizarre cysts demonstrated a tendency to coalesce with surrounding cysts via their cystic wall destruction. Fourteen (52%) patients showed improvement and nine (33%) showed progressing disease. More than half of patients with pulmonary PLCH show improvement at follow-up CT. Even thin-walled cysts harbour active inflammatory cells on histopathology and exhibit improvement at follow-up CT. (orig.)

  7. Histiocytosis: a review focusing on neuroimaging findings

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    Larissa Barcessat Gabbay

    2014-07-01

    Full Text Available Objective: Histiocytosis is a systemic disease that usually affects the central nervous system. The aim of this study is to discuss the neuroimaging characteristics of Langerhans cell histiocytosis (LCH, the most common of these diseases; and the non-Langerhans cells histiocytosis (NLCH, which includes entities such as hemophagocytic syndrome, Erdheim-Chester and Rosai-Dorfman diseases. Method: Literature review and illustrative cases with pathologic confirmation. Results: In LCH, the most common findings are 1 osseous lesions in the craniofacial bones and/or skull base; 2 intracranial, extra-axial changes; 3 intra-axial parenchymal changes (white and gray matter; 4 atrophy. Among the NLCH, diagnosis usually requires correlation with clinical and laboratory criteria. The spectrum of presentation includes intraparenchymal involvement, meningeal lesions, orbits and paranasal sinus involvement. Conclusion: It is important the recognition of the most common imaging patterns, in order to include LCH and NLCH in the differential diagnosis, whenever pertinent.

  8. Verrucous tumor mimicking squamous cell carcinoma in immunocompetent patient.

    Science.gov (United States)

    Ruiz-Villaverde, Ricardo; Sanchez-Cano, Daniel; Martinez-Peinado, Carmen M; Galan-Gutierrez, Manuel

    2016-02-17

    Mycobacteria cause a range of diseases in both immunocompetent and immunosuppressed individuals. An increase in non-tuberculous mycobacterial (NTM) infections targeting skin has been described. Many hypotheses have been developed in order to explain it: the increasing burden of immunocompromised individuals, immigration from endemic countries, improved laboratory identification techniques, and changes inhuman behavior that expose individuals to this NTM. Mycobacterium mucogenicum group comprises M. mucogenicum, Mycobacterium aubagnense, and Mycobacterium phocaicum. This group of organisms was first named Mycobacterium chelonae-like organism in 1982. Most clinically significant cases of those organisms involved catheter-related infections. Nevertheless, we report an interesting patient with a cutaneous infection produced by M. mucogenicum mimicking a squamous cell carcinoma; an excellent response to combined therapy with rifampicin and clarythromicin was observed.

  9. Utility of spiral CAT scan in the follow-up of patients with pulmonary Langerhans cell histiocytosis

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    Abbritti, M. [Respiratory Diseases Section, Dept. Clinical Medicine and Immunological Sciences, Siena University (Italy); Mazzei, M.A. [Dept. Human Pathology and Oncology, Section of Radiological Sciences, Siena University, Siena (Italy); Bargagli, E., E-mail: bargagli2@gmail.com [Respiratory Diseases Section, Dept. Clinical Medicine and Immunological Sciences, Siena University (Italy); Refini, R.M.; Penza, F.; Perari, M.G. [Respiratory Diseases Section, Dept. Clinical Medicine and Immunological Sciences, Siena University (Italy); Volterrani, L. [Dept. Human Pathology and Oncology, Section of Radiological Sciences, Siena University, Siena (Italy); Rottoli, P. [Respiratory Diseases Section, Dept. Clinical Medicine and Immunological Sciences, Siena University (Italy)

    2012-08-15

    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease that occurs almost exclusively in smokers, generally young adults between 20 and 40 years old. Prognostic biomarkers of the disease are lacking. This study describes the clinical-radiological features of a group of PLCH patients and applies a semi-quantitative CT score of the chest to verify the prognostic value of radiological findings in this disease. Clinical-radiological and immunological data from 12 Caucasian patients (6 M, 7 smokers and 5 ex-smokers, mean age 36 {+-} 8 years) were recorded at onset and after a follow-up period of 4 years. Application of the semi-quantitative CT score revealed a prevalently cystic pattern at onset and follow-up in the majority of the patients. Patients with a prevalently nodular pattern developed cystic lesions during follow-up. Interestingly, significant correlations were found between the extent of cystic lesions and DLCO values at onset (time 0: p < 0.05) and at the end of follow-up (time 1, p < 0.05) and with FEV1 values at time 0 (p < 0.05) and time 1 (p < 0.05). Patients with progressive functional decline were those with CT evidence of severe cystic alterations. The results suggest that high resolution CT scan of the chest is mandatory for characterizing PLCH patients at diagnosis and during follow-up. The proposed CT score of the chest showed potential prognostic value.

  10. The adverse prognostic hallmarks in identical twins with Langerhans cell histiocytosis: a clinical report and literature review.

    Science.gov (United States)

    Chai, Damin; Tao, Yisheng; Bao, Zhengqi; Yang, Li; Feng, Zhenzhong; Ma, Li; Liang, Limei; Zhou, Xinwen

    2013-01-01

    Langerhans cell histiocytosis (LCH) is characterized by uncontrolled proliferation of Langerhans cells accompanying eosinophils. It often attacks children under 10 years of age. LCH in identical twins is very rare and its prognosis is different. Here we report identical-twin sisters with LCH. Computed tomography (CT) revealed osteolytic change in each twin's skull, and the elder exhibited poor eyesight. There were massive histiocyte-like cells surrounded by eosinophils in pathologic specimen of the abnormal lesions, which is typical pathologic finding in LCH. These pathologic cells were positive for S-100 and the cell surface protein CD1 antigen (CD1α), the known markers of LCH. After treating them with surgery, no symptoms were seen in the younger until now. While the older was found another soft mass (about 2.0 cm in diameter) in the left temporal area 18 months later. The same treatment was given to the older after admission, and she is healthy to date. To explore the relationship between hallmarks and the prognosis of identical-twin patients with LCH, we retrieved the 16 literatures (16 identical-twin pairs, 31 patients) listed in PubMed during the past 60 years. The data revealed all those patients who have disseminated to the bone marrow, spleen and liver with symptoms of fever and hepatosplenomegaly exhibited worse prognosis (9 out of the 31 patients). The other identical-twin subjects without infiltration of those organs recovered well. In conclusion, this study reveals the adverse hallmarks of prognosis in identical-twin patients with LCH by reviewing relevant literatures.

  11. Common Acute Lymphoblastic Leukemia Ph+ Following Langerhans Cell Histiocytosis in a Multi-Malformed Child with INV (9 (p12;q13 (mat:Case Report

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    Pavelić,Jasminka

    2010-08-01

    Full Text Available The occurrence of Langerhans cell histiocytosis (LCH and another malignancy in the same patient is infrequent but has been recognized. The genetic changes that could be responsible for LCH and/or concomitant leukemia development are obscure. To the best of our knowledge, this is the first description of constitutional maternally derived inv (9 (p12;q13 in an LCH patient, and also of the development of common ALL Ph after LCH diagnosis and therapy. The potential significance of these findings [inv (9LCHALL Ph] and their mutual relationship are unknown. Therefore, cooperative studies of large numbers of patients are needed to identify the common risk factors, if any.

  12. Langerhans Cell Histiocytosis in an Adult with Involvement of the Calvarium, Cerebral Cortex and Brainstem: Discussion of Pathophysiology and Rationale for the Use of Intravenous Immune Globulin

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    Dardis, Christopher; Aung, Thandar; Shapiro, William; Fortune, John; Coons, Stephen

    2015-01-01

    We report a case of Langerhans cell histiocytosis in a 64-year-old male who presented with symptoms and signs of brain involvement, including seizures and hypopituitarism. The diagnosis was confirmed with a biopsy of a lytic skull lesion. The disease affecting the bone showed no sign of progression following a short course of cladribine. Signs of temporal lobe involvement led to an additional biopsy, which showed signs of nonspecific neurodegeneration and which triggered status epilepticus. Lesions noted in the brainstem were typical for the paraneoplastic inflammation reported in this condition. These lesions improved after treatment with cladribine. They remained stable while on treatment with intravenous immune globulin. PMID:25873887

  13. Langerhans Cell Histiocytosis in an Adult with Involvement of the Calvarium, Cerebral Cortex and Brainstem: Discussion of Pathophysiology and Rationale for the Use of Intravenous Immune Globulin

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    Christopher Dardis

    2015-02-01

    Full Text Available We report a case of Langerhans cell histiocytosis in a 64-year-old male who presented with symptoms and signs of brain involvement, including seizures and hypopituitarism. The diagnosis was confirmed with a biopsy of a lytic skull lesion. The disease affecting the bone showed no sign of progression following a short course of cladribine. Signs of temporal lobe involvement led to an additional biopsy, which showed signs of nonspecific neurodegeneration and which triggered status epilepticus. Lesions noted in the brainstem were typical for the paraneoplastic inflammation reported in this condition. These lesions improved after treatment with cladribine. They remained stable while on treatment with intravenous immune globulin.

  14. MRI and clinical features of Langerhans cell histiocytosis (LCH) in the pelvis and extremities: can LCH really look like anything?

    Energy Technology Data Exchange (ETDEWEB)

    Samet, Jonathan [Northwestern University Feinberg School of Medicine, Department of Medical Imaging, Ann and Robert H. Lurie Children' s Hospital of Chicago, Chicago, IL (United States); Weinstein, Joanna [Northwestern University Feinberg School of Medicine, Departments of Pediatrics, Division of Hematology/Oncology/Stem Cell Transplant, Ann and Robert H. Lurie Children' s Hospital of Chicago, Chicago, IL (United States); Fayad, Laura M. [The Johns Hopkins University School of Medicine, The Russell H. Morgan Department of Radiology and Radiological Science, Baltimore, MD (United States)

    2016-05-15

    To assess clinical and MRI features of Langerhans cell histiocytosis in the pelvis and extremities. The MRI and clinical features of 21 pathologically proven cases of LCH involving the pelvis and extremities were studied. Multiple characteristics of the lesions were evaluated (location, size, T1/ T2/post-contrast features, perilesional bone and soft tissue signal, endosteal scalloping, periosteal reaction, soft tissue mass, pathologic fracture). Pre-biopsy radiologic diagnoses were collected from the original clinical reports. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), temperature, and white blood cell count (WBC) were collected at the time of diagnosis when available. The locations of the LCH lesions included five humerus, four femur, five ilium, one tibia, one clavicle, and three scapula. Lesional size ranged from 1.8 to 7.1 cm, with a mean of 3.6 cm. All lesions demonstrated perilesional bone marrow edema, periosteal reaction, endosteal scalloping, and post-contrast enhancement. An associated soft tissue mass was present in 15/21 (71.4 %). Clinically, the WBC, ESR, and CRP were elevated in 2/14 (14 %), 8/12 (67 %), and 4/10 (40 %) of cases, respectively. Fever was documented in 1/15 (7 %) patients and pain was reported in 15/15 (100 %). The clinical and radiologic features of LCH in the pelvis and extremities overlap with infection and malignancy, but LCH must be considered in the differential diagnosis, as it routinely presents with aggressive MRI features, including endosteal scalloping, periosteal reaction, perilesional edema, and a soft tissue mass. Furthermore, an unknown skeletal lesion at presentation without aggressive MRI features is unlikely to represent LCH. (orig.)

  15. Treatment of Children with Multiple System Langerhans Cell Histiocytosis by Japan Langerhans Cell Histiocytosis Study Group Protocol%JLSG方案治疗儿童多系统朗格汉斯细胞组织细胞增生症

    Institute of Scientific and Technical Information of China (English)

    黄俊彬; 薛红漫; 陈岩岩; 黄科

    2013-01-01

    The purpose of this study was to evaluate the efficiency of Japan Langerhans Cell Histiocytosis Study Group (JLSG) Protocol in treatment of children suffering from multiple system langerhans cell histiocytosis (MS-LCH). The clinical features, therapeutic response and prognosis of 11 children who were diagnosed and treated by JLSG in our department during October 2004 through October 2011 were analyzed. Among all 11 cases, 8 males and 3 females, the age at diagnosis was from 3 month to 6.5 years old with a median age of 3 years old. There were 10 cases of LCH with multi-system involvement (MS-LCH) and 1 case of single-system involvement (SS-LCH). Among those MS-LCH patients, 5 patients had risk organ involvement, and the other 5 patients did not develop risk organ involvement. All patients had been treated with JLSG protocol. The results showed that 4 cases achieved good response after 6-week induction treatment and the time of drug discontinuation were 5-20 months without relapse; 3 cases achieved partial response after 6-week induction treatment, among them 1 case did not relapse after discontinuation of drugs for 19 months, 1 case was still receiving maintenance treatment, 1 case abandoned induction treatment; 4 patients got no response(NR) or progressive disease after 6-week of induction treatment and were switched to salvage therapy, among them, 2 patients had stopped treatment for 2-20 months without relapse, 1 patient was still receiving maintenance treatment, one had changed to another therapy. It is concluded that the most of childhood LCH can be effectively controlled by immunochemical therapy based on the JLSG protocol. For children with LCH who has a poor response after 6-week induction treatment, LCH can still be well controlled if switched to salvage treatment.%本研究观察以JLSG(Japan Langerhans Cell Histiocytosis Study Group,JLSG)方案为基础治疗儿童多系统朗格汉斯细胞组织细胞增生症(MS-LCH)的疗效.

  16. [Histiocytosis X. An oligosymptomatic form of Hand-Schüller-Christian disease with a new form and possibilities of diagnosis].

    Science.gov (United States)

    Eberth-Willershausen, W; Steger, O; Barran, W; Burg, G; Ryckmanns, F; Kaudewitz, P

    1984-10-01

    A patient is reported who had a monosymptomatic, cutaneous form of histiocytosis X. The reactivity of cutaneous histiocytosis-X cells with monoclonal anti-T-6 antibody is discussed as a new diagnostic procedure.

  17. Congenital "self-healing" Langerhans cell histiocytosis (Hashimoto-Pritzker disease): a report of two cases with the same cutaneous manifestations but different clinical course.

    Science.gov (United States)

    Mandel, Victor Desmond; Ferrari, Chiara; Cesinaro, Anna Maria; Pellacani, Giovanni; Del Forno, Corrado

    2014-12-01

    Congenital self-healing Langerhans cell histiocytosis or Hashimoto-Pritzker disease is a rare condition present at birth or in the neonatal period characterized by small reddish-brown crusted papulonodular lesions. In most cases these lesions are not accompanied by systemic findings and tend to involute spontaneously within weeks or months, but in other cases there may be extracutaneous involvement and/or recurrence of the disease. This emphasizes that the clinical course is variable and a long-term follow-up is mandatory in order to reveal possible systemic involvement. We describe two cases of congenital self-healing Langerhans cell histiocytosis with widespread and very similar cutaneous manifestations but different clinical course. The first patient had multisystemic disease (with lymph nodes, bones, liver and lungs affected) that required systemic therapy. The second patient had cutaneous and bony lesions that resolved spontaneously. We think that the adjective "self-healing" is misleading and should be abandoned. We stress the importance of a complete systemic evaluation and the necessity of a long-term follow-up.

  18. [Cutaneous histiocytosis X].

    Science.gov (United States)

    Metz, J; Metz, G; Lechner, W

    1980-09-01

    Histiocytosis X comprises three clinical entities whose common substrate is a localized or systemic proliferation of atypical histiocytes. On the basis of the age of manifestation, acuity of the clinical course and organ involvement Abt-Letterer-Siwe's disease, Hand-Schüller-Christian's disease and eosinophilic granuloma can be differentiated from each other, although transitional varieties of these syndromes are possible. Not infrequently oligosymptomatic forms are misinterpreted, especially when the skin is the only involved organ. In the following case report cutaneous histiocytosis X will be discussed in terms of its clinical expression. Electron-microscopy has proved to be the best methods to make the diagnosis of such atypical cases.

  19. Benign cephalic histiocytosis.

    Science.gov (United States)

    Gianotti, F; Caputo, R; Ermacora, E; Gianni, E

    1986-09-01

    Benign cephalic histiocytosis is a self-healing non-X, nonlipid cutaneous histiocytosis of children, characterized by a papular eruption on the head. Mucous membranes and viscera are always spared. In the 13 cases reported herein, the children were otherwise in good general health. The disease appeared during the first three years of life, and spontaneous regression was complete by the age of nine years in the four cases healed to date. The histiocytic infiltrate was localized in the upper and middle dermis and contained no lipids at any stage of evolution. All the histiocytes contained coated vesicles, and 5% to 30% also contained comma-shaped bodies in their cytoplasm.

  20. Blood Stem Cell Transplant in Treating Patients With Hematologic Cancer

    Science.gov (United States)

    2014-06-05

    Adult Langerhans Cell Histiocytosis; Childhood Langerhans Cell Histiocytosis; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms

  1. Preliminary study on the evaluation of Langerhans cell histiocytosis using F-18-fluoro-deoxy-glucose PET/CT

    Institute of Scientific and Technical Information of China (English)

    Zhou Wenlan; Wu Hubing; Han Yanjiang; Wang Shaobo; Dong Ye; Wang Quanshi

    2014-01-01

    Background Limited number of studies have been reported regarding the utilization of F-18-fluoro-deoxy-glucose (F-18-FDG) positron emission tomography/computed tomography (F-18-FDG PET/CT) in Langerhans cell histiocytosis (LCH).The aim of this study was to assess the role of F-18-FDG PET/CT in the diagnosis and treatment of LCH.Methods Eight newly diagnosed and seven recurrent patients with LCH received F-18-FDG PET/CT scans.The diagnosis of LCH was established by pathology,multi-modality imaging,and clinical follow-up.Results F-18-FDG PET/CT was positive in 14 patients with 13 true positives and one false positive.All 45 LCH lesions were F-18-FDG avid including six small bone lesions <1.0 cm in diameter.The mean maximal standardized uptake value (SUVmax) was 7.13±4.91.F-18-FDG uptake showed no significant difference between newly diagnosed lesions vs recurrent lesions (SUVmax:6.50±2.97 vs.7.93±6.60,t=-0.901,P=0.376).Among 45 LCH lesions,68.9% (31/45) were found in bones and 31.1% (14/45) in soft tissue.The most commonly involved bones were the pelvis and vertebrae.There was no significant difference in F-18-FDG uptake between bone lesions vs.non-bone lesions (SUVmax:6.30±2.87 vs.8.97±7.58,t=1.277,P=0.221).In two patients,changes in F-18-FDG uptake on serial PET/CT scans reflected response of lesions to treatment.Conclusions The present study suggests that F-18-FDG PET/CT may be useful for diagnosis and assessing the treatment response of LCH.Because of the small sample size,further research is warranted to confirm our findings.

  2. Smoking and pulmonary Langerhans cell histiocytosis%吸烟与肺朗格汉斯细胞组织细胞增生症

    Institute of Scientific and Technical Information of China (English)

    张金铭; 徐美林; 力提甫·斯拉木

    2008-01-01

    肺朗格汉斯细胞组织细胞增生症的发生与吸烟有关.烟草糖蛋白可引起一系列细胞和细胞因子病理变化.戒烟可使部分患者病情稳定,改善影像学变化,甚至可使疾病(包括肺外病变)痊愈.%Pulmonary Langerhans cell histiocytosis(PLCH)is related to smoking.Tobacco glycoprotein can cause a series of changes of cell pathology and cytokine levels.Smoking cessation is significant in the medical management of many contributors tO PLCH,such as improvement of clinical symptoms and image and recovery of extra-pulmonary manifestation.

  3. Herpes-virus infection in patients with Langerhans cell histiocytosis: a case-controlled sero-epidemiological study, and in situ analysis.

    Directory of Open Access Journals (Sweden)

    Eric Jeziorski

    Full Text Available BACKGROUND: Langerhans cell histiocytosis (LCH is a rare disease that affects mainly young children, and which features granulomas containing Langerhans-type dendritic cells. The role of several human herpesviruses (HHV in the pathogenesis of LCH was suggested by numerous reports but remains debated. Epstein-barr virus (EBV, HHV-4, & Cytomegalovirus (CMV, HHV-5 can infect Langerhans cells, and EBV, CMV and HHV-6 have been proposed to be associated with LCH based on the detection of these viruses in clinical samples. METHODOLOGY: We have investigated the prevalence of EBV, CMV and HHV-6 infection, the characters of antibody response and the plasma viral load in a cohort of 83 patients and 236 age-matched controls, and the presence and cellular localization of the viruses in LCH tissue samples from 19 patients. PRINCIPAL FINDINGS: The results show that prevalence, serological titers, and viral load for EBV, CMV and HHV-6 did not differ between patients and controls. EBV was found by PCR in tumoral sample from 3/19 patients, however, EBV small RNAs EBERs -when positive-, were detected by in situ double staining in bystander B CD20+ CD79a+ lymphocytes and not in CD1a+ LC. HHV-6 genome was detected in the biopsies of 5/19 patients with low copy number and viral Ag could not be detected in biopsies. CMV was not detected by PCR in this series. CONCLUSIONS/SIGNIFICANCE: Therefore, our findings do not support the hypothesis of a role of EBV, CMV, or HHV-6 in the pathogenesis of LCH, and indicate that the frequent detection of Epstein-barr virus (EBV in Langerhans cell histiocytosis is accounted for by the infection of bystander B lymphocytes in LCH granuloma. The latter observation can be attributed to the immunosuppressive micro environment found in LCH granuloma.

  4. Cutaneous histiocytosis with Langerhans cell features induced by scabies: a case report.

    Science.gov (United States)

    Talanin, N Y; Smith, S S; Shelley, E D; Moores, W B

    1994-12-01

    An infant with biopsy-proven scabies developed nodular lesions. Histopathology revealed atypical histiocytes with Langerhans cell features. Within six months after treatment all skin lesions gradually disappeared. We suggest that the nodules in scabies can be due to Langerhans cell proliferation.

  5. Langerhans cell histiocytosis in children diagnosed by fine-needle aspiration

    Directory of Open Access Journals (Sweden)

    Uma Handa

    2015-01-01

    Conclusions: LCH is a rare disease occurring predominantly in children and can be diagnosed with ease on FNA cytology by the presence of characteristic Langerhans cells. The S-100 positivity aids in suggesting a diagnosis of LCH.

  6. Fibrosing mediastinitis with superior vena cava obstruction as the initial presentation of Langerhans' cell histiocytosis in a young child

    Energy Technology Data Exchange (ETDEWEB)

    Trusen, Andreas; Beissert, Matthias; Darge, Kassa [Department of Paediatric Radiology, Institute of Radiodiagnostics, University of Wuerzburg, Josef-Schneider-Strasse 2, 97080 Wuerzburg (Germany); Hebestreit, Helge [University Children' s Hospital, University of Wuerzburg, Wuerzburg (Germany); Marx, Alexander [Institute of Pathology, University of Wuerzburg, Wuerzburg (Germany)

    2003-07-01

    We present a 2-year-old girl with an unusual presentation of Langerhans' cell histiocytosis (LCH). Five months prior to admission to our hospital, she received IV steroids for bronchial obstruction. On admission, clinical signs of SVC obstruction were evident and a mediastinal mass was evident on the chest radiograph and MRI. Biopsy revealed fibrosing mediastinitis. Five months later, osteolysis was present on a skull radiograph. Surgical biopsy of the skull lesion revealed LCH. This case is unique because it demonstrates a rare initial manifestation of LCH that has not been previously reported. Furthermore, the primary, solitary mediastinal manifestation without calcifications was histologically interpreted as fibrosing mediastinitis, and the final diagnosis of LCH was only made after identifying the skull lesion. (orig.)

  7. Choroidal Neovascular Membrane Formation and Retinochoroidopathy in a Patient with Systemic Langerhans Cell Histiocytosis: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Aristomenis Thanos

    2012-04-01

    Full Text Available We report a case of bilateral atrophic retinochoroidopathy with choroidal neovascular membrane (CNVM formation in a patient with systemic Langerhans cell histiocytosis (LCH. A 35-year-old female, diagnosed with LCH at the age of 3, experienced an episode of acute vision loss in her right eye. Visual acuity was counting fingers. Dilated fundus exam and fluorescein angiography revealed the presence of CNVM along with bilateral widespread areas of chorioretinal atrophy. The patient underwent removal of CNVM with excellent postoperative visual acuity (20/25; however, indolent progression of her disease led to gradual deterioration of visual acuity (20/80 in the right eye and 20/320 in the left. This case shows that in contrast to previous reports, intraocular involvement of LCH does not need to be dramatic and clinically evident but it can acquire a chronic degenerative form. This report aims to raise awareness among ophthalmologists concerning the potential intraocular sequelae of LCH.

  8. Langerhans cell histiocytosis: a case report%朗格汉斯细胞组织细胞增生症1例

    Institute of Scientific and Technical Information of China (English)

    张锡宝; Steven; R; Cohen

    2001-01-01

    患儿6个月,生后2个月开始腹部,头皮,腹股沟部出现浅红色丘疹,头皮有脂溢性结痂。腹股沟、会阴区出现糜烂,局部发生溃疡。经病理及免疫组化检查证实为朗格汉斯细胞组织细胞增生症。%A 6-month-old male child, presented light red papules on theabdomen, groin, neck and scalp 2 months after birth. There were crusted, scaling dermatitis on the scalp and erosion/ulcer on the groin and perianal area. Skin biopsy and immunohistochemical study confirmed the diagnosis of Langerhans cell histiocytosis.

  9. Malignant histiocytosis. Case report

    OpenAIRE

    RUIZ, OSCAR; Instituto de Investigaciones Clínicas, UNMSM; Servicio Hematologia Clínica, Hospital Dos de Mayo; QUIÑONES, WILLY; Servicio Hematologia Clínica, Hospital Dos de Mayo; MISAD, OSCAR; Laboratorio de Anatomia Patológica “Oscar Misad; Delgado, Carlos; Instituto de Investigaciones Clínicas, Facultad de Medicina, Universidad Nacional Mayor de San Marcos. Lima, Perú.; Ronceros, Sergio; Instituto de Investigaciones Clínicas, Facultad de Medicina Humana, Universidad Nacional Mayor de San Marcos, Lima, Perú. médico patólogo.; MARANGONI, MANUELA; Departamento de Enfermería, Hospital Dos de Mayo; BARDALES, LUZ; Servicio Hematologia Clínica, Hospital Dos de Mayo; REYES, RAFAEL; Servicio Hematologia Clínica, Hospital Dos de Mayo; CASTILLO, ALFREDO; Servicio Hematologia Clínica, Hospital Dos de Mayo; URRUTIA, KATIA; Servicio Hematologia Clínica, Hospital Dos de Mayo

    2013-01-01

    Fourteen year-old male patient referred from Huancayo who presented one month gastric intolerance, jaundice, fever and a lymph proliferative syndrome. Laboratory tests revealed severe pancytopenia due to phagocytosis. Haematologic and anatomy-pathology diagnosis was human malignant histiocytosis. We present this case due to its low frequency and the emergency character of the disease. Paciente varón de 14 años, procedente de Huancayo, que presenta un mes antes de su hospitalización intoler...

  10. DNA damage repair is unaffected by mimicked heterozygous levels of BRCA2 in HT-29 cells

    Directory of Open Access Journals (Sweden)

    Brian Tannenbaum, Tobechukwu Mofunanya, Alan R. Schoenfeld

    2007-01-01

    Full Text Available Functional loss of both alleles of the breast cancer susceptibility gene, BRCA2, facilitates tumorigenesis. However, the direct effects of BRCA2 heterozygosity remain unclear. Here, BRCA2 heterozygosity was mimicked in HT-29 colon cells by reducing levels of BRCA2 through stable RNA interference. No difference in RAD51 subcellular localization and focus formation was observed between control and mimicked heterozygous cell lines. DNA repair ability, as measured by colony survival following mitomycin C treatment and ultraviolet radiation exposure, was also unaffected by reduced levels of BRCA2. Interestingly, the growth rate of the mimicked BRCA2 heterozygous cell line was significantly lower than that of control cells. Increased expression of p53 in the mimicked heterozygous cells was observed, perhaps in response to BRCA2 deficiency. Levels of p27 were also found to be slightly increased in cells with reduced BRCA2, perhaps contributing to the slower growth rate. Overall, these results suggest that tumors are unlikely to arise directly from BRCA2 heterozygous cells without other genetic events such as loss of the wild-type BRCA2 allele and/or loss of p53 function or other cell cycle inhibitors.

  11. Congenital cutaneous histiocytosis in a piglet.

    Science.gov (United States)

    Hélie, P; Kiupel, M; Drolet, R

    2014-07-01

    A 2-week-old crossbred male piglet with numerous congenital, variably sized macules, plaques, and papules distributed all over the body was submitted for necropsy. Significant gross and histological lesions were restricted to the skin. On light microscopic examination, these cutaneous lesions corresponded to dermal and/or subcutaneous masses composed of spindle-shaped to round cells that multifocally contained hemosiderin; epidermotropism was not observed. Immunohistochemically, the neoplastic cells were strongly positive for CD204; moderately positive for CD163, lysozyme, and vimentin; and negative for Mac 387, α-1-antitrypsin, S-100 protein and E-cadherin; frozen tissues were not available for CD1a and CD11c. Transmission electron microscopic examination of sections from formalin-fixed tissues did not reveal Birbeck's granules. The clinical, morphological, and immunohistochemical results were consistent with a congenital cutaneous histiocytosis of non-Langerhans cell origin. The condition most resembled juvenile xanthogranuloma in humans, a generally skin-limited non-Langerhans histiocytic disorder that can be congenital. Cutaneous and/or systemic histiocytic disorders are well characterized in dogs and have been described in cats, and a case with some similarities to ours has been reported in a neonatal piglet, but this is to our knowledge the first immunohistochemically supported report of histiocytosis in the pig and congenital histiocytosis in animals.

  12. 成年女性下颌骨朗格汉斯细胞组织细胞增生症1例%Langerhans cell histiocytosis: A case report

    Institute of Scientific and Technical Information of China (English)

    葛月华; 敬伟; 廖小宜; 林云锋

    2012-01-01

    本文报道1例下颌骨朗格汉斯细胞组织细胞增生症.患者,女性,34岁,左下后牙龈疼痛1年,伴溢脓2个月,经组织病理及免疫组织化学检查证实为朗格汉斯细胞组织细胞增生症.%A case of Langerhans cell histiocytosis of the mandible was reported. A 34-year-old woman presented with pain for one year on the gingival of the low left jaw, and together with pyorrhea in the past 2 months. The his-topathology and immunohistochemical examination confirmed the diagnosis of Langerhans cell histiocytosis.

  13. Pure cutaneous histiocytosis X.

    Science.gov (United States)

    Magaña-García, M

    1986-03-01

    A 38-month-old boy presented with nodules in the skin of the genital region present for 2 1/2 years. These later spread to the skin of the trunk, head, and extremities. A complete clinical workup could not reveal involvement in any other organ sites and biopsy of one of the cutaneous lesions was diagnosed as histiocytosis X. Because the child was in generally good condition, no treatment was given. Follow-up revealed that the disease had remained limited to the skin, where 15% of the lesions disappeared spontaneously.

  14. Breast cancer cell behaviors on staged tumorigenesis-mimicking matrices derived from tumor cells at various malignant stages.

    Science.gov (United States)

    Hoshiba, Takashi; Tanaka, Masaru

    2013-09-20

    Extracellular matrix (ECM) has been focused to understand tumor progression in addition to the genetic mutation of cancer cells. Here, we prepared "staged tumorigenesis-mimicking matrices" which mimic in vivo ECM in tumor tissue at each malignant stage to understand the roles of ECM in tumor progression. Breast tumor cells, MDA-MB-231 (invasive), MCF-7 (non-invasive), and MCF-10A (benign) cells, were cultured to form their own ECM beneath the cells and formed ECM was prepared as staged tumorigenesis-mimicking matrices by decellularization treatment. Cells showed weak attachment on the matrices derived from MDA-MB-231 cancer cells. The proliferations of MDA-MB-231 and MCF-7 was promoted on the matrices derived from MDA-MB-231 cancer cells whereas MCF-10A cell proliferation was not promoted. MCF-10A cell proliferation was promoted on the matrices derived from MCF-10A cells. Chemoresistance of MDA-MB-231 cells against 5-fluorouracil increased on only matrices derived from MDA-MB-231 cells. Our results showed that the cells showed different behaviors on staged tumorigenesis-mimicking matrices according to the malignancy of cell sources for ECM preparation. Therefore, staged tumorigenesis-mimicking matrices might be a useful in vitro ECM models to investigate the roles of ECM in tumor progression.

  15. Fluidic and air-stable supported lipid bilayer and cell-mimicking microarrays.

    Science.gov (United States)

    Deng, Yang; Wang, Yini; Holtz, Bryan; Li, Jingyi; Traaseth, Nathan; Veglia, Gianluigi; Stottrup, Benjamin J; Elde, Robert; Pei, Duanqing; Guo, Athena; Zhu, X-Y

    2008-05-14

    As drug delivery, therapy, and medical imaging are becoming increasingly cell-specific, there is a critical need for high fidelity and high-throughput screening methods for cell surface interactions. Cell membrane-mimicking surfaces, i.e., supported lipid bilayers (SLBs), are currently not sufficiently robust to meet this need. Here we describe a method of forming fluidic and air-stable SLBs through tethered and dispersed cholesterol groups incorporated into the bottom leaflet. Achieving air stability allows us to easily fabricate SLB microarrays from direct robotic spotting of vesicle solutions. We demonstrate their application as cell membrane-mimicking microarrays by reconstituting peripheral as well as integral membrane components that can be recognized by their respective targets. These demonstrations establish the viability of the fluidic and air-stable SLB platform for generating content microarrays in high throughput studies, e.g., the screening of drugs and nanomedicine targeting cell surface receptors.

  16. 朗格汉斯组织细胞增生症引起的甲损害%Nail changes in Langerhans cell histiocytosis

    Institute of Scientific and Technical Information of China (English)

    陆原; 关杨; 翁翊; 何雯; 钟萍; 徐丽红

    2014-01-01

    Skin lesions occur frequently in Langerhans cell histiocytosis (LCH)whereas nail lesions are rare. Nail lesion, is characterized by various features like paronychial erythema, swelling nail fold destruction, longitudinal grooving, purpuric striae, subungual purpura hyperkeratosis, subungual pustules, deformity, loss of nail plate, paronychia, onycholysis, longitudinal ery-thronychia splinter hemorrhages and pitting of the fingernails and toenails. A 34-year-old male presented with diabetes in-sipidus, exophthalmos and bone defects and mucocutaneous lesions, including seborrheic dermatitis on the face and scalp, in-filtrated nodules and ulcerated plaques, especially in the axillae and anogenital region. Nail changes included paronychial ery-thema, swelling nail fold destruction, subungual purpura, onycholysis, splinter hemorrhages, longitudinal grooving and longitudi nal erythronychia. The diagnosis of Langerhans cell histiocytosis was made by histopathologic examination of skin biopsies. LCH is rare and easy to be misdiagnosed. The confirmed diagnosis is based on the aggregate analysis of its histological features,nail changes in Langerhans cell histiocytosis may be a possible marker of multisystem disease. The role of nail lesion as an unfavor-able prognostic sign is unclear.%朗格汉斯组织细胞增生症(Langerhans cell histiocytosis,LCH)的皮肤损害较多见,而甲损害则罕见。其临床上可表现为指、趾甲的甲周红斑及肿胀、甲襞破坏、纵向开槽、紫癜样纹、甲下紫癜、甲角化过度、甲下脓疱、甲变形、甲板缺失、甲沟炎、甲剥离、纵形红甲和甲蚀斑。报告1例34岁男性患者,具有尿崩症,突眼,骨损害及皮肤黏膜病变,如:头面部脂溢性皮炎样损害,浸润结节和溃疡斑块,特别是在腋下,外生殖器区域。指甲损害表现为甲周红斑及肿胀、甲分离、甲下紫癜、裂片样出血、纵向开槽及纵形红甲。皮损组织病

  17. Langerhans cell histiocytosis: clinical analysis of 126 cases%朗格汉斯细胞组织细胞增多症126例临床分析

    Institute of Scientific and Technical Information of China (English)

    树叶; 曾迎红; 周斌; 陈卫坚; 汤建萍

    2013-01-01

    目的 分析朗格汉斯细胞组织细胞增多症患儿的临床特点.方法 对2006-2011年我院126例朗格汉斯细胞组织细胞增多症进行临床回顾性分析.结果 126例患者中年龄最小为2个月,最大9岁,男女比例是2.5∶1.皮肤病变呈湿疹样、脂溢性、出血性斑丘疹样、黄色结节样和白色斑疹样.在3种传统临床分型中,Letterer-Siwe病所占比例最高,患者年龄最小,受累器官最多,多伴有肝脾肿大、X线胸片异常、造血功能损伤、多部位骨受累,临床分级主要是Ⅲ级和Ⅳ级.Hand-Schüller-Christian病的患儿临床分级各级均有分布,以Ⅱ级最多.骨嗜酸细胞肉芽肿患者年龄最大,除骨骼以外未发现其他器官受累,临床分级大部分为Ⅰ级.治疗采用外科手术或联合化疗.结论 朗格汉斯细胞组织细胞增多症皮损表现有特征性,临床表现多样,病理有诊断意义,治疗和疗效取决于疾病程度和受累器官.%Objective To analyze lesional and clinical characteristics of Langerhans cell histiocytosis in children.Methods A clinical retrospective study was performed on 126 patients with Langerhans cell histiocytosis collected from 2006 to 2011 at the Hunan Children's Hospital.Results Of the 126 patients,the youngest was 2months old,and the oldest was 9 years old.The ratio of male to female was 2.5 ∶ 1.Clinical manifestations included eczematid,seborrheic lesions,hemorrhagic maculopapules,yellow nodules and white macules.Of the three clinical phenotypes of Langerhans cell histiocytosis,Letter-Siwe disease was the most prevalent,and most cases of LetterSiwe disease were associated with hepatosplenomegaly,abnormal chest X-ray,impaired hematopoietic function and multifocal bone injuries.The clinical grade was mainly Ⅲ and Ⅳ in patients with Letter-Siwe disease,Ⅰ in patients with eosinophilic granuloma,and varied from Ⅰ to Ⅳ in patients with Hand-Schuller-Christian disease with Ⅱ as the most common

  18. Radiotherapy in Treatment of Patients with Langerhans Cell Histiocytosis%朗格汉斯组织细胞增生症放疗的疗效观察

    Institute of Scientific and Technical Information of China (English)

    姜瑶; 伍钢; 张盛; 丁乾

    2012-01-01

    Objective To evaluate the efficacy, toxicity and dose of radiotherapy in treatment of patients with Langerhans cell histiocytosis. Methods A total of 21 patients with Langerhans cell histiocytosis were retrospectively analysed. Results The median follow-up period was 4 years. Median total radiotherapy dose for pediatric patients( 14 patients,27 sites ) was 1 600 cGy and for adults ( 7 patients, 9 sites ) 2 000 cGy. Median singal radiotherapy doses for pediatric patients and adults were 180 cGy and 200 cGy, respectively. Local control rate was 96. 3% ( 74. 1% complete remissions ) for pediatric patients compared to 100. 0%( 66.7% complete remissions ) for adults. No patients experienced acute or late side effects of radiotherapy. There is no significant difference in the local control rate and complete remission rate between ≤2 000 cGy and > 2 000 cGy( P >0. 05 ). No patients developed in-field recurrences. Conclusion Radiotherapy can be successfully applied for patients with Langerhans cell histiocytosis. In-field recurrences and side effects of radiotherapy were rarely seen.%目的 探讨放疗对朗格汉斯组织细胞增生症的疗效及不良反应.方法 回顾性分析21例经病理检查确诊的朗格汉斯组织细胞增生症患者的临床资料.结果 非成人组14例(27个病变部位),中位单次放疗剂量180 cGy,中位总放疗剂量1 600 cGy;局部控制率96.3%,完全缓解率为74.1%.成人组7例(9个病灶),中位单次放疗剂量200 cGy,中位总放疗剂量2 000 cGy;局部控制率100.0%,完全缓解率66.7%.放疗剂量≤2 000 cGy者与>2 000 cGy者局部控制率及完全缓解率比较,无显著性差异(P>0.05).中位随访时间4年.随访期间无严重放疗并发症发生;无野内复发.结论 放疗是朗格汉斯组织细胞增生症的有效治疗手段,其局控率高,野内复发及严重放疗并发症少见.

  19. Pulmonary Langerhans cell histiocytosis with cervical lymph node involvement, and coexistence with pulmonary tuberculosis and right pneumothorax: a case report and review of literature.

    Science.gov (United States)

    Gao, Limin; Li, Huifang; Li, Gandi; Liu, Weiping; Li, Jinnan; Zhang, Wenyan

    2015-01-01

    We report an uncommon 22-year-old male Pulmonary Langerhans Cell Histiocytosis (PLCH) case which co-existed with pulmonary tuberculosis (TB). Unlike the common PLCH cases, this PLCH case has cervical lymph node involvement and right pneumothorax. The diagnosis was established by the imaging of lung and the biopsies of the lung and left neck lymph node. Imaging of the chest showed characteristic small nodules and thin-walled cysts and right pneumothorax. The LCH cells in the lung and left neck lymph node were characterized by large convoluted nuclei with cerebriform indentations of the nuclear envelope and longitudinal grooves. The nuclei contained small eosinophilic nucleoli and moderate amount cytoplasm. Immunohistochemically, the histiocytoid cells were positive for Langerin, CD1a and S-100. Acid-fast bacilli were found in sputum and lung biopsy tissue. To the best of our knowledge, this is the first case of PLCH with cervical lymph node involvement, and coexisted with pulmonary tuberculosis, right pneumothorax. A contribution of this case and review three of the five cases of PLCH with extrapulmonary involvement to lymph nodes resolved spontaneously after smoking cessation constitute a novel addition that it is inappropriate to regard pulmonary/nodal LCH as multi-organ or disseminated disease, and the treatment methods are the same whether the PLCH patient with lymph node involvement or not.

  20. Pulmonary Langerhans cell histiocytosis with cervical lymph node involvement, and coexistence with pulmonary tuberculosis and right pneumothorax: a case report and review of literature

    Science.gov (United States)

    Gao, Limin; Li, Huifang; Li, Gandi; Liu, Weiping; Li, Jinnan; Zhang, Wenyan

    2015-01-01

    We report an uncommon 22-year-old male Pulmonary Langerhans Cell Histiocytosis (PLCH) case which co-existed with pulmonary tuberculosis (TB). Unlike the common PLCH cases, this PLCH case has cervical lymph node involvement and right pneumothorax. The diagnosis was established by the imaging of lung and the biopsies of the lung and left neck lymph node. Imaging of the chest showed characteristic small nodules and thin-walled cysts and right pneumothorax. The LCH cells in the lung and left neck lymph node were characterized by large convoluted nuclei with cerebriform indentations of the nuclear envelope and longitudinal grooves. The nuclei contained small eosinophilic nucleoli and moderate amount cytoplasm. Immunohistochemically, the histiocytoid cells were positive for Langerin, CD1a and S-100. Acid-fast bacilli were found in sputum and lung biopsy tissue. To the best of our knowledge, this is the first case of PLCH with cervical lymph node involvement, and coexisted with pulmonary tuberculosis, right pneumothorax. A contribution of this case and review three of the five cases of PLCH with extrapulmonary involvement to lymph nodes resolved spontaneously after smoking cessation constitute a novel addition that it is inappropriate to regard pulmonary/nodal LCH as multi-organ or disseminated disease, and the treatment methods are the same whether the PLCH patient with lymph node involvement or not. PMID:25973117

  1. Radiotherapy for management of Langerhans' cell histiocytosis. Two case reports and a literature review; Strahlentherapie bei der Langerhanszell-Histiozytose. Zwei Einzelfallberichte - Literaturuebersicht

    Energy Technology Data Exchange (ETDEWEB)

    Heyd, R.; Strassmann, G.; Martin, T.; Zamboglou, N. [Staedtische Kliniken Offenbach, Offenbach am Main (Germany). Strahlenklinik; Donnerstag, F. [Staedtische Kliniken Offenbach, Offenbach am Main (Germany). Inst. fuer Roentgendiagnostik

    2000-07-01

    The use of radiotherapy in the treatment of Langerhans' cell histiocytosis was first reported in the literature in 1930 and has been proven as effective in numerous studies. We present the results of two female adults with eosinophilic granuloma of bone who underwent conventionally fractionated radiation therapy with total doses of 7x1.8 Gy and 7x2.0 Gy in four different sites. After observation periods raging from three months to six years local control of the disease was achieved in all treated locations. A review of 18 previously published studies include a total of 310 sites of eosinophilic granuloma of bone in 216 patients. It was demonstrated in 13 studies that the patients had complete relief of symptoms. An average of 94.3% had local control of the symptoms. Furthermore, in 12 studies for a total of 344 cases with involvement of other organs local control was reported in an average of 64.8% (range: 14.3-100%). Based on our own observations and on the literature review we conclude that low dose radiation therapy plays an important role in the management of localised Langerhans' cell histiocytosis. In order to minimise the risk of radiation induced neoplasms an accurate and precise radiation technique is required. (orig.) [German] Der Einsatz der Strahlentherapie zur Behandlung von Langerhanszell-Histiozytosen ist seit 1930 in der Literatur beschrieben und ihre Wertigkeit ist durch eine Reihe von Untersuchungen belegt. Zwei weibliche, erwachsene Patientinnen aus unserem Krankengut wurden aufgrund eines eosinophilen Granulom des Stuetzapparates in insgesamt vier Lokalisationen mit Gesamtdosen von 7x1,8 Gy und 7x2,0 Gy einer postoperativen und primaeren Radiotherapie unterzogen. Bei Nachbeobachtungszeitraeumen von 3 Monaten bis 6 Jahren wurde in allen Lokalisationen jeweils eine komplette Remission der Erkrankung erzielt. Die Analyse von 18 vorangegangenen Studien, in denen 216 Patienten an insgesamt 310 Knochenherden behandelt wurden, ergab, dass in

  2. Successful treatment of a case of acute myeloid leukemia following Langerhans cell histiocytosis in an adolescent: a case report and review of the literature

    Science.gov (United States)

    Xu, Gaixiang; Yang, Min; Huang, Jian; Jin, Jie

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder of unknown etiopathogenesis. Its clinical presentation is variable and ranges from isolated skin or bone disease to a life-threatening multisystem condition. LCH can occur at any age but is more frequent in the pediatric population. The diagnosis depends on clinical, histopathological and radiographic examination and should be confirmed by immunohistochemical study with CD1a, S100 protein and langerin, three markers used widely for identifying Langerhans cells. Herein, we report an adolescent with acute myeloid leukemia (AML-M2) who was treated just with surgical management alone for LCH. As far as we know, this is the first case that the LCH patient without chemotherapy evolved into AML and was successfully cured. Cooperative studies of large numbers of LCH patients are needed to evaluate a possible association between LCH and acute leukemia, and to identify common risk factors or predisposing agents if such be present. The previously reported cases of LCH concomitant with other hematological disorders are also summarized and described compared with the present case. PMID:25932277

  3. Clear cell sarcoma: A case mimicking primary cutaneous malignant melanoma

    Directory of Open Access Journals (Sweden)

    Rodriguez-Martin M

    2009-01-01

    Full Text Available Clear cell sarcoma (CCS is a recently described variant of sarcoma characterized by prominent clear cells showing features similar to clear cell melanoma. This neoplasm was first described by Dr. Franz M. Erzinger. Primary CCS usually arises in deeper soft tissues, in association with fascia, tendons, or aponeuroses. Characteristic translocation t(12;22 (q13;q12 has been considered pathognomonic for CCS. Prognosis is related to tumor size. An early recognition and initial radical surgery is the key to a favourable outcome. We present a patient with an unusual neoplasm that resembled malignant melanoma.

  4. Cell-based microfluidic platform for mimicking human olfactory system.

    Science.gov (United States)

    Lee, Seung Hwan; Oh, Eun Hae; Park, Tai Hyun

    2015-12-15

    Various attempts have been made to mimic the human olfactory system using human olfactory receptors (hORs). In particular, OR-expressed cell-based odorant detection systems mimic the smell sensing mechanism of humans, as they exploit endogenous cellular signaling pathways. However, the majority of such cell-based studies have been performed in the liquid phase to maintain cell viability, and liquid odorants were used as detection targets. Here, we present a microfluidic device for the detection of gaseous odorants which more closely mimics the human olfactory system. Cells expressing hOR were cultured on a porous membrane. The membrane was then flipped over and placed between two compartments. The upper compartment is the gaseous part where gaseous odorants are supplied, while the lower compartment is the aqueous part where viable cells are maintained in the liquid medium. Using this simple microfluidic device, we were able to detect gaseous odorant molecules by a fluorescence signal. The fluorescence signal was generated by calcium influx resulting from the interaction between odorant molecules and the hOR. The system allowed detection of gaseous odorant molecules in real-time, and the findings showed that the fluorescence responses increased dose-dependently in the range of 0-2 ppm odorant. In addition, the system can discriminate among gaseous odorant molecules. This microfluidic system closely mimics the human olfactory system in the sense that the submerged cells detect gaseous odorants.

  5. Long bone Langerhans cell histiocytosis (1 case)%长骨朗格汉斯细胞组织细胞增生症1例

    Institute of Scientific and Technical Information of China (English)

    高莹莹

    2011-01-01

    @@ 长骨朗格汉斯细胞组织细胞增生症(Langherhens cell histiocytosis,LCH)为组织细胞增生症Ⅰ型,临床上少见[1],影像学表现多样,易造成误诊,我院遇到1例,现报道如下. 男,3岁.无明显诱因出现左髋部疼痛,并活动受限1月,加重1周.查体:左髋部及股骨上段皮温不高,皮色未见异常改变,左股骨上段有压痛、叩击痛,左髋部活动受限,肢端血运及活动正常.实验室检查:血常规及血生化全套检验未见明显异常.影像学检查:X线片示(见图1)左股骨上段可见沿长轴走行骨质破坏区,内密度不均,边界欠清;局部皮质不规整,有缺损改变;较成熟的层状广泛骨膜反应,局部显示不规则.

  6. Analysis on Pulmonary Langerhans Cell Histiocytosis%肺郎格罕细胞组织细胞增多症的研究

    Institute of Scientific and Technical Information of China (English)

    黄娜; 刘丹; 袁泉; 李晓亮; 杨小东

    2010-01-01

    目的 探讨肺郎格罕细胞组织细胞增多症(pulmonary Langerhans cell histiocytosis,PLCH)的临床特征、诊断和治疗.方法 回顾性分析1999年-2008年4例病理确诊的PLCH.结果 4例患者均为男性;13~56岁;2例吸烟.临床特征是活动后气短、气胸.胸部CT表现为网格状、囊状或结节状影像.肺活检病理结果:光学显微镜下可见病理性郎格罕细胞,免疫组织化学法检测发现4例S-100均为阳性,2例CDla阳性,1例CD68阳性.结论 PLCH胸部CT表现为网结节或囊性变,病理检查见病理性郎格罕细胞浸润细支气管壁和上皮细胞的间质,免疫组织化学CDla抗原、S-100蛋白阳性可明确诊断.

  7. 肺朗格汉斯细胞组织细胞增生症的CT表现%CT Findings of Pulmonary Langerhans Cell Histiocytosis

    Institute of Scientific and Technical Information of China (English)

    曹毅媛; 廖美焱

    2012-01-01

    目的:分析肺朗格汉斯细胞组织细胞增生症(PLCH)的CT表现,以提高对该病诊断的准确性.方法:回顾性分析经病理证实的2例PLCH患者的临床和CT资料,2例患者均于治疗前后多次行影像学检查.结果:2例患者均合并其他系统的朗格汉斯细胞组织细胞增生症,PLCH的CT表现为多发囊腔和结节,好发于上叶,治疗后结节部分吸收,纤维化.结论:PLCH的CT表现有其特有的规律和特征,熟悉这些表现,有助于对该病的诊断.%Objective: To analyze the CT manifestations of pulmonary Langerhans cell histiocytosis (PLCH), and to improve the diagnosis of the disease. Methods: The clinical and CT data of two cases of pathologically confirmed PLCH were retrospectively studied. Results: The main CT findings of PLCH were multiple cysts and nodules, which became smaller and showed fibrosis after treatment. Conclusion: The CT manifestations of PLCH are special and helpful for the diagnosis.

  8. Histiocitose de células de Langerhans: relato de caso e revisão da literatura Langerhans' cell histiocytosis: case report and review

    Directory of Open Access Journals (Sweden)

    Ada Lobato Quattrino

    2007-08-01

    Full Text Available Os autores descrevem caso de paciente de dois anos de idade, apresentando lesões pápulovesiculosas no tronco que evoluíram para crostas, além de tumorações com necrose na região cervical. Durante a internação, observou-se estrabismo convergente à direita, de instalação súbita. Os exames histopatológico e imuno-histoquímico das lesões cutâneas comprovaram tratar-se de histiocitose de células de Langerhans, sendo o paciente submetido à quimioterapia com melhora importante do quadro.The authors describe the case of a two-year-old boy who presented vesico-papular lesions all over the trunk which became crusted, in addition to tumorations with necrosis in the cervical region. In the course of his hospitalization, he had sudden onset of convergent strabismus on the right side. Histopathological and immunohistochemical examination of cutaneous lesions revealed typical aspects of Langerhans' cell histiocytosis, thus confirming diagnosis. The patient underwent chemotherapy, with a significant improvement in his clinical picture.

  9. Histiocitose das células de Langerhans na região anogenital Langerhans cell histiocytosis of the anogenital region

    Directory of Open Access Journals (Sweden)

    M. S. Neto

    1998-12-01

    Full Text Available A Histiocitose das células de Langerhans (HCL acometendo o trato genital tem uma incidência rara com apenas 48 casos relatados na literatura1-5 . Considerando somente as lesões da região anogenital, esse número cai para dois casos descritos1,2. Reportamos o 3º caso de HCL anogenital em uma paciente de 31 anos, branca, cuja comprovação diagnóstica foi feita através de microscopia eletrônica. O tratamento realizado foi quimioterapia sistêmica e excisão cirúrgica local.The Langerhans cell histiocytosis (LCH of the genital tract is rare, with only 48 cases related in the literature1-5. There were reported only 2 cases in the anogenital region1,2. We reported the third case of LCH in the anogenital region; patient was female, 31 years-old, caucasian and the diagnosis was confirmed by electron microscopic magnification. The treatment was local surgical excision and systemic chemotherapy.

  10. Radiological Findings of Langerhans Cell Histiocytosis%郎格尔汉斯细胞组织细胞增生症的影像学表现

    Institute of Scientific and Technical Information of China (English)

    史洪平; 戴洪修; 杜云

    2001-01-01

    目的进一步提高对郎格尔汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)影像学表现的认识.方法回顾性分析经实验室及病理证实的16例LCH的临床和影像学特点.结果按传统分型,符合勒-雪氏病(LS)3例,韩-薛柯氏病(HSC)2例,骨嗜酸性肉芽肿(EGB)11例.颅骨单发破坏8例,多发破坏2例,下颌骨、肋骨、股骨及尺骨破坏各1例;伴肺内浸润3例,软组织肿块7例.结论 LCH影像学表现缺乏特异性,传统X线检查仍为首选的影像学检查,CT能更准确地显示病变范围.

  11. Metastatic transitional cell carcinoma of the tibia radiologically mimicking osteosarcoma.

    LENUS (Irish Health Repository)

    Cunningham, Laurence Patrick

    2013-01-01

    We report a case of a 73-year-old lady with transitional cell carcinoma and no evidence of metastatic disease presenting with gradual weight loss, pretibial swelling and painful weightbearing. Investigations revealed a lesion of the right tibial diaphysis. The radiological and clinical appearance was that of primary osteosarcoma. Biopsy results revealed metastatic transitional cell carcinoma of the tibia. Intramedullary nailing was performed which relieved pain on weightbearing. The patient declined radiotherapy and was started on a palliative care regimen. This case illustrates the importance of histological diagnosis in the treatment of diaphyseal lesions.

  12. Primary cerebellar extramedullary myeloid cell tumor mimicking oligodendroglioma.

    Science.gov (United States)

    Ho, D M; Wong, T T; Guo, W Y; Chang, K P; Yen, S H

    1997-10-01

    Extramedullary myeloid cell tumors (EMCTs) are tumors consisting of immature cells of the myeloid series that occur outside the bone marrow. Most of them are associated with acute myelogenous leukemia or other myeloproliferative disorders, and a small number occur as primary lesions, i.e., are not associated with hematological disorders. Occurrence inside the cranium is rare, and there has been only one case of primary EMCT involving the cerebellum reported in the literature. The case we report here is a blastic EMCT occurring in the cerebellum of a 3-year-old boy who had no signs of leukemia or any hematological disorder throughout the entire course. The cerebellar tumor was at first misdiagnosed as an "oligodendroglioma" because of the uniformity and "fried egg" artifact of the tumor cells. The tumor disappeared during chemotherapy consisting of 12 treatments. However, it recurred and metastasized to the cerebrospinal fluid (CSF) shortly after the therapy was completed. A diagnosis of EMCT was suspected because of the presence of immature myeloid cells in the CSF, and was confirmed by anti-myeloperoxidase and anti-lysozyme immunoreactivity of the cerebellar tumor. The patient succumbed 1 year and 3 months after the first presentation of the disease.

  13. [Pulmonary Langerhans histiocytosis and Hodgkin's lymphoma].

    Science.gov (United States)

    Paris, A; Dib, M; Rousselet, M-C; Urban, T; Tazi, A; Gagnadoux, F

    2011-09-01

    Pulmonary Langerhans histiocytosis (PLH) is a rare disease due to the accumulation of Langerhans cells at the level of the bronchioles. These dendritic immunocytes form granulomata and destroy the wall of the airway. We report a case of PLH developing at the same time as Hodgkin's lymphoma in a young woman who smoked tobacco and cannabis. We observed a complete remission of the PLH lesions parallel to the remission of the Hodgkin's lymphoma after chemotherapy, in the absence of any change in the consumption of tobacco and cannabis. This observation leads us to discuss the potential relationships between PLH on one hand, and smoking, the lymphoma and its treatment on the other.

  14. Histiocytic sarcoma that mimics benign histiocytosis.

    Science.gov (United States)

    Boisseau-Garsaud, A M; Vergier, B; Beylot-Barry, M; Nastasel-Menini, F; Dubus, P; de Mascarel, A; Eghbali, H; Beylot, C

    1996-06-01

    A 28-year-old man presented with a histiocytic sarcoma of a very uncommon origin, as it had developed for several years like a benign cutaneous histiocytosis resembling generalized eruptive histiocytoma before becoming acute, with nodal and massive pulmonary involvement. Despite various chemotherapies, the patient died within 8 months. Skin biopsies showed histiocytic proliferation in the dermis and node biopsies showed histiocytic proliferation with a sinusoidal pattern. Immunohistochemical analysis, performed on paraffin-embedded sections, demonstrated strong labeling of tumoral cells for CD68 and moderate labeling for CD3 and CD4. CD30 labeling was negative. S-100 protein was positive on a Langerhans' cell reactive subpopulation. Electron microscopy confirmed the histiocytic nature of malignant cells and showed cytoplasmic inclusions such as regularly laminated bodies, dense bodies and pleomorphic inclusions. No Birbeck granules were seen. A gene rearrangement study of T-cell receptor gamma and immunoglobulin heavy chain genes showed a germline configuration. Histiocytic sarcoma is an extremely rare true histiocytic malignancy, the existence of which has been recently debated since it has often been mistaken in the past for large cell lymphomas. Such a deceptive onset as benign cutaneous histiocytosis has not been described in the literature to our knowledge.

  15. Extraosseous extension of Gaucher cell deposits mimicking malignancy

    Energy Technology Data Exchange (ETDEWEB)

    Hermann, G. (Dept. of Radiology, Mount Sinai Medical Center of the City Univ. of New York, NY (United States)); Shapiro, R. (Dept. of Radiology, Mount Sinai Medical Center of the City Univ. of New York, NY (United States)); Abdelwahab, I.F. (Dept. of Radiology, Mount Sinai Medical Center of the City Univ. of New York, NY (United States)); Klein, M.J. (Dept. of Pathology, Mount Sinai Center of the City Univ. of New York, NY (United States)); Pastores, G. (Dept. of Human Genetics, Mount Sinai Medical Center of the City Univ. of New York, NY (United States)); Grabowski, G. (Cincinnati Children' s Hospital, Cincinnati Univ., Coll. of Medicine, OH (United States))

    1994-05-01

    Two cases are described in which patients with type I Gaucher disease developed extraosseous soft tissue masses consisting of Gaucher cell deposits. In one instance the mass destroyed the posterior cortex of the left distal femur and protruded into the soft tissues. In the second case the lesion involved the proximal tibia and gradually extended into the soft tissues. While the incidence of neoplastic disorder such as lymphoproliferative disease appears to be more common in Gaucher disease patients than in the general population, lesions of benign etiology that mimic these aggressive processes should be considered in the differential diagnosis when cortical destruction with coexisting soft tissue most is found in these patients. (orig.)

  16. New advances in the treatment of multisystem Langerhans cell histiocytosis%多系统累及朗格汉斯细胞组织细胞增生症治疗进展

    Institute of Scientific and Technical Information of China (English)

    张珍; 余红

    2014-01-01

    朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)是一种罕见的树突细胞和网状细胞系统增生性疾病,目前分为单系统LCH(SS-LCH)和多系统LCH(MS–LCH)。MS-LCH的预后较差,及时有效地干预是患者存活的关键。目前有化学治疗、免疫治疗、造血干细胞移植治疗等多种治疗方案。文章就MS-LCH相关治疗方案的进展进行综述。%Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of dendritic cells and the reticular cells. It is divided into single-system langerhans cell histiocytosis (SS-LCH) and multi-system langerhans cell system (MS-LCH). MS-LCH has a very poor prognosis. The key of survival is timely and effective treatment. The treatment protocols include chemotherapy, immunotherapy and stem cells transplantation. In this review, the new advances in the treatment of MS-LCH were systemically reviewed.

  17. Primary intraosseous squamous cell carcinoma mimicking periapical disease: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Yoon Joo; Oh, Song Hee; Kang, Ju Han; Choi, Hwa Young; Kim, Gyu Tae; Choi, Yong Suk; Hwang, Eui Hwan [Dept. of Oral and Maxillofacial Radiology, School of Dentistry, Kyung Hee University, Seoul (Korea, Republic of); Yu, Jae June [Dept. of Oral and Maxillofacial Radiology, Kangdong Sacred Heart Hospital, Hallym Medical Center, Seoul (Korea, Republic of)

    2012-09-15

    Primary intraosseous squamous cell carcinoma (PIOSCC) is a rare carcinoma, which arises within the jaws without connection to the oral mucosa and presumably develops from a remnant of odontogenic epithelium. We present a case of solid type PIOSCC in a 52-year-old male patient complaining of dull pain on his left lower molar. In this case, early stage PIOSCC mimicking a periapical lesion might lead to a one-year delay in treatment due to the misdiagnosis of osteomyelitis after extraction of the third molar. The clinical, radiological, and histologic features are described. In this case, there was initial radiographic evidence for PIOSCC mimicking a periapical lesion. Incautious radiographic interpretation and treatment procedures had delayed the correct diagnosis and resulted in extensive bony destruction during the patient's disease progression.

  18. Clinicopathological analysis of langerhans cell histiocytosis%朗格汉斯细胞组织细胞增生症临床病理学分析

    Institute of Scientific and Technical Information of China (English)

    王秋鹏; 虞义建; 王教辰; 翁寿向; 甘梅富

    2014-01-01

    The clinicopathological data of 13 patients with Langerhans cell histiocytosis (LCH) from January 2004 to January 2014 were retrospectively analyzed.There were 8 males and 5 females with a mean age of 35 (6-63)years.For all 13 cases,there were 15 lesions.Histologically,there was a diffuse distribution of Langerhans cells accompanied by a variable number of eosinophils and some other inflammatory cells.Immunohistochemical study showed tumor cells were positive for S-100 protein,CD1a and Langerin.During a follow-up period of 6-101 months,all survived.The diagnosis of LCH was assisted by immunophenotypic analyses for S-100,CD1a and Langerin.Combination therapy was effective.Most patients had an excellent prognosis.%回顾性分析2004年1月至2014年1月收治的13例朗格汉斯细胞组织细胞增生症患者临床病理资料,其中男性8例,女性5例,患者年龄6 ~63岁,平均年龄35岁,多为成年人.13例患者共有15处病变,镜下见组织细胞样瘤细胞弥漫分布,可见核沟,背景内有数量不等嗜酸性粒细胞及其他炎细胞浸润.免疫组化结果显示肿瘤细胞表达S-100、CD1a、朗格汉斯细胞产生的特异性凝集素.治疗后随访6~101个月,均健在.免疫组化标记有助于明确诊断及鉴别诊断.

  19. BRAF V600E mutation correlates with suppressive tumor immune microenvironment and reduced disease-free survival in Langerhans cell histiocytosis

    Science.gov (United States)

    Zeng, Kaixuan; Wang, Zhe; Ohshima, Koichi; Liu, Yixiong; Zhang, Weichen; Wang, Lu; Fan, Linni; Li, Mingyang; Li, Xia; Wang, Yingmei; Yu, Zhou; Yan, Qingguo; Guo, Shuangping; Wei, Jie; Guo, Ying

    2016-01-01

    ABSTRACT Langerhans cell histiocytosis (LCH) is a neoplasm of myeloid origin characterized by a clonal proliferation of CD1a+/CD207+ dendritic cells. Recurrent BRAF V600E mutation has been reported in LCH. In the present report, we confirm the feasibility of the high-specificity monoclonal antibody VE1 for detecting BRAF V600E mutation in 36/97 (37.1%) retrospectively enrolled patients with LCH; concordant immunohistochemistry and Sanger sequencing results were seen in 94.8% of cases. We then assessed the tumor immune microenvironment status in LCH, and found that the GATA binding protein 3 (GATA3)+/T-bet+ ratio could distinguish between clinical multi-system/single-system (SS) multifocal and SS unifocal LCH. Notably, we found that BRAF V600E mutation is significantly correlated with increased programmed cell death 1 ligand 1 (PDL1) expression and forkhead box protein 3 (FOXP3)+ regulatory T cells (p < 0.001, 0.009, respectively). Moreover, Cox multivariate survival analysis showed that BRAF V600E mutation and PDL1 were independent prognostic factors of poor disease-free survival (DFS) in LCH (hazard ratio [HR] = 2.38, 95% confidence interval [CI] 1.02–5.56, p = 0.044; HR = 3.06, 95%CI 1.14–7.14, p = 0.025, respectively), and the superiority of PDL1 in sensitivity and specificity as biomarker for DFS in LCH was demonstrated by receiver operator characteristic (ROC) curves when compared with BRAF V600E and risk category. Collectively, this study identifies for the first time relationship between BRAF V600E mutation and a suppressive tumor immune microenvironment in LCH, resulting in disruption of host–tumor immune surveillance, which is DFS. Our findings may provide a rationale for combining immunotherapy and BRAF-targeted therapy for treating patients with BRAF V600E mutant LCH. PMID:27622040

  20. Hemosiderotic clear-cell acanthoma: A pigmented mimicker

    Directory of Open Access Journals (Sweden)

    Leonardo Bugatti

    2011-01-01

    Full Text Available The authors report on a case of a 65-year-old man with pigmented clear-cell acanthoma located on the right thigh. Dermoscopy disclosed a peculiar picture consisting of diffuse black pigmentation with a superficial greyish veil in the central portion, dotted-to-globular dark red-black structures mainly located at the periphery with a homogenous regular reticular arrangement; peripheral translucid desquamation. Dermoscopic features are correlated with the histology, where hemosiderin deposits present in a sheet-like arrangement in the perivascular papillary dermis and in a band-like disposition in the reticular dermis at the base of the lesion can account for the pigmented picture. The lesion arose on a trauma-prone skin site; thus the authors believe that traumatic irritation may be responsible for the clinical and dermoscopic pictures, giving rise to a reaction similar in a way to the Auspitz′s sign provocated by trauma for psoriasis. Red blood cells extravasation from extremely superficialized capillaries may have led to hemosiderin deposition in the papillary and the reticular dermis.

  1. Clear cell chondrosarcoma mimicking chondroblastoma in a skeletally immature patient

    Energy Technology Data Exchange (ETDEWEB)

    Cannon, Christopher P. [Department of Orthopaedic Surgery, Madigan Army Medical Center, Ft. Lewis, WA (United States); Nelson, Scott D. [Department of Pathology and Laboratory Medicine, University of California, Los Angeles School of Medicine, CA (United States); Seeger, Leanne L. [Department of Radiological Sciences, University of California, CA (United States); Eckardt, Jeffrey J. [Department of Orthopaedic Surgery, University of California, Los Angeles School of Medicine, CA (United States)

    2002-06-01

    We report the case of a clear cell chondrosarcoma (CCCS) occurring in the femoral head of a 14-year-old skeletally immature boy. Radiographic examination revealed a well-defined, osteolytic lesion in the epiphysis of the femoral head. Given the patient's age and the radiographic appearance of the lesion, chondroblastoma was high on the differential diagnosis. A frozen section was performed at the time of open biopsy was felt to be consistent with either chondroblastoma or CCCS. CCCS in a skeletally immature patient was felt to be unlikely, so curettage and bone grafting was performed. Final pathology review, however, confirmed the diagnosis of CCCS. The patient was taken back to surgery 4 weeks later for a wide resection and hemiarthroplasty. (orig.)

  2. Literature review of the treatment of Langerhans cell histiocytosis%骨骼朗格汉斯细胞增生症的治疗进展

    Institute of Scientific and Technical Information of China (English)

    杜鑫辉; 董扬; 杨庆诚

    2012-01-01

    @@ 朗格汉斯细胞增生症是以朗格汉斯细胞异常累积为特征的疾病,其发病原因尚不明确,发病率约为1∶1 500 000.患者以儿童为主,男性多见.根据朗格汉斯细胞增生症病灶累及部位及临床表现不同可分为嗜酸性肉芽肿,汗雪氏病(Hand-Schüller-Christian disease)及里斯氏病(Letterer-Siwe disease).%Langerhans cell hisdocytosis (LCH) is a poorly understood disease, with different patterns of clinical presentation. There are many methods concerning the treatment of Langerhans cell hisdocytosis, but the optimal choice is still to be argued. Here we present a literature review of all The papers included by WEB of KNOWLEDGE of the late 10 years on the clinical features, diagnostic methods and especially, the treatment of Langerhans cell histiocytosis. Based on the location of the lesion and clinical features, LCH can be classified into eosinophilic granuloma. Hand-Schillier-Christian disease and Letterer-Siwc disease. The symptoms vary according to the affected lesion, which can be asymptomatic or pain. LHC can affect any bone of the body, usually appearing as a radiolucent area with a sclerosis in the diaphysis or metaphysis of long bone. LHC of the spine often presents with, vertebra body destruction and compression. The radiographic studies could provide evidence that indicating the diagnosis of LHC, the final decision is usually made by pathologist by studying the biopsy specimen. The treatment of LHC- includes chemotherapy, radiotherapy, intralesional injection of steroids and surgery. Each method lias its own indications and the treatment of patients should be individualized.

  3. The Progress of Diagnosis and Treatment of Langerhans Cell Histiocytosis%朗格汉斯细胞组织细胞增生症诊疗进展

    Institute of Scientific and Technical Information of China (English)

    袁聪聪; 陈渟桑; 高景; 邢丽华

    2015-01-01

    Langerhans Cell Histiocytosis(LCH)is a rare disease caused by the proliferation and infiltration of Langerhans cells. LCH was reported in many countries in all age group all over the world. The etiology research of LCH is unclear,whether it is a reactive rather than a neoplastic disorder remains unanswered. The current gold standard for the diagnosis of LCH is the positivity of CD1a and/or Langerin(or BG)in the lesions. The supportive treatment,chemotherapy,stem cell transplant and organ transplant are the main approaches target LCH,and systemic treatment still undefined.%朗格汉斯细胞组织细胞增生症(LCH)是一种有朗格汉斯细胞克隆增殖和移行引起的罕见疾病。LCH 在世界许多国家均有报道,各年龄段均有可能患病。病因尚不明确,是由反应性紊乱还是肿瘤性紊乱引起尚未可知。诊断的金标准是损伤中出现 CD1a 和/或Langerin(或 BG)阳性。常采用支持疗法、化疗、干细胞移植和器官移植等手段,但目前还没有系统性的治疗方案。

  4. "Pure" cutaneous histiocytosis-X.

    Science.gov (United States)

    Wolfson, S L; Botero, F; Hurwitz, S; Pearson, H A

    1981-11-15

    The case histories of two young children who experienced skin rashes involving various areas of the body are reported. The diagnosis of pure cutaneous histiocytosis-X was established after extensive studies revealed no other organ involvement. The patients were treated with oral corticosteroids. Currently, both children are in good health, show no evidence of disease, and have been followed over a four-to-five-year period. Therapy with corticosteroids may not be indicated with pure cutaneous histiocytosis-X unless there is evidence of extracutaneous dissemination or rapid progression of the disease.

  5. Ischiogluteal bursitis mimicking soft-tissue metastasis from a renal cell carcinoma

    Energy Technology Data Exchange (ETDEWEB)

    Voelk, M.; Gmeinwieser, J.; Manke, C.; Strotzer, M. [Department of Radiology, University Hospital, Regensburg (Germany); Hanika, H. [Department of Urology, St. Josef Hospital, Regensburg (Germany)

    1998-09-01

    We report a case of ischiogluteal bursitis mimicking a soft-tissue metastasis from a renal cell carcinoma. A 66-year-old woman suffered from pain over the left buttock 6 months after she was operated on for renal cell carcinoma of the left kidney. CT of the abdomen and pelvis revealed a tumor-like lesion adjacent to the left os ischii, which was suspected to be a soft-tissue metastasis. Percutaneous biopsy revealed no evidence of malignancy, but the histopathological diagnosis of chronic bursitis. (orig.) With 2 figs., 8 refs.

  6. 朗格汉斯细胞组织细胞增生症治疗进展%Progress in the treatment of Langerhans cell histiocytosis

    Institute of Scientific and Technical Information of China (English)

    泥永安(综述); 孙立荣(审校)

    2015-01-01

    Treatment of Langerhans cell histiocytosis (LCH) needs to be tailored for each individual patient according to LCH classiifcation currently. Single-system LCH (SS-LCH) has an excellent prognosis. However, there is a poor prognosis in multisystem LCH (MS-LCH) with risk organs (RO) involvement and refractory or recurrent LCH (Re-LCH). The prognosis of MS-LCH with RO involvement and Re-LCH has been improved markedly accompanying with progress of chemotherapy in recent years. The 5-year survival rate of MS-LCH reached above 80%, and the effective rate of Re-LCH reached above 60% after chemotherapy. Re-LCH can be cured by hematopoietic stem cell transplantation.%目前朗格汉斯细胞组织细胞增生症(LCH)在治疗上采用分型、分级而分治的策略。研究表明,单系统病变(SS-LCH)的预后好,而伴危险器官受累的多系统病变(MS-LCH)及难治性/复发性病例(Re-LCH)预后差。随着化学疗法的进步,伴危险器官受累的MS-LCH 5年生存率已达80%以上,Re-LCH的治疗有效率达60%以上,Re-LCH可行造血干细胞移植以达到根治。

  7. Clinical Features of Langerhans Cell Histiocytosis of Temporal Bone%颞骨朗格汉斯组织细胞增生症临床分析

    Institute of Scientific and Technical Information of China (English)

    李晓红; 韩维举

    2015-01-01

    Objective Temporal bone Langerhans Cell Histiocytosis (LCH) is mainly characterized by otorrhea, mas⁃toid mass, granuloma in the middle ear and external auditory canal, and hearing loss,while facial paralysis is rare. This study is aimed at analyzing clinical and prognostic features of temporal bone LCH. Methods Clinical data from 16 cases of LCH of the temporal bone treated from July 2009 to July 2014 at the Chinese PLA General Hospital were reviewed, including fol⁃lowing ups clinical presentation, ,The 2009 International Histocyte Society guidelines for diagnosis, clinical classification and outcome evaluation were adopted in the analysis. Correlation between age and clinical classifications and prognosis was also analyzed. Results Among the 16 patients, 10 were children (≤4 years old) and 6 were adults (24-68 years old). Multiple system involvement was seen in 4 of the 16 patients, and the rest patients showed only single system involvement. While age was not correlated to treatment outcomes, clinical classification appeared to be correlated to the prognosis. Conclusion The number of organ affected by LCH may be a good prognosis indicator in these patients.%目的:分析颞骨朗格汉斯组织细胞增生症(Langerhans cell histiocytosis,LCH)的临床特点、诊断、治疗和预后。方法回顾性收集解放军总医院耳鼻咽喉头颈外科2009年6月至2014年6月收治并经病理证实的颞骨LCH作为研究对象,按2009年国际组织细胞协会提出的诊断标准、临床分类和疗效评定标准,分析其临床表现、诊断、治疗及预后等临床特点,根据年龄、临床分类分组,比较组间预后差异。结果本组颞骨LCH主要表现为耳漏、耳聋、耳颞部包块、肉芽肿等;16例患者中,幼儿组10例(年龄≤4岁),成人组6例(年龄24岁~68岁),经统计分析组间预后差异无统计学意义。按国际组织细胞协会的分类,多系统组4例,单系统组12

  8. 郎格罕组织细胞增生症26例临床分析%Clinical analysis of 26 cases of langerhans cell histiocytosis

    Institute of Scientific and Technical Information of China (English)

    陆耀红; 傅深; 彭莉华; 孙宜; 李兆斌; 邵雨卉

    2009-01-01

    目的:郎格罕组织细胞增生症( Langerhans cell histiocytosis, LCH),临床表现复杂多样,容易误诊.本研究在于了解其临床特征,提高对该病的认识,减少误诊率.方法:回顾性分析本院2003年1月-2007年7月确诊的26例LCH患者的临床表现、影像、病理资料及治疗方法.结果:LCH侵犯的组织器官有骨骼20例(76.92%)、淋巴结2例(7.69%)、肺、脾、耳、肌肉各2例,肝、胃、垂体、鼻腔、口腔各1例;侵犯1个器官23例(88.46%),侵犯2个器官1例(3.85%),侵犯4个器官1例(3.85%),侵犯6个器官1例(3.85%).临床表现多样化,症状与影像学缺乏特异性表现,确诊依靠病理诊断.单发病灶以手术、放疗为主.局部治疗效果好.结论:LCH临床表现多样,误诊率高.多系统病变采取化疗,单发病灶者手术及放疗疗效好.

  9. Diagnósticos diferenciales de la histiocitosis a células de Langerhans The differential diagnostics of Langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    C. N. Chirino

    2007-06-01

    Full Text Available La histiocitosis a células de Langerhans (HCL debe diferenciarse de las siguientes entidades: eritema tóxico neonatorum (ETN, dermatitis seborreica (DS, foliculitis pustulosa eosinofílica (FPE, incontinencia pigmenti (IP, mastocitosis/urticaria pigmentosa (M/UP, acrodermatitis enteropática (ADE, síndrome de Wiskott-Aldrich (WAS, acropustulosis infantil (API. Además se deben considerar la enfermedad de Rosai- Dorfman (ERD, xantomas diseminados, melanosis pustulosa neonatal (MPN, candidiasis congénita, listeriosis neonatal, herpes simple perinatal y la varicela neonatal. Debido a que los métodos auxiliares de laboratorio no siempre están disponibles o los resultados laboratoriales algunas veces son extemporáneos, y puesto que el médico práctico a menudo necesita tomar decisiones precozmente, es que la epidemiología resulta útil, pues brinda el marco adecuado para ordenar y jerarquizar las sospechas diagnósticas frente a un caso concreto, con un paciente determinado, en un momento específico.The differential diagnostics of Langerhans cell histiocytosis should include the following disorders: erythema toxicum neonatorum, seborrheic dermatitis, eosinophilic pustular folliculitis, incontinentia pigmenti, mastocytosis / urticaria pigmentosa, acrodermatitis enteropathica, Wiskott-Aldrich syndrome, infantile acropustulosis, Rosai- Dorfman disease, xanthoma disseminatum, neonatal pustular melanosis, congenital candidiasis, perinatal listeriosis, perinatal herpes simplex, neonatal varicella. Since the auxiliary methods of lab are not always available, or lab results are sometimes extemporaneous, the physicians often needs to make quick decisions. The epidemiology is useful because it offers the appropriate mark to prioritize the diagnostic in specific cases

  10. 难治性儿童朗格汉斯组织细胞增生症的治疗进展%Treatment of childhood refractory Langerhans cells histiocytosis

    Institute of Scientific and Technical Information of China (English)

    吴方方

    2016-01-01

    The definition of refractory Langerhans cells histiocytosis (LCH) is risk organs (bone marrow, liver, spleen)involvement and the first-line treatment failure.It has a very poor prognosis.The survival depends on timely and effective salvage treatment.Currently the treatment for refractory LCH includes isolated 2-chlorodeoxyadenosine, 2-chlorodeoxyadenosine combined with high-dose cytarabine, indomethacin, hematopoietic stem cell transplantation and targeted therapy.In this paper, the advances in treatment of the refractory LCH in children were reviewed.%难治性朗格汉斯组织细胞增生症(Langerhans cell histiocytosis,LCH)是指具有多脏器浸润尤其是具有危险器官(骨髓、肝、脾)浸润和初治治疗失败的LCH病例,预后极差,及时有效的治疗是患者存活的关键.目前有单药克拉屈滨(2-chlorodeoxyadenosine,2-CdA)、2-CdA联合大剂量阿糖胞苷、消炎痛、造血干细胞移植、靶向治疗等多种二线治疗方案.该文就近年儿童难治性LCH的相关治疗方案进展进行综述.

  11. Fasting-Mimicking Diet Reduces HO-1 to Promote T Cell-Mediated Tumor Cytotoxicity.

    Science.gov (United States)

    Di Biase, Stefano; Lee, Changhan; Brandhorst, Sebastian; Manes, Brianna; Buono, Roberta; Cheng, Chia-Wei; Cacciottolo, Mafalda; Martin-Montalvo, Alejandro; de Cabo, Rafael; Wei, Min; Morgan, Todd E; Longo, Valter D

    2016-07-11

    Immune-based interventions are promising strategies to achieve long-term cancer-free survival. Fasting was previously shown to differentially sensitize tumors to chemotherapy while protecting normal cells, including hematopoietic stem and immune cells, from its toxic side effects. Here, we show that the combination of chemotherapy and a fasting-mimicking diet (FMD) increases the levels of bone marrow common lymphoid progenitor cells and cytotoxic CD8(+) tumor-infiltrating lymphocytes (TILs), leading to a major delay in breast cancer and melanoma progression. In breast tumors, this effect is partially mediated by the downregulation of the stress-responsive enzyme heme oxygenase-1 (HO-1). These data indicate that FMD cycles combined with chemotherapy can enhance T cell-dependent targeted killing of cancer cells both by stimulating the hematopoietic system and by enhancing CD8(+)-dependent tumor cytotoxicity.

  12. Histiocitose das células de Langerhans: experiência de 16 anos Langerhans cell histiocytosis: a 16-year experience

    Directory of Open Access Journals (Sweden)

    Márcia Kanadani Campos

    2007-02-01

    Full Text Available OBJETIVOS: Descrever a apresentação clínica da histiocitose das células de Langerhans e comparar sua evolução de acordo com a idade, estadiamento e resposta ao tratamento. MÉTODOS: Análise retrospectiva dos dados referentes a 33 crianças com histiocitose das células de Langerhans acompanhadas no Hospital das Clínicas da Universidade Federal de Minas Gerais no período de 1988 a 2004. RESULTADOS: A idade ao diagnóstico variou de 2 meses a 16 anos (mediana: 2,5 anos. Dezessete crianças eram do sexo masculino. O tempo de seguimento variou de 21 dias a 16,2 anos (mediana: 3,4 anos. As manifestações clínicas mais comuns ao diagnóstico foram lesões osteolíticas, linfadenomegalia e lesões cutâneas. A sobrevida global para todo o grupo foi de 86,1% aos 16 anos (IC95% 66,6-94,6. Os óbitos ocorreram em pacientes com doença multissistêmica e disfunção orgânica ao diagnóstico. Os pacientes que apresentaram resposta "melhor" à sexta semana de tratamento apresentaram uma probabilidade estimada de sobrevida global significativamente maior em relação aos que apresentaram progressão da doença. A sobrevida global foi significativamente maior para os pacientes com doença em um único sistema. A probabilidade de sobrevida livre de eventos para todo o grupo foi de 30,9% aos 16 anos (IC95% 15,6-47,5, sendo significativamente maior para os portadores de doença em um único sistema. A idade não se associou com a sobrevida livre de eventos. A seqüela mais comum foi o diabetes insipidus. Não foram observados casos de neoplasias secundárias. CONCLUSÃO: A histiocitose das células de Langerhans apresenta uma grande variedade de manifestações clínicas, com alta taxa de recidivas e baixa taxa de mortalidade.OBJECTIVES: To describe the clinical course of Langerhans cell histiocytosis and to compare its outcome according to age, staging of the disease and treatment response. METHODS: Retrospective analysis of data on 33 children with

  13. 颞骨朗格汉斯细胞组织细胞增生症一例报道%TemPoral Bone Langerhans Cell Histiocytosis:RePort of One Case

    Institute of Scientific and Technical Information of China (English)

    臧健; 姜学钧

    2014-01-01

    Langerhans cell histiocytosis,a rare disease,often occurs in skull,pelvis,ribs etc. The literatures re-porting it is not many at home or abroad. This paper reported 1 case of langerhans cell histiocytosis in temporal bone and intro-duces its clinical features,imaging findings,pathological characteristics,treatment and prognosis to improve physicians' knowl-edge about it.%朗格汉斯细胞组织细胞增生症是一种较罕见的疾病,目前国内外文献报道较少。该病最常见的发病部位为骨骼,如颅骨、盆骨、肋骨等。本文结合1例发生在颞骨的朗格汉斯细胞组织细胞增生症患者的临床资料,就该病的临床特征、影像学表现、病理特点以及治疗和预后情况进行介绍,以期加强临床医师对该病的认识。

  14. Congenital self healing Langerhans cell histiocytosis%先天性自愈性朗格汉斯组织细胞增生症

    Institute of Scientific and Technical Information of China (English)

    皮肖冰; 王晓霞

    2011-01-01

    报告1例先天性自愈性朗格汉斯组织细胞增生症.患儿男,2个月.出生时即有全身多发性皮肤结节,皮肤科检查:左耳后、左面部、胸部、背部、左下肢、右示指共6处鲜红色结节,中央形成火山口状溃疡,组织病理示结节由梭形细胞构成,部分细胞胞质丰富,嗜酸性,可见多角、多核瘤巨细胞,间质纤维丰富,局部瘤细胞增生活跃.免疫组化:CD1a(+),S-100蛋白(±),CD68(-),切除5处皮肤结节,右示指处结节未切除,在10个月内自行消失,结合患儿临床症状及随访结果诊断为先天性自愈性朗格汉斯组织细胞增生症.%A 2-month-old male was born with 6 nodules on the left posterior auris, left face, the breast, the back ,the left leg and right index finger.Physical examination showed red nodules with central crateriform ulceration.Histopathological examination revealed spindle cells, some were mononuclear histiocytes with abundant eosinophilic or foamy cytoplasm.Polynucleart tumor giant cells and abundant interstitial substance were seen in the dermis.These mononuclear histiocytoid cells were stained positive for S-100 protein and CD1a negative for CD 68.5 nudules were excised, and spontaneous resolving occurred to the remaining nodule in the right index finger within 10 months.The diagnosis of congenital self-healing Langerhans cell histiocytosis was finally established.

  15. Mimicking cellular transport mechanism in stem cells through endosomal escape of new peptide-coated quantum dots

    Science.gov (United States)

    Narayanan, Karthikeyan; Yen, Swee Kuan; Dou, Qingqing; Padmanabhan, Parasuraman; Sudhaharan, Thankiah; Ahmed, Sohail; Ying, Jackie Y.; Selvan, Subramanian Tamil

    2013-07-01

    Protein transport is an important phenomenon in biological systems. Proteins are transported via several mechanisms to reach their destined compartment of cell for its complete function. One such mechanism is the microtubule mediated protein transport. Up to now, there are no reports on synthetic systems mimicking the biological protein transport mechanism. Here we report a highly efficient method of mimicking the microtubule mediated protein transport using newly designed biotinylated peptides encompassing a microtubule-associated sequence (MTAS) and a nuclear localization signaling (NLS) sequence, and their final conjugation with streptavidin-coated CdSe/ZnS quantum dots (QDs). Our results demonstrate that these novel bio-conjugated QDs enhance the endosomal escape and promote targeted delivery into the nucleus of human mesenchymal stem cells via microtubules. Mimicking the cellular transport mechanism in stem cells is highly desirable for diagnostics, targeting and therapeutic applications, opening up new avenues in the area of drug delivery.

  16. Clinicopathologic analysis of Langerhans cell histiocytosis%眼眶朗格罕细胞组织细胞增生症临床病理分析

    Institute of Scientific and Technical Information of China (English)

    柳晓辉; 李晓华; 袁敏; 尹瑞杰; 马秋飞

    2014-01-01

    Objective To investigate the clinical and pathological features of Langerhans cell histiocytosis (LCH).Methods In this retrospective study,the clinical data and histopathological features of LCH patients treated in Henan Eye Hospital were collected and analyzed.Results There were 7 cases,including 2 males and 5 females.Mean age was 4.07 years old.Images of CT were easy to find osteolysis.Histopathology could find typical Langerhans cell.Tissues were accompanied by significant bleeding.Conclusions Because of its various clinical manifestations and prognosis,pathological biopsy is essential to some paediatric patients.%目的 探讨眼眶朗格罕细胞组织细胞增生症的组织病理学表现与临床诊治,提高对该病的认识.方法 收集2008年1月至2011年12月在河南省立眼科医院确诊的朗格罕细胞组织细胞增生症患者7例(7只眼),对其临床诊治资料及组织病理学表现进行回顾性分析.结果 眼眶朗格罕细胞组织细胞增生症患儿共7例(7只眼),其中男2例、女5例,平均年龄4.07岁.CT检查易发现溶骨性骨质破坏,组织病理学可发现典型的朗格罕细胞组织细胞,组织均伴有明显的出血.结论 眼眶朗格罕细胞组织细胞增生症多见于儿童,临床表现多样,病情轻重不一,对于某些患者必要时行手术活检病理检查,有利于诊断治疗.

  17. Stenotrophomonas maltophilia with histopathological features mimicking cutaneous gamma/delta T-cell lymphoma

    Directory of Open Access Journals (Sweden)

    Natalie Kash

    2015-01-01

    Full Text Available We report a case of cutaneous Stenotrophomonas maltophilia infection which presented with clinical and histopathological findings that mimicked a gamma/delta (γδ T-cell lymphoma. In this case, tissue culture of the biopsy specimen was key to determining the diagnosis and allowing appropriate treatment with oral trimethoprim–sulfamethoxazole and topical silvadene. A prompt complete resolution of lesions was observed following antibiotic treatment, with no recurrence of disease over the last 5 years, supporting an infectious rather than malignant etiology. In our patient, radiation therapy was indicated based on the misdiagnosis of γδ T-cell lymphoma, which was supported both clinically and histopathologically. However, tissue culture in this case avoided unnecessary radiation exposure and highlights the role of tissue culture in the evaluation of the biopsy of an undiagnosed cutaneous lesion.

  18. [Generalized granuloma annulare or diffuse dermal histiocytosis?].

    Science.gov (United States)

    Kretzschmar, L; Biel, K; Luger, T A; Goerdt, S

    1995-08-01

    Generalized granuloma annulare is a rare variant of granuloma annulare affecting the trunk and extremities with a multitude of lesions. In contrast to localized granuloma annulare, generalized granuloma annulare occurs in older patients, shows a stronger association with diabetes, and is characteristically chronic. Like our 55-year-old patient, most patients present with papules and annular plaques; less often, macular or non-annular lesions may be encountered. Histology often fails to show necrobiotic or necrotic connective tissue changes demarcated by a palisading granuloma. Instead, there are diffuse dermal, band-like or nodular aggregations of histiocytes intermingled with some multinucleated giant cells and a predominantly lymphocytic infiltrate in the periphery. Because of its special characteristics, it has been suggested that generalized granuloma annulare might constitute a separate disease entity and that it should be classed among the primary cutaneous histiocytoses as a diffuse dermal histiocytosis. Using immunohistochemistry to determine the macrophage phenotype of the lesional histiocytes, we have shown that generalized granuloma annulare is not a cutaneous histiocytosis. Neither MS-1 high-molecular-weight protein, a new specific marker for cutaneous non-Langerhans cell histiocytoses, nor CD1a, the well-known marker for Langerhans cells and Langerhans cell histiocytoses, is expressed by the lesional histiocytes of our patient. In contrast, the antigen expression pattern was diagnostic for non-infectious granulomas and was highly similar to that in localized granuloma annulare. In contrast to the successful treatment of localized granuloma annulare reported with intralesional interferon beta-1, systemic treatment with interferon alpha-2b (9 x 10(6) units three times a week) was ineffective.

  19. CT manifestations of pulmonary Langerhans cell histiocytosis%肺郎格汉斯细胞组织细胞增生症的CT表现

    Institute of Scientific and Technical Information of China (English)

    强军; 余卫; 高万勤; 宋海乔; 马英剑

    2010-01-01

    Objective To analyzes the CT manifestations of pulmonary Langerhans cell histiocytosis (PLCH). Methods CT features of 11 patients with PLCH proved pathologically were analyzed retrospectively. Results The main findings in 11 PLCHs were cysts and nodules. Two cases only had cysts,and 1 only had nodules, which most had cavitations. The other 8 cases showed cysts and nodules with 4 cases mainly manifested with cysts and nine mainly manifested with nodules. Two cases had pulmonary interstitial changes. One case only had cysts in the left upper lung field and 10 cases had lesions not only in the upper and middle but in the lower lung field, which 2 cases had more lesions in the lower and costophrenic angle field and 8 cases had less lesions in the costo-phrenic angle field. Three of these 8 cases had more lesions in the superior lobe and apical segment of lower lobe. Conclusion CT manifestations of PLCH are helpful for the early diagnosis.%目的 分析肺郎格汉斯细胞组织细胞增生症(PLCH)的CT表现.方法 11例患者均行CT平扫,其中8例加HRCT扫描.11例均经病理确诊为PLCH.结果 11例PLCH的主要CT表现为囊腔和结节.2例仅表现为囊腔,1例仅表现为结节且多数结节伴空洞;余8例为囊腔与结节影合并存在,其中4例以囊腔为主要表现,无一例以结节影为主,2例出现间质性改变.病变的分布:1例仅有囊腔且位于左上肺;10例上、中、下肺均可见,其中2例以下肺及肋膈角区明显;3例以上叶和下叶背段明显,中叶及舌叶病变相对较轻;8例肋膈角区相对较轻.结论 PLCH的CT表现有一定的规律和特征,熟悉这些表现,有助于对该病的早期诊断.

  20. A Novel Chip for Cyclic Stretch and Intermittent Hypoxia Cell Exposures Mimicking Obstructive Sleep Apnea

    Directory of Open Access Journals (Sweden)

    Noelia Campillo

    2016-07-01

    Full Text Available Intermittent hypoxia (IH, a hallmark of obstructive sleep apnea (OSA, plays a critical role in the pathogenesis of OSA-associated morbidities, especially in the cardiovascular and respiratory systems. Oxidative stress and inflammation induced by IH are suggested as main contributors of end-organ dysfunction in OSA patients and animal models. Since the molecular mechanisms underlying these in vivo pathological responses remain poorly understood, implementation of experimental in vitro cell-based systems capable of inducing high-frequency IH would be highly desirable. Here, we describe the design, fabrication and validation of a versatile chip for subjecting cultured cells to fast changes in gas partial pressure and to cyclic stretch. The chip is fabricated with polydimethylsiloxane (PDMS and consists of a cylindrical well covered by a thin membrane. Cells cultured on top of the membrane can be subjected to fast changes in oxygen concentration (equilibrium time 6 s. Moreover, cells can be subjected to cyclic stretch at cardiac or respiratory frequencies independently or simultaneously. Rat bone marrow-derived mesenchymal stem cells (MSCs exposed to IH mimicking OSA and cyclic stretch at cardiac frequencies revealed that hypoxia-inducible factor 1α (HIF-1α expression was increased in response to both stimuli. Thus, the chip provides a versatile tool for the study of cellular responses to cyclical hypoxia and stretch.

  1. A Novel Chip for Cyclic Stretch and Intermittent Hypoxia Cell Exposures Mimicking Obstructive Sleep Apnea

    Science.gov (United States)

    Campillo, Noelia; Jorba, Ignasi; Schaedel, Laura; Casals, Blai; Gozal, David; Farré, Ramon; Almendros, Isaac; Navajas, Daniel

    2016-01-01

    Intermittent hypoxia (IH), a hallmark of obstructive sleep apnea (OSA), plays a critical role in the pathogenesis of OSA-associated morbidities, especially in the cardiovascular and respiratory systems. Oxidative stress and inflammation induced by IH are suggested as main contributors of end-organ dysfunction in OSA patients and animal models. Since the molecular mechanisms underlying these in vivo pathological responses remain poorly understood, implementation of experimental in vitro cell-based systems capable of inducing high-frequency IH would be highly desirable. Here, we describe the design, fabrication, and validation of a versatile chip for subjecting cultured cells to fast changes in gas partial pressure and to cyclic stretch. The chip is fabricated with polydimethylsiloxane (PDMS) and consists of a cylindrical well-covered by a thin membrane. Cells cultured on top of the membrane can be subjected to fast changes in oxygen concentration (equilibrium time ~6 s). Moreover, cells can be subjected to cyclic stretch at cardiac or respiratory frequencies independently or simultaneously. Rat bone marrow-derived mesenchymal stem cells (MSCs) exposed to IH mimicking OSA and cyclic stretch at cardiac frequencies revealed that hypoxia-inducible factor 1α (HIF-1α) expression was increased in response to both stimuli. Thus, the chip provides a versatile tool for the study of cellular responses to cyclical hypoxia and stretch. PMID:27524971

  2. 郎格罕细胞组织细胞增生症发病机制及诊治进展%Progress in Pathogenesis, Diagnosis and Treatment of Langerhans Cell Histiocytosis

    Institute of Scientific and Technical Information of China (English)

    隆毅

    2012-01-01

    郎格罕细胞组织细胞增生症(Iangerhans Cell Histiocytosis,LCH)是郎格罕细胞异常增生引起的一组疾病.本病多发生于儿童时期,以婴幼儿时期多见.据报道,英国和爱尔兰每年发病率为4.2/1 000 000[1].男性发病多于女性,我国男女发病比为(1.4~3.4)∶1.本病病因及发病机制尚不完全清楚.由于临床表现多样,误诊率高.LCH常致多系统受累,预后不佳.现就本病发病机制及诊治进展做一综述.

  3. Whole-body MRI in children with Langerhans cell histiocytosis for the evaluation of the skeletal system; Ganzkoerper-MRT bei Langerhans-Zell-Histiozytose im Kindes- und Jugendalter zur Beurteilung des Skelettsystems

    Energy Technology Data Exchange (ETDEWEB)

    Steinborn, M. [Inst. fuer Diagnostische und Interventionelle Radiologie, Abt. Kinderradiologie, Staedtisches Klinikum Muenchen GmbH, Klinikum Schwabing (Germany); Woertler, K.; Rummeny, E.J. [Inst. fuer Roentgendiagnostik der Technischen Univ. Muenchen, Klinikum Rechts der Isar (Germany); Nathrath, M. [Abt. fuer Paediatrische Onkologie und Haematologie, Kinderklinik und Poliklinik des Klinikums Rechts der Isar der Technischen Univ. Muenchen, Kinderklinik Schwabing (Germany); KKG Osteosarkom, Helmholtz Zentrum, Muenchen (Germany); Schoeniger, M. [Abt. fuer Paediatrische Onkologie und Haematologie, Kinderklinik und Poliklinik des Klinikums Rechts der Isar der Technischen Univ. Muenchen, Kinderklinik Schwabing (Germany); Hahn, H. [KKG Osteosarkom, Helmholtz Zentrum, Muenchen (Germany)

    2008-07-15

    Purpose: the usefulness of whole-body MRI (WB-MRI) for the detection of skeletal lesions in patients with Langerhans cell histiocytosis should be documented on the basis of case presentations. Materials and methods: in six patients with histologically proven Langerhans cell histiocytosis, 14 WB-MRI examinations were performed to evaluate the skeletal system within disease staging (6 primary, 8 follow-up examinations). The examinations were performed on a 1.5 Tesla, 32-channel whole-body scanner. The examination protocol consisted of T1-weighted and STIR sequences in coronal and sagittal orientation. For comparison, radiographs of the initial skeletal lesions and those that were additionally detected on WB-MRI were available. Results: in 4 patients no additional skeletal lesions were found on WB-MRI besides the initial lesion leading to the diagnosis of unifocal single system disease. In 2 patients WB-MRI was able to identify additional skeletal lesions. In a 5 1/2 year-old boy with the primary lesion located in the cervical spine, a second lesion was detected in the lumbar spine on the initial scan and in the skull and proximal femur during follow-up examination. In a 12 year-old girl with a primary lesion of the thoracic spine, WB-MRI diagnosed additional lesions in the pelvic bone and the tibia. In both patients the diagnosis of multifocal skeletal involvement led to chemotherapy. During follow-up examination, the healing response under therapy could be demonstrated. Comparison with conventional imaging showed that especially lesions located in the spine or the pelvis were not detectable on radiographs even when knowing the MR results. (orig.)

  4. Histiocitose de células de Langerhans em margem anal: relato de caso e revisão da literatura Perianal Langerhans cell histiocytosis: case report and literature review

    Directory of Open Access Journals (Sweden)

    Jaime Coelho Carlos Magno

    2007-03-01

    Full Text Available Um caso incomum de histiocitose de células de Langerhans comprometendo margem anal em adulto de cor branca com 34 anos de idade é descrito. Durante dezenove meses o paciente apresentou ulceração extensa em margem anal, dolorosa, com sangramento, evoluindo para incontinência fecal. A hipótese diagnóstica inicial ficou entre doença de Crohn, sífilis, tuberculose, pioderma gangrenoso e donovanose. O diagnóstico histopatológico, após a terceira biópsia, foi sugestivo de histiocitose X, diagnóstico esse confirmado pelo estudo imunoistoquímico positivo para CD1a e proteína S100. O paciente foi tratado com seis sessões de injeção intralesional de triancinolona e talidomida por via oral, durante três meses, evoluindo com remissão completa da lesão anal e recuperação da continência esfincteriana.A rare case of Langerhans cell histiocytosis with perianal involvement in a 34 year old white man is presented. During nineteen months this patient had a complaint of anal pain with bleeding, due to a large perianal ulcer. The initial diagnosis was Crohn's disease, anal tuberculosis, syphilis, pyoderma gangrenosum or donovanosis. After the third biopsy, the surgical specimens showed microscopic changes suggestives of Langerhans cell histiocytosis. The imunohistochemical study was positive to S100 protein and CD1a. The patient was treated with six doses of intralesional triancinolona and oral thalidomide for three months. Treatment was well tolerated and complete resolutions of peri-anal ulcer occurred.

  5. Histiocitosis de células de Langerhans localizada en hueso malar: Presentación de un caso Localized langerhans cell histiocytosis of the zygomatic bone: A case report

    Directory of Open Access Journals (Sweden)

    María Loreto Castellón Zirpel

    2011-09-01

    Full Text Available La histiocitosis de células de Langerhans localizada (HCLL, conocida como granuloma eosinófilo, representa entre el 50 y el 60% de todos los casos de histiocitosis de células de Langerhans. El tratamiento clásico para la HCLL ha sido el curetaje o la resección de las lesiones óseas. Hay publicaciones de casos tratados con inyección intralesional de corticosteroides, combinado con curetaje. Se presenta un caso clínico de un paciente de tres años de edad con diagnóstico de HCLL que compromete en su extensión el hueso malar, tratado con infiltraciones de corticosteroides y posterior curetaje de la lesión. A un año de realizado el tratamiento, el paciente se encuentra asintomático y con una regeneración ósea del hueso malar, evidenciable en la tomografía axial computarizada.Localized Langerhans cell histiocytosis (LLCH, also known as eosinophilic granuloma, represents 50 to 60% of all cases of Langerhans cell histiocytosis. The standard treatment for LLCH has been lesion curettage or resection. Cases treated with intralesional corticosteroid injections combined with curettage have been described. We report the case of a three-year-old patient diagnosed of LLCH with extensive zygomatic bone involvement, who was treated with corticosteroid infiltrations and subsequent curettage of the lesion. One year after treatment, the patient is asymptomatic with zygomatic reossification evidenced on computed tomography.

  6. Benign cephalic histiocytosis: report of four cases.

    Science.gov (United States)

    de Luna, M L; Glikin, I; Golberg, J; Stringa, S; Schroh, R; Casas, J

    1989-09-01

    We cared for four patients with benign cephalic histiocytosis, a self-healing non-X, nonlipid cutaneous histiocytosis of children. The age of onset of the disease was 5 to 9 months, with papules and erythematous macules involving the head (mainly the cheeks), and posterior spread to the trunk and limbs in three patients. Microscopic examination of skin biopsies revealed a histiocytic infiltrate in the superficial dermis that was S100 protein-negative by immunoperoxidase (PAP method). One patient showed comma-shaped bodies and desmosomelike junctions on electron microscopy. No Birbeck's granules were present. Benign cephalic histiocytosis is a self-limiting condition that requires no treatment.

  7. Pure cutaneous histiocytosis X of the vulva.

    Science.gov (United States)

    Rose, P G; Johnston, G C; O'Toole, R V

    1984-10-01

    Histiocytosis X of the vulva is extremely rare. In most reported cases, vulvar disease is associated with multiple organ involvement and systemic disease manifestations aiding in the diagnosis. Reported is a case of histiocytosis X presenting solely with vulvar lesions for eight years' duration. The diagnosis, pathology, and subsequent management are discussed. The literature of histiocytosis X of the vulva is reviewed, and its presentations and treatments summarized. Vulvar lesions unresponsive to therapy warrant biopsy and other causes of histiocytic reactions must be excluded to establish the diagnosis.

  8. Fasting-Mimicking Diet Promotes Ngn3-Driven β-Cell Regeneration to Reverse Diabetes.

    Science.gov (United States)

    Cheng, Chia-Wei; Villani, Valentina; Buono, Roberta; Wei, Min; Kumar, Sanjeev; Yilmaz, Omer H; Cohen, Pinchas; Sneddon, Julie B; Perin, Laura; Longo, Valter D

    2017-02-23

    Stem-cell-based therapies can potentially reverse organ dysfunction and diseases, but the removal of impaired tissue and activation of a program leading to organ regeneration pose major challenges. In mice, a 4-day fasting mimicking diet (FMD) induces a stepwise expression of Sox17 and Pdx-1, followed by Ngn3-driven generation of insulin-producing β cells, resembling that observed during pancreatic development. FMD cycles restore insulin secretion and glucose homeostasis in both type 2 and type 1 diabetes mouse models. In human type 1 diabetes pancreatic islets, fasting conditions reduce PKA and mTOR activity and induce Sox2 and Ngn3 expression and insulin production. The effects of the FMD are reversed by IGF-1 treatment and recapitulated by PKA and mTOR inhibition. These results indicate that a FMD promotes the reprogramming of pancreatic cells to restore insulin generation in islets from T1D patients and reverse both T1D and T2D phenotypes in mouse models. PAPERCLIP.

  9. GBT440 inhibits sickling of sickle cell trait blood under in vitro conditions mimicking strenuous exercise

    Directory of Open Access Journals (Sweden)

    Kobina Dufu

    2016-09-01

    Full Text Available In sickle cell trait (SCT, hemoglobin A (HbA and S (HbS are co-expressed in each red blood cell (RBC. While homozygous expression of HbS (HbSS leads to polymerization and sickling of RBCs resulting in sickle cell disease (SCD characterized by hemolytic anemia, painful vaso-occlusive episodes and shortened life-span, SCT is considered a benign condition usually with minor or no complications related to sickling. However, physical activities that cause increased tissue oxygen demand, dehydration and/or metabolic acidosis leads to increased HbS polymerization and life-threatening complications including death. We report that GBT440, an agent being developed for the treatment of SCD, increases the affinity of oxygen for Hb and inhibits in vitro polymerization of a mixture of HbS and HbA that simulates SCT blood. Moreover, GBT440 prevents sickling of SCT blood under in vitro conditions mimicking strenuous exercise with hypoxia, dehydration and acidosis. Together, our results indicate that GBT440 may have the potential to protect SCT individuals from sickling-related complications during conditions that favor HbS polymerization.

  10. Renal cell carcinoma with areas mimicking renal angiomyoadenomatous tumor/clear cell papillary renal cell carcinoma.

    Science.gov (United States)

    Petersson, Fredrik; Grossmann, Petr; Hora, Milan; Sperga, Maris; Montiel, Delia Perez; Martinek, Petr; Gutierrez, Maria Evelyn Cortes; Bulimbasic, Stela; Michal, Michal; Branzovsky, Jindrich; Hes, Ondrej

    2013-07-01

    We present a cohort of 8 renal carcinomas that displayed a variable (5%-95% extent) light microscopic appearance of renal angiomyoadenomatous tumor/clear cell papillary renal cell carcinoma (RAT/CCPRCC) without fulfilling the criteria for these tumors. All but 1 case predominantly (75%-95% extent) showed histopathologic features of conventional clear cell renal cell carcinoma. In 5 of 7 cases with mostly conventional clear renal cell carcinoma (CRCC) morphology, a diagnosis of CRCC was supported by the molecular genetic findings (presence of von Hippel-Lindau tumor suppressor [VHL] mutation and/or VHL promoter methylation and/or loss of heterozygosity [LOH] for 3p). Of the other 2 cases with predominantly characteristic CRCC morphology, 1 tumor did not reveal any VHL mutation, VHL promoter methylation, or LOH for 3p, and both chromosomes 7 and 17 were disomic, whereas the other tumor displayed polysomy for chromosomes 7 and 17 and no VHL mutation, VHL promoter methylation, or LOH for 3p. One tumor was composed primarily (95%) of distinctly RAT/CCPRCC-like morphology, and this tumor harbored a VHL mutation and displayed polysomy for chromosomes 7 and 17. Of the 5 cases with both histomorphologic features and molecular genetic findings of CRCC, we detected significant immunoreactivity for α-methylacyl-CoA racemase in 2 cases and strong diffuse immunopositivity for cytokeratin 7 in 3 cases. Despite the combination of positivity for α-methylacyl-CoA racemase and cytokeratin 7 in 2 cases, there was nothing to suggest of the possibility of a conventional papillary renal cell carcinoma with a predominance of clear cells.

  11. Stem Cell Enrichment with Selectin Receptors: Mimicking the pH Environment of Trauma

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    Michael R. King

    2013-09-01

    Full Text Available The isolation of hematopoietic stem and progenitor cells (HSPCs is critical for transplantation therapy and HSPC research, however current isolation techniques can be prohibitively expensive, time-consuming, and produce variable results. Selectin-coated microtubes have shown promise in rapidly isolating HSPCs from human bone marrow, but further purification of HSPCs remains a challenge. Herein, a biomimetic device for HSPC isolation is presented to mimic the acidic vascular microenvironment during trauma, which can enhance the binding frequency between L-selectin and its counter-receptor PSGL-1 and HSPCs. Under acidic pH conditions, L-selectin coated microtubes enhanced CD34+ HSPC adhesion, as evidenced by decreased cell rolling velocity and increased rolling flux. Dynamic light scattering was utilized as a novel sensor to confirm an L-selectin conformational change under acidic conditions, as previously predicted by molecular dynamics. These results suggest that mimicking the acidic conditions of trauma can induce a conformational extension of L-selectin, which can be utilized for flow-based, clinical isolation of HSPCs.

  12. Surgical treatment for skull or intracranial langerhan cell histiocytosis in children: experience of 13 cases%儿童颅脑朗格罕斯细胞组织细胞增生症13例手术治疗体会

    Institute of Scientific and Technical Information of China (English)

    沈沉浮; 袁贤瑞; 刘景平; 余小河

    2010-01-01

    @@ 朗格罕斯细胞组织细胞增生症(Langerhan cell histiocytosis,LCH)是以树突状细胞和网状细胞系统增生为特征的一组疾病,缺乏恶性病变的组织学特征,但行为上表现为侵袭性生长.

  13. Oral candidiasis mimicking an oral squamous cell carcinoma: report of a case.

    Science.gov (United States)

    Pontes, Hélder Antônio Rebelo; Paiva, Helena Borges; de Freitas Silva, Brunno Santos; Fonseca, Felipe Paiva; da Silva, Fernanda Bragança Monteiro; Pontes, Flávia Sirotheau Corrêa; Dos Santos Pinto, Décio

    2012-03-01

    Oral candidiasis is a significant problem in immune-compromised patients. The most common forms of mucosal candidiasis are oropharyngeal, oesophageal and vaginal, and more than 90% of HIV positive persons will manifest at least one episode of oropharyngeal candidiasis. Local and systemic factors such as uninterrupted daily use of a prosthesis by patients, smoking habit, as well as high glucose intake may contribute to the development of the lesion. The aim of this article is to report an uncommon case of oral candidiasis presenting an aggressive clinical behaviour in a 64-year-old male patient, with a significant smoking habit and a medical history of non-controlled diabetes. The lesion affected the hard and soft palate of the right side, revealing erythematous and ulcerated areas, elevated borders and central portions resembling necrosis, mimicking the clinical features of oral squamous cell carcinoma. However, the correct diagnosis of oral candidiasis was obtained after histopathological and cytological examinations and the patient was easily treated with traditional antifungal drugs and correction of his glucose levels.

  14. Cutaneous histiocytosis in dogs.

    Science.gov (United States)

    Mays, M B; Bergeron, J A

    1986-02-15

    Multifocal cutaneous histiocytic lesions were recognized in 9 dogs. Clinically, the dogs had multiple erythematous plaques or nodules in the skin (1 to 5 cm diameter). Histologically, the lesions were comprised of dermal or pannicular infiltrates of large histiocytic cells, with varying numbers of other inflammatory cells intermixed. By electron microscopy, the cells resembled those of canine cutaneous histiocytoma. The lesions seemed to wax and wane and appeared in new sites, regardless of treatment. The dogs ranged in age from 2 to 13 years; 7 dogs were under 6 years of age. Both sexes and various breeds were represented. An infectious agent could not be identified.

  15. Generalized eruptive histiocytosis associated with a novel fusion in LMNA-NTRK1.

    Science.gov (United States)

    Pinney, Sarah S; Jahan-Tigh, Richard R; Chon, Susan

    2016-08-15

    Non-Langerhans cell histiocytosis (NLCH) is a histiocyte disorder comprised of dermal dendritic histiocytes with a characteristic staining pattern. Erdheim-Chester disease (ECD) is a subset of NLCH in which patients experience bone pain with corresponding changes on imaging. In addition, these patients show other evidence of systemic involvement, which can also be identified with imaging. This disease can occasionally present with cutaneous findings. We present a case of generalized eruptive histiocytosis (GEH), misdiagnosed as ECD, found to have an NTRK1 gene rearrangement. This is the first report of an NTRK1 kinase fusion with NLCH. The implication is unclear and further studies are warranted.

  16. Castleman Disease in the Kidney and Retroperitoneum Mimicking Renal Cell Carcinoma with Retroperitoneal Lymphadenopathy: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Ko, Hee Sun; Woo, Ji Young; Hong, Hye Suk; Jung, Ah Young; Yang, Ik; Lee, Yul [Dept. of Radiology, Kangnam Sacred Heart Hospital, Hallym University College of Medicine, Seoul (Korea, Republic of)

    2012-09-15

    Castleman disease, or angiofollicular lymph node hyperplasia, is a fairly rare benign tumor of lymphoid origin with unknown etiology. Castleman disease arises mostly in the mediastinum, and some cases of renal and retroperitoneal involvement have been reported. However, Castleman disease that simultaneously involves the kidney and regional lymph nodes has not been reported in radiologic literature. We report a case of renal and pararenal Castleman disease, mimicking renal cell carcinoma with retroperitoneal lymphadenopathy.

  17. Lupus mimickers.

    Science.gov (United States)

    Calixto, Omar-Javier; Franco, Juan-Sebastian; Anaya, Juan-Manuel

    2014-08-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by multisystem organ involvement, heterogeneity of clinical features, and variety in degree of severity. The differential diagnosis is a crucial aspect in SLE as many other autoimmune diseases portray clinical similarities and autoantibody positivity. Lupus mimickers refer to a group of conditions that exhibit both clinical features and laboratory characteristics, including autoantibody profiles that resemble those present in patients with SLE, and prompt a diagnostic challenge in everyday clinical practice. Thus, lupus mimickers may present as a lupus-like condition (i.e., 2 or 3 criteria) or as one meeting the classification criteria for SLE. Herein we review and classify the current literature on lupus mimickers based on diverse etiologies which include infections, malign and benign neoplasms, medications, and vaccine-related reactions.

  18. Pseudocarcinomatous hyperplasia mimicking squamous cell carcinoma in a case of CD56-positive cytotoxic T-cell lymphoma.

    Science.gov (United States)

    Ginsberg, David; Hill, Hilary; Wilson, Barbara; Plaza, Jose A; Schieke, Stefan M

    2015-03-01

    We present the case of an 84-year-old patient with a cutaneous CD56 positive cytotoxic T-cell lymphoma associated with substantial pseudocarcinomatous hyperplasia mimicking squamous cell carcinoma (SCC). The patient presented with a 7-month history of several progressive, ulcerated plaques on his right forearm. An initial biopsy showed changes consistent with a diagnosis of SCC for which the patient underwent surgical treatment. Several months later, the patient developed recurrent ulcerated plaques on the right forearm of which several biopsies were performed. The biopsies repeatedly showed marked pseudocarcinomatous hyperplasia resembling SCC. Deeper punch biopsies, however, showed a dense superficial and deep infiltrate of markedly atypical lymphocytes. Immunohistochemical analysis revealed strong positive staining for CD3, CD8, CD56 with negative stains for CD30 and Epstein-Barr virus-encoded small non-polyadenylated RNAs (EBER). Staining for beta F1 and gamma-delta T-cell receptor (γδ TCR) were both negative. This constellation was most consistent with a diagnosis of cutaneous peripheral T-cell lymphoma, unspecified in association with marked pseudocarcinomatous hyperplasia. Our case adds cutaneous peripheral T-cell lymphoma, unspecified to the list of conditions associated with pseudocarcinomatous hyperplasia (PCH) and illustrates once again the potential pitfalls of distinguishing marked pseudocarcinomatous hyperplasia from SCC.

  19. Lymph node involvement by mycosis fungoides and Sézary syndrome mimicking angioimmunoblastic T-cell lymphoma.

    Science.gov (United States)

    LeBlanc, Robert E; Lefterova, Martina I; Suarez, Carlos J; Tavallaee, Mahkam; Kim, Youn H; Schrijver, Iris; Kim, Jinah; Gratzinger, Dita

    2015-09-01

    Clinical management of cutaneous T-cell lymphoma (CTCL) and angioimmunoblastic T-cell lymphoma (AITL) differs markedly. Diagnostic distinction is critical. Herein, we describe a series of 4 patients with clinically, molecularly, and histopathologically annotated mycosis fungoides or Sézary syndrome whose nodal disease mimicked AITL. The patients otherwise exhibited classic clinical manifestations of mycosis fungoides/Sézary syndrome preceding the onset of lymphadenopathy by 1 to 5 years. Skin biopsies revealed epidermotropic infiltrates characteristic of CTCL. Lymph node biopsies revealed dense CD4+ T-cell infiltrates that coexpressed follicular helper T-cell markers and were accompanied by proliferations of high endothelial venules and arborizing CD21+ follicular dendritic cell networks. Two patients had T-cell receptor gene rearrangement studies performed on their skin, lymph node, and peripheral blood demonstrating identical polymerase chain reaction clones in all 3 tissues. A small secondary clonal B-cell population was present in 1 patient that mimicked the B-cell proliferations known to accompany AITL and persisted on successive nodal biopsies over several years. This latter phenomenon has not previously been described in CTCL. The potential for patients to be misdiagnosed with AITL for lack of consideration of advanced-stage CTCL with nodal involvement underscores the necessity of information sharing among the various pathologists and clinicians involved in the care of each patient.

  20. 颈椎郎格罕细胞组织细胞增生症的MR征象分析%Magnetic resonance imaging findings of cervical Langerhans' cell histiocytosis

    Institute of Scientific and Technical Information of China (English)

    庞超楠; 刘晓光; 袁慧书

    2011-01-01

    Objective To study the MR findings of the cervical Langerhans' cell histiocytosis ( LCH ) . Methods MR findings were retrospectively analyzed in 25 cases of Langerhans' cell histiocytosis. All cases were confirmed pathologically. Results In all the 25 cases. 2 of 25 cases had multiple lesions, and adjacent vertebrae involved in 4,the anterior structure of vertcbral involved in 7 , both vertebral body and appendix involved in 18. Different degree of vertebral compression were found in 24 cases, and soft-tissue masses were found in all cases. All lesions were presented as hypo-isointensity on T1 WI, hyperintensity on T2 WI and STIR. The lesions in 13 cases with significant enhancement after Gd-DTPA injection and without cystic necrosis. The intraspinal canals were involved locally,the ranges of the soft-tissuc masses in 20 cases beyond the bone lesions. Conclusion MRI findings of cervical LCH can be regarded as characteristic,which were helpful for diagnosis, but the definite diagnosis is depending on biopsy.%目的 探讨颈椎郎格罕细胞组织细胞增生症的MR表现.方法 回顾分析25例经病理证实的颈椎郎格罕细胞组织细胞增生症的MR征象.结果 25例中有2例为多发病变,4例有邻近椎骨侵犯,7例累及脊椎前部结构,18例同时累及椎骨的前、后部结构,24例椎体有压缩,25例可见软组织肿块.全部病例在T1WI上为等、稍低或低信号,在T2WI上为稍高或高信号,脂肪抑制序列上为高信号,13例行增强扫描病变均明显强化,无坏死囊变.20例在脊柱长轴方向上侵犯的范围超过受累脊椎水平,17例软组织侵犯椎管,呈局限性生长.结论 颈椎郎格罕细胞组织细胞增生症的MR征象具有一定特征性,对该病的诊断具有较大的帮助,但确诊仍需依靠病理活检.

  1. Mimicking the germinal center reaction in hybridoma cells to isolate temperature-selective anti-PEG antibodies.

    Science.gov (United States)

    Su, Yu-Cheng; Al-Qaisi, Talal S; Tung, Hsin-Yi; Cheng, Tian-Lu; Chuang, Kuo-Hsiang; Chen, Bing-Mae; Roffler, Steve R

    2014-01-01

    Modification of antibody class and binding properties typically requires cloning of antibody genes, antibody library construction, phage or yeast display and recombinant antibody expression. Here, we describe an alternative "cloning-free" approach to generate antibodies with altered antigen-binding and heavy chain isotype by mimicking the germinal center reaction in antibody-secreting hybridoma cells. This was accomplished by lentiviral transduction and controllable expression of activation-induced cytidine deaminase (AID) to generate somatic hypermutation and class switch recombination in antibody genes coupled with high-throughput fluorescence-activated cell sorting (FACS) of hybridoma cells to detect altered antibody binding properties. Starting from a single established hybridoma clone, we isolated mutated antibodies that bind to a low-temperature structure of polyethylene glycol (PEG), a polymer widely used in nanotechnology, biotechnology and pharmaceuticals. FACS of AID-infected hybridoma cells also facilitated rapid identification of class switched variants of monoclonal IgM to monoclonal IgG. Mimicking the germinal center reaction in hybridoma cells may offer a general method to identify and isolate antibodies with altered binding properties and class-switched heavy chains without the need to carry out DNA library construction, antibody engineering and recombinant protein expression.

  2. Spindle-cell variant of primary cutaneous follicle center lymphoma spreading to the hepatobiliary tree, mimicking Klatskin tumor.

    Science.gov (United States)

    Rozati, Sima; Kerl, Katrin; Kempf, Werner; Tinguely, Marianne; Zimmermann, Dieter R; Dummer, Reinhard; Cozzio, Antonio

    2013-01-01

    Primary cutaneous follicle center lymphoma (pcFCL) is an indolent type of primary cutaneous B-cell lymphoma (pcBCL) rarely disseminating to other organs. PcBCL with spindle-cell morphology has been described as a rare variant of pcFCL but the prognosis data of this variant is sparse. We report a rare case of spindle-cell pcFCL with CD20(+), CD79a(+), CD3(+), Bcl-6(+), Mum-1(-) and CD10(-) tumor cells that infiltrated the hepatic hilum, mimicking a Klatskin tumor. On the basis of the sparse published data on spindle-cell morphology of pcBCL, this growth pattern should elicit awareness of an increased risk of systemic involvement in the otherwise indolent pcFCL.

  3. 肾脏郎格罕细胞组织细胞增生症1例报道并文献复习%Report of one case of renal Langerhans cell histiocytosis and literature review

    Institute of Scientific and Technical Information of China (English)

    韩苏军; 李长岭

    2011-01-01

    目的 探讨肾脏郎格罕细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)的临床病理特征.方法 对1例肾脏LCH患者的临床病理资料进行分析并结合文献复习.结果 患者年龄23岁,女性,表现为无症状性左肾肿物.腹部CT示左肾肿物,大小约4.3 cm×6.1 cm,考虑肾癌.行根治性左肾切除术.免疫组化显示CD1α,S-100,CD68,Lysozyme呈阳性.病理诊断为LCH.随诊3个月时未见肿瘤复发或转移.结论 肾脏LCH极其罕见,确诊依赖病理学及免疫组化检查.

  4. Clinical and pathologic analysis of Langerhans cell histiocytosis%36例朗格罕细胞组织细胞增生症的临床与病理分析

    Institute of Scientific and Technical Information of China (English)

    刘庆玲; 邹继珍; 师晓东; 刘嵘; 李君惠; 王天有

    2008-01-01

    目的 分析朗格罕细胞组织细胞增生症(langerhans cell histiocytosis,LCH)传统分型和单系统/多系统分型及Lavin-Osband分级之间的联系,观察LCH病理结果,并对比临床和预后中的不同.方法 回顾性调查36例LCH,分析比较其临床、病理及预后.结果 36例LCH中14例单系统LCH,Lavin-Osband Ⅰ、Ⅱ级,治疗后痊愈或好转12例.22例多系统LCH,多为Ⅲ、Ⅳ级;治疗后未愈或恶化9例,死亡2例.病理检查17/17例Cdla阳性;电镜6/10例找到Birbeck颗粒;12/12例Fascin染色阳性.结论 现行分型分级利于直观评估病情、预后.Cdla染色比电镜找Bir-beck颗粒更简便易行.Fascin在LCH诊断中有一定作用,但其价值需大样本量的试验证实.

  5. Two rare cases of laryngeal intralymphatic histiocytosis

    DEFF Research Database (Denmark)

    Reznitsky, Martin; Daugaard, Søren; Charabi, Birgitte Wittenborg

    2016-01-01

    was extensively examined but the only abnormal finding was a low CD4 count. The breathing difficulties fluctuated during the diagnostic process and settled after a year. Case 2 was a 35-year-old man who presented with persistent laryngeal swelling. Biopsies from the epiglottis showed intralymphatic histiocytosis...

  6. Histiocytosis X: treatment with topical nitrogen mustard.

    Science.gov (United States)

    Berman, B; Chang, D L; Shupack, J L

    1980-07-01

    The case histories of two elderly patients with cutaneous histiocytosis X treated topically with nitrogen mustard are presented. The cutaneous lesions cleared within 2 to 3 weeks, and remission was maintained with daily topical administration of nitrogen mustard. The clinical impression of improvement was substantiated by light and electron microscopic studies prior to and after therapy.

  7. Multiple cutaneous histiocytosis in two dogs.

    Science.gov (United States)

    Thornton, R N; Tisdall, C J

    1988-12-01

    Two cases of canine cutaneous histiocytosis are described. Diagnosis depended on overall consideration of clinical and histopathological features of the disease, as well as its response to anti-inflammatory therapy. No aetiological agent was visible using light and electron microscopy.

  8. Desmoplastic small round cell tumor of the kidney mimicking Wilms tumor: a case report and review of the literature.

    Science.gov (United States)

    da Silva, Rogério Cardoso; Medeiros Filho, Plínio; Chioato, Lucimara; Silva, Tácio R B; Ribeiro, Sérgio M; Bacchi, Carlos E

    2009-12-01

    Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, malignant neoplasm usually present with the widespread abdominal serosal involvement and affects mainly adolescents and young adults. When presenting within visceral organs, as kidney, the diagnosis of DSRCT imposes significant difficulties. We present a case of primary DSRCT of the kidney in a 10-year-old boy mimicking clinically and pathologically Wilms tumor. The tumor showed morphologic and immunohistochemical features of DSRCT and the presence of the Ewing sarcoma and Wilm tumor 1 fusion transcripts resulting from the t(11;22) (p13;q12) reciprocal translocation. DSRCT should be considered in the differential diagnosis of Wilm tumor and other small blue-round cell tumors of the kidney.

  9. Histiocitose de células de Langerhans: rápida resolução após cessação do tabagismo Langerhans-cell histiocytosis: rapid resolution after smoking cessation

    Directory of Open Access Journals (Sweden)

    José Miguel Chatkin

    2005-04-01

    Full Text Available Descreve-se um caso de histiocitose pulmonar de células de Langerhans com relação estreita entre cessação do tabagismo e melhora radiológica. A tomografia computadorizada de alta resolução revelava múltiplos nódulos pequenos localizados em lobos superiores e campos médios de ambos os pulmões. O exame microscópico dessas lesões mostrava infiltração histiocitária, que se corava fortemente pela proteína S100. Apesar de a remissão espontânea poder ser uma possível explicação para essa evolução, este relato de caso mostra um rápido desaparecimento das lesões após o abandono do tabagismo.We describe a case of pulmonary Langerhans cell histiocytosis with a close temporal relationship between smoking cessation and radiological improvement. High-resolution computed tomography revealed multiple small nodules located in the upper and middle lobes of both lungs. Microscopy of these lesions showed histiocytic infiltration that reacted strongly to staining for S100 protein. The histiocytes resembling Langerhans cells showed strong reactivity for S100 protein. Smoking cessation was recommended and the patient complied. Chest X-ray and computed tomography performed 6 and 24 months later revealed almost complete resolution of the radiographic abnormalities. Despite the possibility that this evolution was attributable to spontaneous remission, in this case, the lesions did, in fact, disappear rapidly after smoking cessation.

  10. Histiocitose de células de Langerhans com acometimento vulvar e com resposta terapêutica à talidomida: relato de caso Langerhans cells histiocytosis with vulvar involvement and responding to thalidomide therapy: case report

    Directory of Open Access Journals (Sweden)

    Lana Bezerra Fernandes

    2011-08-01

    Full Text Available A histiocitose de células de Langerhans é representante de um raro grupo de síndromes histiocitárias, sendo caracterizada pela proliferação das células de Langerhans. Suas manifestações variam de lesão solitária a envolvimento multissistêmico, sendo o acometimento vulvar incomum. Segue-se o relato de caso refratário da doença limitada à pele, em mulher de 57 anos. A paciente apresentava história de pápulas eritematosas ulceradas em couro cabeludo, face, vulva, tronco e axila há seis anos. O diagnóstico da doença é difícil, sendo confirmado neste caso através de estudo imuno-histoquímico e se obteve resposta terapêutica e eficaz, com a administração de talidomidaLangerhans cell histiocytosis is a member of a group of rare histiocytic syndromes and is characterized for the proliferation of histiocytes called Langerhans'cells. Its manifestations vary from a solitary injury to systemic involvement, and vulvar lesions are uncommon. We describe a refractory case of cutaneous limited disease in a 57-year-old woman. She presented with a 6-year history of an erythematous papular eruption of the scalp, face, vulva, trunk and axillae. The diagnosis is difficult and in this case it was confirmed through immunohistochemical study and clinical improvement was achieved with thalidomide

  11. Cutaneous histiocytosis X. The presence of S-100 protein and its use in diagnosis.

    Science.gov (United States)

    Rowden, G; Connelly, E M; Winkelmann, R K

    1983-07-01

    The cellular localization of glial S-100 protein was investigated in paraffin-embedded sections of cutaneous histiocytosis X and in a variety of cutaneous infiltrative disorders, including juvenile xanthogranuloma, necrobiotic xanthogranuloma, papular xanthoma, eruptive histiocytoma, and reticulohistiocytosis. Immunoperoxidase staining with a rabbit anti-calf brain-S-100 antibody demonstrated strong and consistent activity in atypical histiocytes in all histiocytosis X specimens. No detectable S-100 protein was demonstrated in either histiocytes or giant cells in the non-X histiocytic disorders investigated. Positive controls were included within both groups of disorders in respect to melanocyte, epidermal Langerhans' cell, and dermal Schwann's cell staining. These findings are interpreted as evidence for diversity in the mononuclear phagocyte system and demonstrate the practicality of such a simple test in diagnostic problems involving infiltrative histiocytic disorders of the skin.

  12. Multiple von Meyenburg complexes mimicking diffuse liver metastases from esophageal squamous cell carcinoma

    Institute of Scientific and Technical Information of China (English)

    Stefan Fritz; Thilo Hackert; Hendrik Blaker; Werner Hartwig; Lutz Schneider; Markus W Buchler; Jens Werner

    2006-01-01

    Von Meyenburg complexes are benign liver lesions consisting of adenomatous bile duct proliferates. We present two patients suffering from esophageal cancer accompanied by the occurrence of von Meyenburg complexes.Preoperative computerized tomography (CT) of the liver had not shown these lesions. In one of the patients, diffuse nodular manifestation was found in both liver lobes,mimicking diffuse hepatic metastases. Intraoperative frozen section revealed the benign nature of the lesions in both cases. The patients underwent esophageal resection without complications. To the best of our knowledge, the coincidence of von Meyenburg complexes and esophageal cancer has never been reported before. This uncommon entity should be taken into consideration as a differential diagnosis of liver lesions in malignancies. It underlines the importance of intraoperative frozen section for liver lesions of unknown origin.

  13. Langerhans cell histiocytosis of the temporal bone:22 cases analysis%颞骨朗格汉斯组织细胞增生症22例临床分析

    Institute of Scientific and Technical Information of China (English)

    陈良; 王武庆; 徐慧; 迟放鲁

    2010-01-01

    Objective To analyze the clinical manifestations,diagnosis,therapy and prognosis of Langerhans cell histiocytosis(LCH)of the temporal bone.Methods Twenty-two patients with LCH of the temporal bone in our hospital were retrospectively summed up from January 1994 to January 2008.Following up 1-15 years,their clinical manifestations,diagnosis,therapy and prognosis were studied.Survival analysis and disease free survival Log-rank test were used.Results Among 22 patients,1 case belonged to the multisystem high risk group,3 cases to the muhisystem low risk group,and 18 cases to the single system group.The clinical characters were primarily the ear presentations,CT of the temporal bones showed extensive osteolytic destructions.The misdiagnosis rate was 72.7%.Twenty-two cases were received different combined modality therapies.Following up 1-15 years,21 cases were survival and 1 case dead (4.5%),while 5 cases showed residual of insipidus,dwarfism,epilepsy or unilateral serious heating loss (22.7%).For disease free survival curve,there was a significant difference between the multisystem group and the single system group(X~2=5.87,P<0.05).Conclusions As LCH of the temporal bone,the single system cases are predominant.The ear area clinical manifestations are primary.This disease is easily misdiagnosed.The therapy selection should rely on the involved systems of the disease.The prognosis of the single system group is much better than the multisystem group.%目的 分析颞骨朗格汉斯组织细胞增生症(Langerhans cell histiocytosis,LCH)的临床表现、诊断、治疗及预后.方法 回顾性分析我院15年来收治的22例颞骨LCH,研究其临床表现、诊断、治疗及预后,并随访1~15年,进行生存分析和无病生存率的Log-rank检验.结果 22例中1例为多系统高危组,3例为多系统低危组,18例为单系统组.临床表现以耳部为主,颞骨CT见大范围的溶骨性骨质破坏.22例患者的误诊率为72.7%(15/22).所有患者采

  14. 儿童郎格尔汉斯细胞组织细胞增生症的CT表现(附13例分析)%Langerhans cell histiocytosis in children: CT manifestation(report of 13 cases)

    Institute of Scientific and Technical Information of China (English)

    李欣; 杨志勇; 贾万英

    2001-01-01

    目的 探讨CT对郎格尔汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)造成多脏器损害诊断的价值及限度。方法 分析13例经临床、实验室、病理检查确诊的LCH的CT表现。局限性LCH 4例,广泛性LCH 9例。全部病例均行颅脑、胸部、肝、脾CT平扫检查,4例行增强检查。结果 男性多于女性,颅穹隆骨破坏依次为颞骨8例、顶骨3例、枕骨2例、额骨1例。CT发现早期骨破坏敏感性高。肿块变化可反映病变由活跃增殖到静止消退的病理过程。下丘脑-垂体轴侵犯影像表现晚于临床。CT可显示LCH不同阶段的肺损害。结论 LCH各脏器损害的影像学表现缺乏特异性。好发部位的典型CT表现可提示诊断,同时对辅助LCH分型、确定治疗方案、估计预后有一定价值。%Objective To study the value and limitation of CT in the damage to multiple organs in children with Langerhans cell histiocytosis (LCH). Methods Analysis of CT manifestations was performed in 13 cases of LCH proved by clinical features, laboratory data, and pathologic changes. Localized LCH was found in 4 cases, and disseminated LCH in 9 cases. All cases were examined by plain scan in craniocerebral, chest, liver, and spleen, and four cases by contrast-enhanced CT scan. Results The male outnumbered female in this group. Cranial bones were involved in the sequence of temporal bone(8 cases), parietal bone (3 cases), occipital bone (2 cases), and frontal bone (1 case). CT findings were very sensitive in showing the early destructions of the bones. The characteristic manifestation on CT was multiple lytic lesions of the flat bones at any sites, without reactive sclerosis and soft tissue mass accompanied with the bony lesion. Imaging findings presented later than the clinical manifestations for LCH involving the hypothalamus/pituitary axis. Involvement of the lung at various stages by LCH could be demonstrated by CT scan

  15. Clinical analysis of Langerhans cell histiocytosis in 38 children%小儿郎格罕组织细胞增生症38例临床分析

    Institute of Scientific and Technical Information of China (English)

    王西阁; 栾斌; 张晓莉; 张冬梅

    2006-01-01

    目的 探讨郎格罕组织细胞增生症(Langerhans cell histiocytosis,LCH)的临床特点,提高临床诊治水平.方法 对近15年来我院收治的38例LCH患儿临床资料进行回顾性分析.结果 38例患儿中发病年龄为3个月~13岁,男女比例为1.92:1.主要症状为发热、皮疹、骨损害、肝脾淋巴结肿大、突眼、尿崩、耳溢脓等.不同分型、分级患儿在器官受累范围和程度上有明显不同.按分型分级给予不同方案化疗18例,好转15例,有效率83.33%.结论 LCH临床表现复杂多样,诊断依靠病理活检.提高诊断率的关键在于加深对LCH特点的认识和多部位多例次的活检.国际组织细胞学会LCH分型分级对于选择合理的治疗方案、判断预后有指导意义.

  16. Langerhans cell histiocytosis in childhood: a clinicopathologic and immunophenotypic analysis of 38 cases%38例儿童Langerhans细胞组织细胞增生症临床病理观察

    Institute of Scientific and Technical Information of China (English)

    谢小志; 张忠德; 奚政君; 吴湘如

    2006-01-01

    目的探讨儿童Langerhans细胞组织细胞增生症(Langerhans cell histiocytosis, LCH)的临床特点、病理形态学及免疫表型特征,并分析其与预后的关系.方法分析38例儿童LCH的临床及病理资料,其中18例做了免疫组化染色.结果在38例儿童LCH中,单发为30例(占78.9%),多发为8例(占21.1%).病变发生于骨组织34例(占89.5%)(其中1例伴有皮肤病变),发生于皮肤组织3例,发生于淋巴结1例.镜下病变主要由Langerhans细胞及嗜酸性粒细胞组成.免疫组化结果显示Langerhans细胞阳性表达CD1a为100%(13/13),S-100蛋白为88.2%(15/17),vimentin为90.0%(9/10),CD68为81.8%(9/11),Mac387为30.0%(3/10),lysozyme为40.0%(4/10),CK、EMA、CD45均阴性.结论儿童LCH最多发生于骨组织,在病理学上具有特殊的形态学表现以及免疫组化表型.疾病的预后与病理分型及临床分级有关.

  17. Clinical analysis of Langerhans cell histiocytosis in one newborn%新生儿朗格罕斯细胞组织细胞增生症1例报告

    Institute of Scientific and Technical Information of China (English)

    汪盈; 赵志光; 孙忠敏

    2014-01-01

    Objective To understand the clinical features and laboratory examination and diagnosis of Langerhas cell histio-cytosis (LCH) in newborns. Methods Retrospective analysis of the clinical data of a neonatal case of LCH was performed. Results A one-day-old girl was admitted with chief complaint of eating less, less crying and skin rash for one day. The unequal sized rash was on the head, limbs, trunk, toe and oral mucosa. The skin rashes were protruding from the skin and were partially broken and crusted. Biopsy of the skin tissue showed the expression of S-100 and CD1α, consistent with the diagnosis of LCH. Conclusions When new-borns have special rash after birth, LCH should be considered. To avoid misdiagnosis, skin biopsy should be performed.%目的:了解新生儿朗格罕斯细胞组织细胞增生症(LCH)的临床、实验室特点及诊断要点。方法回顾性分析1例新生儿LCH的临床资料。结果女性患儿,生后1 d因少吃、少哭,皮疹入院,头部、四肢、躯干、脚趾、口腔可见多发皮疹,突出皮面,大小不等,部分破溃结痂。皮肤活检提示病变组织表达S-100(+),CD1α(+),符合LCH诊断。结论新生儿生后有特殊皮疹,需警惕LCH,应进行皮肤活检,以免误诊。

  18. Combined basal cell carcinoma and Langerhans cell histiocytosis of the scrotum in a patient with occupational exposure to coal tar and dust

    Energy Technology Data Exchange (ETDEWEB)

    Izikson, L.; Vanderpool, J.; Brodsky, G.; Mihm, M.C.; Zembowicz, A. [Harvard University, Boston, MA (US). Massachusetts General Hospital

    2004-09-01

    The patient was a 77-year-old male former smoker, with history of several basal cell carcinomas (BCCs) in sun-protected areas around the waistline, who presented with another small ulceration on the anterior right upper scrotum near the base of the penis. BCC was suspected clinically and the lesion was treated with cryosurgery. The tumor recurred, became raised, and began to bleed. An excisional biopsy was performed. It showed nodular BCC surrounded by a cellular proliferation of round histiocytic cells with convoluted, lobulated and reniform nuclei and abundant cytoplasm . The patient had no history of exposure to ionizing radiation, chemotherapy, immunosuppressive medications, prior lymphoma or other malignancy. However, he spent 4 years on a ship loading coal into the furnace of a steam engine, during which he slept in adjacent quarters that were covered with coal dust. Additionally, he had a several-year history of occupational skin exposure to machine oil, oil refinery waste, sulfur waste, hydraulic fluid, and asbestos. He also reported a history of nude sunbathing. The scrotal lesion was re-excised and the patient remains disease-free more than 1 year after the diagnosis.

  19. Cervical meningeal histiocytosis demonstrated by magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Drolshagen, L.F.; Kessler, R.; Partain, C.L.

    1987-01-01

    Involvement of the central nervous system by histiocytosis X is usually restricted to the parasellar region. A rare case of histiocytosis X involving the cervical meninges in a 12-month-old boy is demonstrated and the magnetic resonance features of this tumor are described.

  20. Atypical reactive histiocytosis. A case report.

    Directory of Open Access Journals (Sweden)

    Jorge E. Barleta del Castillo

    2004-08-01

    Full Text Available This paper presents the case of a 50 year old chronic alcoholic and heavy smoker female that was assisted at the provincial university hospital ¨Dr. Gustavo Aldereguía Lima¨ in Cienfuegos city due to a severe adenic syndrome and who was diagnosed as a case of atypical reactive histiocytosis , problem which disappeared with the abstinence of toxic habits, improving her health.

  1. 中枢神经系统受累的郎格罕斯细胞组织细胞增生症诊治进展%Progress in the diagnosis and treatment of Langerhans cell histiocytosis with central nervous system involvement

    Institute of Scientific and Technical Information of China (English)

    张莉; 郑胡镛

    2013-01-01

    Langerhans cell histiocytosis (LCH) is a rare disease of the monocyte-macrophage system. The clinical presentation of LCH varies. It commonly has multi-organ involvement, but rarely has the involvement of central nervous system (CNS). The standards for the diagnosis and treatment of LCH are not consistent in different countries. In this review, the features of magnetic resonance imaging (MRI), the neuropathologic changes, the clinical manifestation, the risk factors, and the available therapeutic experiences of LCH patients with CNS involvement were summarized, so as to improve the clinical recognition of the disease for the clinicians.%胃郎格罕斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)是一种少见的单核巨噬系统疾病,临床表现多样,可有多脏器受累,但其中枢神经系统合并症相对少见.国内外对其诊断及治疗标准仍不统一.该文回顾及总结中枢神经系统受累LCH患者的磁共振成像(MRI)特点、神经病理学变化、临床表现、危险因素以及一些治疗经验,以提高临床医师对LCH患者中枢神经系统合并症的认识.

  2. 2例郎格罕细胞组织细胞增生症的临床病理分析%Clinicopathologic Features of Two Cases with Langerhans Cell Histiocytosis

    Institute of Scientific and Technical Information of China (English)

    韩莉; 韩玉华

    2013-01-01

    Objective?To investigate the clinical and pathological features of langerhans cell histiocytosis(LCH). Methods The 2 cases of LCH were analyzed about the features of clinic, pathology and immunohistochemisty. Results LCH cell with abundant eosinophilic cytoplasms, and longitudinally grooved or reniform nuclei .Lymphocytes and a few eosinophils also could be seen. Two cases showed that the langerhans cell were with pale cytoplasms, there were numerous eosinophils cells. The immunity displayed that CD1a (+),S-100 (+),HLA-DR (+). Conclusions A diagnosis of LCH was made based on clinical manifestation and histopathological features, both CD1a (+) and S-100 (+) were needed. The prognosis was almost good and need follow-up.%目的:探讨郎格罕细胞组织细胞增生症(LCH)的临床病理特点,提高对其认识,减少误诊。方法2例郎格罕细胞组织细胞增生症患者,用HE及免疫组化的方法分析其临床、病理及免疫特色。结果病理形态LCH细胞大,圆形或卵圆形,胞质丰富略嗜酸性,有的核偏位可见核沟,伴嗜酸性粒细胞浸润。免疫组化示:CD1a(+),S-100(+),HLA-DR部分(+)。结论2例患者现恢复良好,但此类肿瘤生物学行为不确定故仍建议密切随访。病理检查细胞核形态有特异性,明确诊断需CD1a(+),S-100(+)。

  3. Self-regressing S100-negative CD1a-positive cutaneous histiocytosis.

    Science.gov (United States)

    Tardío, Juan C; Aguado, Marta; Borbujo, Jesús

    2013-06-01

    In the skin, the antigen-presenting cells are mainly represented by Langerhans cells, indeterminate cells, and interstitial dendritic cells, which show distinctive immunophenotype and/or ultrastructure. We report a case of a cutaneous-limited self-regressing histiocytosis with a peculiar immunohistochemical profile (CD1a-positive and S100 protein-negative) that is not observed in any of the known cutaneous antigen-presenting cell or nowadays recognized neoplasm. This lesion is probably related to indeterminate dendritic cell tumors, but very few cases with such immunoprofile have been reported up-to-date, and their exact nosologic position and outcome remain to be clarified.

  4. Clinical analysis of 28 cases of Langerhans cell histiocytosis%朗格汉斯细胞组织细胞增生症28例临床分析

    Institute of Scientific and Technical Information of China (English)

    苏海辉; 单士军; 赵滨; 胡晓丽

    2014-01-01

    探讨儿童朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)的临床特点及治疗方法,以提高LCH的临床诊疗水平.方法:28例儿童LCH,确诊前均行组织病理检查,按Lavin-Osband分级和临床分型,Ⅰ型和I级、Ⅱ级LCH患儿中单一骨损害采用手术刮除,单一皮肤损害、Ⅱ型、Ⅲ型、Ⅲ级、Ⅳ级采用泼尼松诱导化疗和随后长春新碱加泼尼松(VP)持续化疗.结果:LCH可累及多个系统和脏器,伴有不同的功能障碍,5例单一皮肤型LCH因皮疹减轻或消退家属拒绝化疗.7例单发的骨损害采用手术刮除后痊愈,10例单一皮肤型LCH和6例系统型LCH采用联合化疗.6周后总体疗效评价:治愈率46.43%;18个月后总体疗效评价:治愈率82.14%.结论:LCH是一组特异性以骨髓源性朗格汉斯细胞细胞(Langerhans cell,LC)和成熟的嗜酸性粒细胞增生为特征的先天性疾病,表现为骨、皮肤和淋巴结等局部器官或全身的LC的异常增生.LCH主要发生在儿童,成人少见,临床表现多种多样,经免疫组化可以获得正确诊断,但目前临床缺乏理想统一的治疗方案.泼尼松诱导化疗和随后长春新碱加泼尼松(VP)持续化疗治疗儿童LCH的疗效满意,不良反应、并发症少,值得临床推广.

  5. Trapping and dynamic manipulation of polystyrene beads mimicking circulating tumor cells using targeted magnetic/photoacoustic contrast agents

    Science.gov (United States)

    Wei, Chen-Wei; Xia, Jinjun; Hu, Xiaoge; Gao, Xiaohu; O’Donnell, Matthew

    2012-01-01

    Abstract. Results on magnetically trapping and manipulating micro-scale beads circulating in a flow field mimicking metastatic cancer cells in human peripheral vessels are presented. Composite contrast agents combining magneto-sensitive nanospheres and highly optical absorptive gold nanorods were conjugated to micro-scale polystyrene beads. To efficiently trap the targeted objects in a fast stream, a dual magnet system consisting of two flat magnets to magnetize (polarize) the contrast agent and an array of cone magnets producing a sharp gradient field to trap the magnetized contrast agent was designed and constructed. A water-ink solution with an optical absorption coefficient of 10  cm−1 was used to mimic the optical absorption of blood. Magnetomotive photoacoustic imaging helped visualize bead trapping, dynamic manipulation of trapped beads in a flow field, and the subtraction of stationary background signals insensitive to the magnetic field. The results show that trafficking micro-scale objects can be effectively trapped in a stream with a flow rate up to 12  ml/min and the background can be significantly (greater than 15 dB) suppressed. It makes the proposed method very promising for sensitive detection of rare circulating tumor cells within high flow vessels with a highly absorptive optical background. PMID:23223993

  6. Tooth Enamel Protein Amelogenin Binds to Ameloblast Cell Membrane-Mimicking Vesicles via its N-terminus

    Science.gov (United States)

    LOKAPPA, SOWMYA BEKSHE; CHANDRABABU, KARTHIK BALAKRISHNA; MORADIAN-OLDAK, JANET

    2015-01-01

    We have recently reported that the extracellular enamel protein amelogenin has affinity to interact with phospholipids and proposed that such interactions may play key roles in enamel biomineralization as well as reported amelogenin signaling activities. Here, in order to identify the liposome-interacting domains of amelogenin we designed four different amelogenin mutants containing only a single tryptophan at positions 25, 45, 112 and 161. Circular dichroism studies of the mutants confirmed that they are structurally similar to the wild-type amelogenin. Utilizing the intrinsic fluorescence of single tryptophan residues and fluorescence resonance energy transfer FRET, we analyzed the accessibility and strength of their binding with an ameloblast cell membrane-mimicking model membrane (ACML) and a negatively charged liposome used as a membrane model. We found that amelogenin has membrane-binding ability mainly via its N-terminal, close to residues W25 and W45. Significant blue shift was also observed in the fluorescence of a N-terminal peptide following addition of liposomes. We suggest that, among other mechanisms, enamel malformation in cases of Amelogenesis Imperfecta (AI) with mutations at the N-terminal may be the result of defective amelogenin-cell interactions. PMID:26188506

  7. Hallazgos en el macizo facial y la base del cráneo de pacientes pediátricos con histiocitosis de células de Langerhans Craniofacial and skull base findings in Langerhans cell histiocytosis in pediatric patients

    Directory of Open Access Journals (Sweden)

    Daniel Forlino

    2013-03-01

    Full Text Available La histiocitosis de células de Langerhans (HCL es una entidad poco frecuente, con una incidencia anual de 2,6 a 5,4 por millón de niños en la población general. Tiene manifestaciones óseas (lesiones osteolíticas solitarias o múltiples en huesos planos, largos e irregulares o multisistémicas. Se describen los hallazgos imagenológicos de una serie retrospectiva de 17 pacientes pediátricos, de 1 a 12 años de edad, con histiocitosis de células de Langerhans en el macizo facial y la base del cráneo. Las manifestaciones incluyeron lesiones osteolíticas y masa de partes blandas, que ocupaban las cavidades adyacentes, como la órbita, los senos paranasales, la caja timpánica y la mastoides. En el maxilar inferior puede provocar reabsorción del reborde alveolar con apariencia de dientes flotantes. La complejidad anatómica del área de estudio requiere su valoración mediante tomografía computada (TC y resonancia magnética (RM con contraste. La histiocitosis de células de Langerhans debe considerarse dentro de los diagnósticos diferenciales de las lesiones del macizo facial y la base del cráneo, especialmente en pacientes pediátricos.Langerhans cell histiocytosis (CLH is an uncommon entity, of unknown etiology, with an incidence of 2.6 at 5.4 per 1,000,000 children/year in the general population. It may have bone manifestations (solitary or multiple osteolytic lesions in flat, long and irregular bones or multiorgan manifestations. We report the radiological findings in retrospective series of 17 pediatric patients aged 1 to 12 years old, with CLH in craniofacial and skull base. Radiological findings included osteolytic and soft tissue lesions occupying the orbit, sinuses, tympanic cavity and mastoid. In the mandible, reabsorption of the alveolar ridge with the appearance of floating teeth was observed. The anatomical complexity of the area studied required evaluation by CT and MRI with contrast. LCH disease should be considered in

  8. 儿童多系统性朗格汉斯细胞组织细胞增生症8例临床病理分析%Multi-system Langerhans cell histiocytosis in children:a clinicopathologic study of 8 cases

    Institute of Scientific and Technical Information of China (English)

    赵曼丽; 朱坤; 杨敏; 顾伟忠; 赵云; 舒艳; 汤宏峰

    2015-01-01

    Objective:To study clinical and histopathological characteristics, diagnostic criteria, differential diagnosis and prognosis of systematic Langerhans cell histiocytosis in children.Methods: Clinical and pathological features were studied in 8 cases of multi-system Langerhans cell histiocytosis with review of the literatures.Results:hTere were 1 males and 7 females. hTe youngest was 13-day-old and the oldest was 3 years and 5 months old. Among those MS-LCH patients, 6 patients had risk organ involvement. The clinical symptoms included fever, skin eruption or anemia, with inifltrative lesion of liver, spleen, lymph node, bone and hematopoietic system. Microscopically, the disease was characterized by clonal hyperplasia of histiocytes with folded nucleus. Immunohistochemical staining showed that the histiocytes were positive for CD1a and S-100.Conclusion: Multi-system Langerhans cell histiocytosis presents complex clinical symptoms. hTe diagnosis needs a combination of clinical features, image examination and histopathological characters. If the risk organ was involved, the prognosis is poor.%目的:探讨多系统朗格汉斯细胞组织细胞增生症(multi-system Langerhans cell system,MS-LCH)的临床特点、病理诊断、鉴别诊断及预后等。方法:结合相关文献,对8例MS-LCH的临床资料、病理学表现和免疫组织化学结果进行分析。结果:男性1例,女性7例,年龄从13 d~3岁5个月,其中6例累及高危器官。临床表现为发热、皮疹或贫血,伴有肝、脾、淋巴结、骨、垂体等多系统器官受累。镜下见受累器官内朗格汉斯组织细胞增生,核呈分叶状或不规则形。免疫组化显示朗格汉斯细胞阳性表达CD1a和S-100。结论:MS-LCH临床表现复杂,诊断需要结合临床表现、影响学检查及组织病理学检查。累及高危器官者,预后较差。

  9. Langerhans cell histiocytosis: a clinicopathologic and immunohistochemical analysis of 258 cases%Langerhans细胞组织细胞增生症258例临床病理特征和免疫表型分析

    Institute of Scientific and Technical Information of China (English)

    许霞; 刘卫平; 杨群培; 王威亚; 廖殿英; 赵莎; 毕成峰; 林莉; 闵敏

    2012-01-01

    Objectives To observe the clinicopathologic features of Langerhans cell histiocytosis (LCH),and to evaluate the values of langerin,CD1a and S-100 protein expression in diagnosis of the tumor.Methods Total 258 cases of Langerhans cell histiocytosis in the past 18 years ( from 1992 to 2008 )were collected,morphologic review and immunohistochemical staining were performed. Results In all 258 cases,the ages of patients older than 16 years or younger than 2 years were 126 (48.8%) and 37 ( 14.3% ),respectively,in the remaining 95 (36.8% ) of the cases,the age of the patients ranged from 2 to 16 years.For all of 258 cases,there were 364 diseased sites. Bony lesions accounted for 77.2%(281 cases),especially the skull (112 cases,39.9% ),followed by lymph node (25 cases,6.9% ) and skin ( 14 cases,3.8% ).Clinically,unisystem or unifocal disease was predominant (201 cases,77.9% ),followed by unisystem and muhifocal disease (21 cases, 8.1%), multi-system disease (26 cases,10.1% ),isolated pulmonary LCH (2 cases,0.8% ),and unclassified (8 cases,3.1% ).Histologically,variable number of Langerhans cells was present in 265 samples of 258 cases.Muhinucleated giant cells were found in 166 (62.6% ) of the samples.Eosinophils were the major infiltrating non-neoplastic cells,and eosinophilic abscess was seen in 57 cases (21.5%).Coagulative necrosis and dead bone were detected in 29 ( 10.9% ) and 124 (46.8% ) of the cases,respectively.Immunohistochemically,the expression of S-100 protein,CD1 a and langerin was 99.1% (209/211),100% (206/206) and 98.5% (193/196),respectively,and the sensitivity of them had no statistical difference.Conclusions In this group of LCH cases,the ratio of adult patients is high,but the proportion of multi-organ lesion is low. No significant difference of the sensitivity is found among langerin,CD1a and S-100 expression in diagnosis of LCH.%目的 观察Langerhans细胞组织细胞增生症(LCH)的

  10. Disseminated intravascular large-cell lymphoma with initial presentation mimicking Guillain-Barré syndrome.

    Science.gov (United States)

    Jiang, Qin Li; Pytel, Peter; Rowin, Julie

    2010-07-01

    We report a patient with intravascular large B-cell lymphoma who initially presented with acute ascending weakness and sensory changes. Electrodiagnostic testing and cerebral spinal fluid (CSF) studies were initially suggestive of a demyelinating polyneuropathy. Further clinical evaluation and testing were consistent with mononeuropathy multiplex. Autopsy revealed disseminated intravascular large-cell lymphoma. Intravascular large-cell lymphoma should be considered in the differential diagnosis of a rapidly evolving neuropathy associated with other organ involvement.

  11. PHENYTOIN-ASSOCIATED LYMPHOADENOPATHY MIMICKING A PERIPHERAL T-CELL LYMPHOMA

    Directory of Open Access Journals (Sweden)

    Mark E. Johns

    2010-09-01

    Full Text Available We report a case of phenytoin-induced pseudolymphoma in a 28-year-old male with a history of autism and seizure disorder.  The patient presented with bilateral cervical lymphadenopathy that was shown to be moderately to markedly FDG-avid on a whole body PET/CT scan.  Flow cytometry analysis of peripheral blood and bone marrow mononuclear cells detected identical T cell population with aberrant immunophenotype.  Additionally, a TCR beta gene was found to be clonally rearranged in both peripheral blood and bone marrow supporting a clonal origin of atypical T cells. However, no such clonal population of T-cells could be detected in a pathologic specimen obtained from an excisional biopsy of one of the patient’s cervical lymph nodes. After discontinuing the patient’s phenytoin, his lymphadenopathy has nearly completely resolved and circulation clonal T cell population disappeared with 12 months of follow-up.

  12. Histiocitose de células de Langerhans: doença de Letterer-Siwe - importância do diagnóstico dermatológico em dois casos Langerhans cell histiocytosis: Letterer-Siwe disease - the importance of dermatological diagnosis in two cases

    Directory of Open Access Journals (Sweden)

    Leonardo Mello Ferreira

    2009-08-01

    Full Text Available A histiocitose de células de Langerhans é proliferação clonal de células fenotipicamente semelhantes às células de Langerhans. Anteriormente denominada Letterer-Siwe, é a forma mais comum e mais grave dessa enfermidade, acometendo sobretudo crianças até os dois anos de idade. São apresentados dois casos dessa rara doença, diagnosticados após parecer dermatológico, destacando-se seus aspectos mais característicos.Langerhans cell histiocytosis is defined as a clonal proliferation of Langerhans phenotypic-like cells. Letterer-Siwe disease is the most common and serious of these entities, affecting mainly infants up to two years of age. We present two cases of this rare disease, diagnosed after dermatological examination, highligthing its typical aspects.

  13. Langerhans cell histiocytosis of atlantoaxial joint in a middle-aged man presenting with deafness as first symptom and soft-tissue mass at neck showing excellent response to radiotherapy alone: Report of an extremely rare and unusual clinical condition and review of literature

    Science.gov (United States)

    Mondal, Dodul; Julka, P. K.; Jana, Manisha; Walia, Ritika; Chaudhuri, Tamojit

    2014-01-01

    Langerhans cell histiocytosis (LCH) is a disorder of clonal proliferation of dendritic cell mainly occurring in children. Spine involvement is rare. This usually presents with pain and torticollis when neck is involved. Histopathology with immunohistochemistry is confirmatory. Local curative therapy with excision or curettage is used for localized disease. Radiotherapy is usually reserved for selected cases. Systemic chemotherapy is the treatment of choice for widespread systemic disease. In this article, we present an unusual presentation of atlantoaxial LCH with mastoid involvement resulting in hearing loss as the first symptom and quadruparesis in a middle aged male patient, which was also associated with soft-tissue mass at the nape of the neck and deafness. The patient was treated with radical radiotherapy, which provided excellent response to the disease. Involvement of atlantoaxial joint and temporal bone associated with soft-tissue mass neck and deafness in a middle-aged man is an extremely rare clinical situation. PMID:25506166

  14. Visualizing the Histotripsy Process: Bubble Cloud-Cancer Cell Interactions in a Tissue-Mimicking Environment.

    Science.gov (United States)

    Vlaisavljevich, Eli; Maxwell, Adam; Mancia, Lauren; Johnsen, Eric; Cain, Charles; Xu, Zhen

    2016-10-01

    Histotripsy is a non-invasive ultrasonic ablation method that uses cavitation to mechanically fractionate tissue into acellular debris. With a sufficient number of pulses, histotripsy can completely fractionate tissue into a liquid-appearing homogenate with no cellular structures. The location, shape and size of lesion formation closely match those of the cavitation cloud. Previous work has led to the hypothesis that the rapid expansion and collapse of histotripsy bubbles fractionate tissue by inducing large stress and strain on the tissue structures immediately adjacent to the bubbles. In the work described here, the histotripsy bulk tissue fractionation process is visualized at the cellular level for the first time using a custom-built 2-MHz transducer incorporated into a microscope stage. A layer of breast cancer cells were cultured within an optically transparent fibrin-based gel phantom to mimic cells inside a 3-D extracellular matrix. To test the hypothesis, the cellular response to single and multiple histotripsy pulses was investigated using high-speed optical imaging. Bubbles were always generated in the extracellular space, and significant cell displacement/deformation was observed for cells directly adjacent to the bubble during both bubble expansion and collapse. The largest displacements were observed during collapse for cells immediately adjacent to the bubble, with cells moving more than 150-300 μm in less than 100 μs. Cells often underwent multiple large deformations (>150% strain) over multiple pulses, resulting in the bisection of cells multiple times before complete removal. To provide theoretical support to the experimental observations, a numerical simulation was conducted using a single-bubble model, which indicated that histotripsy exerts the largest strains and cell displacements in the regions immediately adjacent to the bubble. The experimental and simulation results support our hypothesis, which helps to explain the formation of the

  15. Regeneration of the lung: Lung stem cells and the development of lung mimicking devices.

    Science.gov (United States)

    Schilders, Kim A A; Eenjes, Evelien; van Riet, Sander; Poot, André A; Stamatialis, Dimitrios; Truckenmüller, Roman; Hiemstra, Pieter S; Rottier, Robbert J

    2016-04-23

    Inspired by the increasing burden of lung associated diseases in society and an growing demand to accommodate patients, great efforts by the scientific community produce an increasing stream of data that are focused on delineating the basic principles of lung development and growth, as well as understanding the biomechanical properties to build artificial lung devices. In addition, the continuing efforts to better define the disease origin, progression and pathology by basic scientists and clinicians contributes to insights in the basic principles of lung biology. However, the use of different model systems, experimental approaches and readout systems may generate somewhat conflicting or contradictory results. In an effort to summarize the latest developments in the lung epithelial stem cell biology, we provide an overview of the current status of the field. We first describe the different stem cells, or progenitor cells, residing in the homeostatic lung. Next, we focus on the plasticity of the different cell types upon several injury-induced activation or repair models, and highlight the regenerative capacity of lung cells. Lastly, we summarize the generation of lung mimics, such as air-liquid interface cultures, organoids and lung on a chip, that are required to test emerging hypotheses. Moreover, the increasing collaboration between distinct specializations will contribute to the eventual development of an artificial lung device capable of assisting reduced lung function and capacity in human patients.

  16. Pseudoepitheliomatous hyperplasia mimicking esophageal squamous cell carcinoma in a patient with lye-induced esophageal stricture.

    Science.gov (United States)

    Han, Jang Soo; Lee, Sang Woo; Suh, Kang Heum; Kim, Seung Young; Hyun, Jong Jin; Jung, Sung Woo; Koo, Ja Seol; Yim, Hyung Joon

    2014-06-01

    Pseudoepitheliomatous hyperplasia is a benign condition that may be caused by prolonged inflammation, chronic infection, and/or neoplastic conditions of the mucous membranes or skin. Due to its histological resemblance to well-differentiated squamous cell carcinoma, pseudoepitheliomatous hyperplasia may occasionally be misdiagnosed as squamous cell carcinoma. The importance of pseudoepitheliomatous hyperplasia is that it is a self-limited condition that must be distinguished from squamous cell carcinoma before invasive treatment. We report here on a rare case of esophageal pseudoepitheliomatous hyperplasia in a 67-year-old Korean woman with a lye-induced esophageal stricture. Although esophageal pseudoepitheliomatous hyperplasia is infrequently encountered, pseudoepitheliomatous hyperplasia should be considered in the differential diagnosis of esophageal lesions.

  17. Hypoxia-mimicking bioactive glass regenerative effects on dental stem cells

    Science.gov (United States)

    Noor, Siti Noor Fazliah Mohd; Azevedo, Maria; Mohamad, Hasmaliza; Autefage, Hélène

    2016-12-01

    Vascularization is an important aspect of tissue regeneration. Hypoxia, low oxygen concentration, is a known stimulus for the release of vascular endothelial growth factors (VEGF) which play important roles in vascularization. The current study aimed to assess the effect of a cobalt-containing bioactive glass (BG) in stimulating hypoxia and promoting vascularization. To incorporate cobalt into BG, 1 mol% of calcium was substituting with cobalt, and this formulation was compared to the one without cobalt. Both BGs were processed via melt-derived method. The BG powders with particle size less than 38 µm were incubated with cell culture medium for 4 hours at 37°C on continuous rolling, and then the medium was filtered using 0.22 µm syringe filters. Prior to use, the BG-conditioned media were supplemented with 10% (v/v) fetal bovine serum and 1% (v/v) antibiotic-antimycotic, and were allowed to equilibrate overnight inside a CO2 incubator. The conditioned media were used on human dental stem cells (stem cells from permanent (DPSC) and deciduous (SHED) teeth) and assessed for their capacity to stimulate the release of angiogenic factors from the cells. The results showed that cobalt ions were released from the cobalt-containing BG, following partial dissolution of the glasses in cell culture medium, and promoted VEGF release from the cells. In conclusion, the incorporation of cobalt in BG may have potential to be used for tissue regeneration by promoting vascularization through the activation of hypoxia pathway and the release of VEGF.

  18. Contribution to the radiological study of the eosinophilic granuloma of the mandible (Unifocal granuloma due to Langherans' cell histiocytosis); Contributo allo studio radiologico del granuloma eosinofilo della mandibola (granuloma unifocale da istiocitosi delle cellule di Lagherhans)

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    Chigi, Gino; Pastremoli, Alessandro; Pisi, Paolo; Pastremoli, Alfredo [Bologna Univ., Bologna (Italy). Dipartimento di scienze odontomastologiche; Bianchi, Giuseppe [Istituti Ortopedici Rizzoli, Bologna (Italy)

    2005-04-01

    Purpose: The radiological diagnosis of osteolytic lesions of the mandible still constitutes a challenge in some pathological conditions in which the clinical data and the case history are relatively uniform and the radiological picture is lacking in any characteristics. Materials and methods: We reviewed the conventional radiograms of six cases of Langherans' cell histiocytosis (LCH) of the mandible examined over the last ten years. The X-ray examinations were performed in the lateral-oblique projection to allow a view of the horizontal portion of the mandible almost completely free of overlapping images of other bone structures. Results: We identified a series of radiological patterns for these reticulo-endotheliopathies capable of causing granuloma formed by polinuclear eosinophils, plasma cells, lymphocytes, and large mononuclear macrophages with granulopexic and phagocytic activity that proliferate in the bone tissue and can be identified as the Langherans' cells of skin, mucosa, periodontal cavities and bone marrow. The granulomatous tissue penetrates the affected organ elements and, in its spread, it compresses, atrophies, and destroyed the damaged tissue, replacing it. The alterations produced by Langherans' cell histiocytic granuloma are most common in the skeletal system affecting, in order of frequency, the skull, the long bones of the limbs, the foot, the ribs and the spine. The oral mucosa is rarely involved. Conclusions: The radiological investigation of unifocal Langherans' cell histiocytic granuloma of the mandible is essential in the study of perimandibular swelling, although diagnosis is based on biopsy alone. In addition to digital or conventional radiography, other useful examinations are bone scintigraphy, Colour Doppler US, MR and CT, which enables a correct localisation necessary for planning the biopsy and treatment. [Italian] Scopo: La diagnosi radiologica delle lesioni osteolitiche della mandibola costituisce un problema

  19. Orbital wall infarction mimicking periorbital cellulitis in a patient with sickle cell disease

    Energy Technology Data Exchange (ETDEWEB)

    Ozkavukcu, Esra; Fitoz, Suat; Erden, Ilhan [Ankara University School of Medicine, Department of Radiology, Ankara (Turkey); Yagmurlu, Banu [Kirikkale University School of Medicine, Department of Radiology, Kirikkale (Turkey); Ciftci, Ergin [Ankara University School of Medicine, Department of Paediatric Infectious Diseases, Ankara (Turkey); Ertem, Mehmet [Ankara University School of Medicine, Department of Paediatric Haematology, Ankara (Turkey)

    2007-04-15

    Orbital wall infarction and subperiosteal haematomas are unusual manifestations of sickling disorders. Here we report an 11-year-old girl with sickle cell anaemia having multiple skull infarctions including the orbital bony structures associated with subperiosteal haematomas. The diagnosis was made by MRI, which showed bone marrow changes and associated haemorrhagic collections. The patient was successfully managed without surgical intervention. (orig.)

  20. Real-time dynamics of emerging actin networks in cell-mimicking compartments.

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    Siddharth Deshpande

    Full Text Available Understanding the cytoskeletal functionality and its relation to other cellular components and properties is a prominent question in biophysics. The dynamics of actin cytoskeleton and its polymorphic nature are indispensable for the proper functioning of living cells. Actin bundles are involved in cell motility, environmental exploration, intracellular transport and mechanical stability. Though the viscoelastic properties of actin-based structures have been extensively probed, the underlying microstructure dynamics, especially their disassembly, is not fully understood. In this article, we explore the rich dynamics and emergent properties exhibited by actin bundles within flow-free confinements using a microfluidic set-up and epifluorescence microscopy. After forming entangled actin filaments within cell-sized quasi two-dimensional confinements, we induce their bundling using three different fundamental mechanisms: counterion condensation, depletion interactions and specific protein-protein interactions. Intriguingly, long actin filaments form emerging networks of actin bundles via percolation leading to remarkable properties such as stress generation and spindle-like intermediate structures. Simultaneous sharing of filaments in different links of the network is an important parameter, as short filaments do not form networks but segregated clusters of bundles instead. We encounter a hierarchical process of bundling and its subsequent disassembly. Additionally, our study suggests that such percolated networks are likely to exist within living cells in a dynamic fashion. These observations render a perspective about differential cytoskeletal responses towards numerous stimuli.

  1. Pulmonary mixed squamous cell and glandular papilloma mimicking adenocarcinoma: a case study and literature review.

    Science.gov (United States)

    Lin, Dongliang; Jiang, Yanxia; Wang, Jigang; Ding, Li; Xin, Fangjie; Zhao, Han; Li, Yujun

    2013-08-01

    Mixed squamous cell and glandular papilloma of the lung is an extremely rare benign neoplasm. Here we present another case of mixed squamous cell and glandular papilloma in a 64-year-old female nonsmoker. Histologically, the tumor was composed of mainly papillary structures covered with squamous, glandular and transitional epithelium. Some glandular structures extending into adjacent bronchiolar and alveolar spaces with mucus were similar to adenocarcinoma. Immunohistochemical analysis showed the different kinds of epithelia had similar immunophenotype. The different components were positive for cytokeratin (CK)7, CK19, CAM5.2, CK5/6, CK34βE12, and TTF-1, but negative for CK20. The transitional morphology and immunohistochemistry indicate the different components likely come from a same kind of progenitor in the bronchiolar wall.

  2. Low grade urothelial carcinoma mimicking basal cell hyperplasia and transitional metaplasia in needle prostate biopsy

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    Julian Arista-Nasr

    2016-04-01

    Full Text Available ABSTRACT Purpose The vast majority of urothelial carcinomas infiltrating the bladder are consistent with high-grade tumors that can be easily recognized as malignant in needle prostatic biopsies. In contrast, the histological changes of low-grade urothelial carcinomas in this kind of biopsy have not been studied. Materials and Methods We describe the clinicopathologic features of two patients with low-grade bladder carcinomas infiltrating the prostate. They reported dysuria and hematuria. Both had a slight elevation of the prostate specific antigen and induration of the prostatic lobes. Needle biopsies were performed. At endoscopy bladder tumors were found in both cases. Results Both biopsies showed nests of basophilic cells and cells with perinuclear clearing and slight atypia infiltrating acini and small prostatic ducts. The stroma exhibited extensive desmoplasia and chronic inflammation. The original diagnosis was basal cell hyperplasia and transitional metaplasia. The bladder tumors also showed low-grade urothelial carcinoma. In one case, the neoplasm infiltrated the lamina propria, and in another, the muscle layer. In both, a transurethral resection was performed for obstructive urinary symptoms. The neoplasms were positive for high molecular weight keratin (34BetaE12 and thrombomodulin. No metastases were found in either of the patients, and one of them has survived for five years. Conclusions The diagnosis of low-grade urothelial carcinoma in prostate needle biopsies is difficult and may simulate benign prostate lesions including basal cell hyperplasia and urothelial metaplasia. It is crucial to recognize low-grade urothelial carcinoma in needle biopsies because only an early diagnosis and aggressive treatment can improve the prognosis for these patients.

  3. Voriconazole-Induced Periostitis Mimicking Chronic Graft-versus-Host Disease after Allogeneic Stem Cell Transplantation.

    Science.gov (United States)

    Sweiss, Karen; Oh, Annie; Rondelli, Damiano; Patel, Pritesh

    2016-01-01

    Voriconazole is an established first-line agent for treatment of invasive fungal infections in patients undergoing allogeneic stem cell transplantation (ASCT). It is associated with the uncommon complication of periostitis. We report this complication in a 58-year-old female undergoing HSCT. She was treated with corticosteroids with minimal improvement. The symptoms related to periostitis can mimic chronic graft-versus-host disease in patients undergoing HSCT and clinicians should differentiate this from other diagnoses and promptly discontinue therapy.

  4. Renal Cell Carcinoma Mimicking Igg4-Related Pseudotumor in Autoimmune Pancreatitis

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    Muhammad Ali Khan

    2014-09-01

    Full Text Available Context Autoimmune pancreatitis is classified into two distinct clinical profiles. Care report Type 1 autoimmunepancreatitis (AIP is considered to be a manifestation of a novel clinicopathological entity called IgG4 related sclerosingdisease, diagnosed using the Mayo Clinic HISORt criteria. Extra-pancreatic manifestations can include involvement of bileducts, salivary gland, lung nodules, thyroiditis, tubulointerstitial nephritis, renal masses, and retroperitoneal fibrosis. Type2 autoimmune pancreatitis on the other hand is confirmed by histologically seen duct centric pancreatitis without elevationof IgG4 or involvement of other organs. In type 1 autoimmune pancreatitis, extrapancreatic manifestations like bile ductstrictures, tubulointerstitial nephritis, renal nodules, retroperitoneal fibrosis respond to steroid therapy. Conclusion Wepresent a case of type 1 autoimmune pancreatitis in which the renal mass did not respond to steroid therapy and was later on found to be renal cell carcinoma. To the best of our knowledge this is only the third reported case of autoimmune pancreatitis in which the patient had renal cell carcinoma. Our case highlights the importance of close follow up of lesions that do not respond to steroid treatment which in this case proved to be renal cell cancer.

  5. Anaplastic large cell lymphoma ALK-negative clinically mimicking alcoholic hepatitis – a review

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    Fernando Peixoto Ferraz de Campos

    2013-10-01

    Full Text Available Anaplastic large cell lymphoma (ALCL, described less than 30 years ago by Karl Lennert and Herald Stein in Kiel, West Germany, is a T-cell or null non-Hodgkin lymphoma, with distinctive morphology (hallmark cells, prominent sinus and/or perivascular growth pattern, characteristic immunophenotype (CD30+, cytotoxic granules protein+, CD3–/+ and specific genetic features as translocations involving the receptor tyrosine kinase called anaplastic lymphoma kinase (ALK on 2p23 and variable partners genes, which results in the expression of ALK fusion protein. The absence of ALK expression is also observed and is associated with poorer prognosis that seen with ALK expression. ALK-negative ALCL is more frequent in adults, with both nodal and extra nodal clinical presentation and includes several differential diagnoses with other CD30+ lymphomas. Liver involvement by ALCL is rare and is generally seen as mass formation; the diffuse pattern of infiltration is even more unusual. The authors present a case of a 72-year-old man who presented clinical symptoms of acute hepatic failure. The patient had a long history of alcohol abuse and the diagnosis of alcoholic hepatitis was highly considered, although the serum lactic dehydrogenase (LDH value was highly elevated. The clinical course was fulminant leading to death on the fourth day of hospitalization. Autopsy demonstrated diffuse neoplastic hepatic infiltration as well as splenic, pulmonary, bone marrow, and minor abdominal lymph nodes involvement by the tumor. Based on morphological, immunophenotypical, and immunohistochemical features, a diagnosis of ALK- negative ALCL was concluded. When there is marked elevation of LDH the possibility of lymphoma, ALCL and other types, should be the principal diagnosis to be considered.

  6. Acute scrotum due to arterial bleeding mimicking non-seminomatous germ cell tumor

    Institute of Scientific and Technical Information of China (English)

    F.Christoph; M.Schradert; A.Amirmaki; K.Miller

    2004-01-01

    Men with testicular tumors usually present with painless increase in testis size incidentally noticed by the patient. We report a case of a young patient presenting as an emergency with acute onset of massive right-sided testicular pain without previous injury. After physical examination testicular torsion could not be excluded. Ultrasound examination of the tesds was suspicious for tesdcular tumor. Surgical exploration of the right testis by inguinal approach was performed revealing subcapsular arterial bleeding due to a small nonseminomatous germ cell tumor non-palpable on clinical examination. (Asian J Andro12004 Dec;6:379-381)

  7. Voriconazole-Induced Periostitis Mimicking Chronic Graft-versus-Host Disease after Allogeneic Stem Cell Transplantation

    Directory of Open Access Journals (Sweden)

    Karen Sweiss

    2016-01-01

    Full Text Available Voriconazole is an established first-line agent for treatment of invasive fungal infections in patients undergoing allogeneic stem cell transplantation (ASCT. It is associated with the uncommon complication of periostitis. We report this complication in a 58-year-old female undergoing HSCT. She was treated with corticosteroids with minimal improvement. The symptoms related to periostitis can mimic chronic graft-versus-host disease in patients undergoing HSCT and clinicians should differentiate this from other diagnoses and promptly discontinue therapy.

  8. Suture Granuloma Mimicking Renal Cell Carcinoma: Magnetic Resonance Imaging (MRI and Pathologic Correlation

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    İbrahim İlker Öz

    2014-11-01

    Full Text Available Solid renal masses are generally distinguished with contrast enhancement and intratumoral fatty foci by radiological examinations. The present of enhancement is most important criteria for diagnosis of malignant lesions. Generally, a contrast enhanced solid mass in kidney is accepted as a neoplasm. Foreign body granuloma is an extraordinary cause of enhanced solid renal mass. This case of a renal suture granuloma demonstrated peripheral enhanced exophytic renal mass mimic renal cell carcinoma, and underwent surgery. At the solid renal mass with different radiological features, biopsy is an option to determining the necessity of surgery as well as the surgical approach.

  9. An unusual oral squamous cell carcinoma of the mandible, mimicked inflammatory hyperplastic lesions: A case report

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    Hamed Hosseinkazemi

    2015-12-01

    Full Text Available Abstract   Squamous cell carcinoma (SCC is the most common malignant tumors of oral cavity. The ratio of men to women is about 2: 1. Generally, it   is admitted that 60% of carcinoma of the mandibular gingival are located in the posterior of premolars. Gingiva is one of the less common sites of oral squamous cell carcinoma (OSCC. Due to the variable clinical and behavioral presentations, it can easily be misdiagnosed as benign neoplasms or other inflammatory reactions. We encountered a 76-year-old woman with an unusual OSCC on the anterior mandibular ridge, imitating inflammatory hyperplastic (IH lesion in May 2013. She complained that her denture was not seated suitably because of a mandibular lesion. After biopsy of the lesion, the surgeon noticed that real bone resorption was not visible in the x-ray image. Then histopathological evaluation detected the OSCC. Patient was referred to the CT-Scan and MRI. Three months later, the lesion recurred, enlarged and extended rapidly and she was emphasized the importance of a secondary surgery in a timely fashion.. She did not accept and then underwent radiotherapy and chemotherapy. In November 2013, the patient passed away because of the progress of OSCC. This case reminded us to keep the possibility of oral SCC in mind while examining every intra-oral lesion.

  10. Primary central nervous system anaplastic large-cell lymphoma mimicking lymphomatosis cerebri.

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    Sugino, Toshiya; Mikami, Takeshi; Akiyama, Yukinori; Wanibuchi, Masahiko; Hasegawa, Tadashi; Mikuni, Nobuhiro

    2013-01-01

    Primary central nervous system lymphoma (PCNSL) is usually diffuse large B-cell lymphoma. Anaplastic large-cell lymphoma (ALCL) rarely occurs in the central nervous system. PCNSL always presents as single or multiple nodular contrast-enhancing mass lesions within T2-hyperintense areas on magnetic resonance imaging (MRI). Infrequently, diffuse infiltrating change with little contrast enhancement called lymphomatosis cerebri can be seen in PCNSL. In this report, we describe a 75-year-old immunocompetent man who had progressive dementia. On MRI, diffuse white matter lesions with little contrast enhancement were observed to gradually progress, which was clinically consistent with his worsening condition. A biopsy specimen revealed non-destructive, diffusely infiltrating, anaplastic large CD30-positive lymphoma, indicating a diagnosis of ALCL. After the biopsy, he was treated by whole brain irradiation (total 46 Gy) and focal boost irradiation (total 14 Gy). However, his performance status worsened and there was no symptom improvement. The patient died 8 months after symptom onset. The clinical course, diagnostic workup, pathologic correlates, and treatment outcomes are described herein.

  11. [A case of papillary renal cell carcinoma mimicking a hemorrhagic renal cyst].

    Science.gov (United States)

    Yamamuro, Taku; Mitsuzuka, Koji; Sato, Masahiko; Izumi, Hideaki; Kawamorita, Naoki; Saito, Hideo; Kaiho, Yasuhiro; Ito, Akihiro; Nakagawa, Haruo; Arai, Yoichi

    2012-12-01

    A right renal cyst was found in a 69-year-old man with stage IV chronic kidney disease on abdominal ultrasonography performed to investigate a right upper abdominal swelling. Aspiration cytology of the cyst revealed no malignancy, but malignancy could not be ruled out on magnetic resonance imaging because of the cyst's wall thickness and heterogeneous contents. At one-year of follow-up, emergent abdominal surgery was performed due to incidental perforation of ascending colon diverticulitis. At that time, cystic fenestration was performed because the large renal cyst obstructed the operative procedure. Pathological examination showed type-1 papillary renal cell carcinoma, and radical nephrectomy was performed after the patient's general condition improved. Hemodialysis was started after the operation, and there has been no disease recurrence for two years.

  12. Occult renal cell carcinoma with acrometastasis and ipsilateral juxta-articular knee lesions mimicking acute inflammation

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    Bhaskar Borgohain

    2012-01-01

    Full Text Available Generally, skeletal peripheral metastases below the elbow and the knee are rare. Skeletal metastases to the hand or foot are very rare; but when they do it may be a revealing clinical finding. Purely lytic lesions are commonly seen in metastases from lung, renal, and thyroid tumors, but they are also known to occur in primary myeloma, brown tumor and lymphomas. A 70-year-old man was brought to the emergency department with acute painful swelling involving his right hand and the right knee. Due to significant accompanying soft tissue swellings cellulitis, acute osteomyelitis and gouty arthropathy were included in the initial differential diagnosis. Radiographs showed pure lytic bony lesion with complete disappearance of lower two third of the second metacarpal, trapezium and trapezoid bones of the right hand along with a lytic subarticular lesion of medial condyle of ipsilateral femur. Chest X-ray (CXR was normal but sonography of the abdomen readily demonstrated a large renal mass, later confirmed at biopsy as renal cell carcinoma (RCC. Clinicians should be cognizant of the strong association between digital acrometastases and renal cell carcinoma in male patients with normal CXR findings. In suspected hand acrometastasis associated with a soft tissue component outside the contours of normal bone, screening the abdomen by sonography should be done prior to bone biopsy and before costly or time-consuming investigations are offered. Metastatic RCC should be included in the differential diagnosis of all unilateral expansile bony lesions of the digit. It is particularly important if such lesion/lesions are accompanied by local inflammation. Screening the abdomen by sonography may be of particular value in such elderly male patient when Chest X-ray shows no abnormality.

  13. 郎罕组织细胞增多症1例及文献回顾%Langerhans' Cell Histiocytosis one Case Report and Literatures Review

    Institute of Scientific and Technical Information of China (English)

    刘立刚; 李军; 汤应雄; 潘铁成; 魏翔

    2006-01-01

    郎罕组织细胞增多症(1angerhans' cell histiocytosis,LCH)是以过量活化的郎罕细胞(1angerhans cell)在身体各组织器官增殖为特征性标志的一组疾病的总称,旧称黄色瘤、网状内皮性肉芽肿等。肺郎罕组织细胞增多症(pulmonary langerhan's cell histiocytosis,PLCH)是其中的1种亚型,为少见肺部疾病,其临床表现多样。本文报道一例以肺部损害和胸壁纵隔损害为共同表现的LCH。

  14. Giant cell arteritis mimicking infiltrative leptomeningeal disease of the optic nerves.

    Science.gov (United States)

    Kornberg, Michael D; Ratchford, John N; Subramaniam, Rathan M; Probasco, John C

    2015-04-09

    A 67-year-old man presented with several days of progressive, painless left eye vision loss. He reported mild jaw claudication but denied headache, scalp tenderness or constitutional symptoms. Examination revealed palpable temporal arteries, blurring of the left optic disc, and 20/100 vision in the left eye with mild relative afferent pupillary defect. Inflammatory markers were sent, and methylprednisolone was initiated for presumptive giant cell arteritis (GCA). Erythrocyte sedimentation rate was normal, however, and C reactive protein was only mildly elevated, prompting further investigation. Orbital MRI revealed nodular enhancement of the optic nerve sheaths bilaterally from optic nerve head to chiasm, raising concern for an infiltrative leptomeningeal process such as sarcoidosis or lymphoma. Methylprednisolone was temporarily stopped while a broad work up for inflammatory and neoplastic causes was pursued. Fluorodeoxyglucose-positron emission tomography ultimately revealed hypermetabolism in the temporal, ophthalmic and occipital arteries suggesting GCA, which was confirmed by temporal artery biopsy. Steroids were restarted, and the patient's vision stabilised.

  15. Squamous Cell Carcinoma of the Bladder Mimicking Interstitial Cystitis and Voiding Dysfunction

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    Colton Prudnick

    2013-01-01

    Full Text Available Squamous cell carcinoma (SCC of the bladder is a relatively uncommon cause of bladder cancer accounting for <5% of bladder tumors in the western countries. SCC has a slight male predominance and tends to occur in the seventh decade of life. The main presenting symptom of SCC is hematuria, and development of this tumor in the western world is associated most closely with chronic indwelling catheters and spinal cord injuries. A 39-year-old Caucasian female presented with bladder and lower abdominal pain, urinary frequency, and nocturia which was originally believed to be interstitial cystitis (IC but was later diagnosed as SCC of the bladder. Presentation of SCC without hematuria is an uncommon presentation, but the absence of this symptom should not lead a practitioner to exclude the diagnosis of SCC. This case is being reported in an attempt to explain the delay and difficulty of diagnosis. Background on the risk factors for SCC of the bladder and the typical presenting symptoms of bladder SCC and IC are also reviewed.

  16. Clinical analysis of Langerhans cell histiocytosis of head and face%头面部郎格罕细胞组织细胞增生症的临床分析

    Institute of Scientific and Technical Information of China (English)

    徐刚; 刘冰; 何刚; 费伟; 谢可

    2013-01-01

    Objective:To study the diagnosis and treatment for Langerhans cell histiocytosis(LCH) of head and face.Method:Clinical data of 19 cases suffering from LCH of head and face verified by pathology was retrospectively analyzed.The clinical presentation,diagnosis and treatment were discussed.Result:Of all the 19 cases,5 patients had an involvement of the scalp or skull,5 had the maxilla involvement and 9 temporal bone.Among the 19 patients,type Ⅰ stage Ⅰ was 8 cases(all adults),type Ⅱ stage Ⅰ 7 cases(all children),type Ⅲ stage Ⅱ 2(1 adult,1 child),stage Ⅲ 1 and stage Ⅳ 1(both child).Their main clinical manifestations included scalp mass,defect of skull,swelling or mass of facial maxilla and of ear or temporal bone,otorrhea,granuloma of auricular canal,and so on.Fifteen cases were cured by surgical curettage combined with radiotherapy and 4 by surgical curettage combined with chemotherapy.One patient had the sequelae of diabetes insipidus.Conclusion:Diagnosis of LCH should be based on synthetical analyses of clinical manifestations,features of imaging and histopathology.Surgical curettage,radiotherapy and chemotherapy are effective treatment regimens.The prognosis is generally good if the patient gets correct diagnosis and timely rational treatment.%目的:研究头面部郎格罕细胞组织细胞增生症(LCH)的诊断和治疗.方法:回顾分析19例经病理证实的头面部LCH患者的临床资料.对其临床表现、诊断和治疗进行探讨.结果:19例中发生于头皮颅骨5例,上颌骨5例,颞骨9例.Ⅰ型Ⅰ级8例(均为成人),Ⅱ型Ⅰ级7例(均为小儿),Ⅲ型Ⅱ级2例(小儿1例,成人1例),Ⅲ级1例(小儿),Ⅳ级1例(小儿).临床主要表现为头皮包块、颅骨缺损、面颌部肿胀或包块、耳颞肿胀、耳漏、耳道肉芽等.15例接受手术加放疗后痊愈,4例手术后接受化疗治愈.遗留尿崩症1例.结论:根据临床表现、影像学及组织病理学特征可对LCH做出诊断.手术、放疗

  17. 郎格罕细胞组织细胞增生症57例临床分析%Clinical analysis of Langerhans cell histiocytosis (LCH) in 57 children

    Institute of Scientific and Technical Information of China (English)

    赵彦; 杨斌; 黄乐; 吕玲

    2012-01-01

    目的 探讨儿童郎格罕细胞组织细胞增生症的临床特点.方法 收集某院57例住院患儿资科,分析其临床、病理及免疫组织化学特点.结果 平均发病年龄2.34岁,男女发病比例1.48∶1.皮肤累及率50.88%.皮疹形态多样,以头面部、躯干部出血性斑丘疹、脂溢性或丘疹样皮疹多见,部分为疱疹、结痂、色素脱失.发热、肝脾大发生率分别为54.39%、56.14%.淋巴结累及率31.58%,以颈部和腹股沟淋巴结为主.骨质浸润发生率24.56%,主要累及颅骨,还可累及锁骨和四肢骨等.结论 儿童郎格罕细胞组织细胞增生症可累及多个脏器,表现多样,以年龄分组比较,皮疹在婴幼儿组更多见,其他主要临床表现两组无显著差异.%OBJECTIVE To explore the clinical features of Langerhans cell histiocytosis (LCH) in children. METHODS 57 cases of LCH from were collected from inpatient department of our hospital The features of clinic, pathology and immunohisto-chemistry were analyzed. RESULTS The mean age of onset was 2.34 years old. The male/female ratio was 1.48 :1. Skin lesions occurred in 50.88% of the patients. The eruptions mainly distributed on trunk and head, some of them presented as hemorrhagic maculopapules, sebaceous flux erythra and papular eruption. Other of the eruptions showed as emphlysis and incontinence of pigmentation. Fever and hepatosplenomegia occurred in patients with a rate of 54.39% and 56.14%, respectively. 31.58% of the patients had got lymphadenectasis, the neck and inguinal lymph nodes were the common site to be affected. Ossature involvement occurred in 24.56% of the patients. Skull was the main site to be injured, and the others were clavicula, limbs bones and so on. CONCLUSION Multiple systems can be involved in LCH of children. After the comparison between presentation of different age children, we found rash was more common presentation in younger group, and there was no difference between two groups

  18. 颅脑朗格汉斯细胞组织细胞增生症的影像学诊断%Imaging Diagnosis of Langerhans Cell Histiocytosis in Skull and Brain

    Institute of Scientific and Technical Information of China (English)

    袁小平; 潘恒; 黄穗乔

    2016-01-01

    Objective To discuss imaging diagnosis of Langerhans cell histiocytosis(LCH) in skull and brain for definite clinical diagnosis. Methods A retrospective analysis was conducted in 32 cases which were conifrmed by operation and pathology in our hospital.ResultsAmong 32 cases, there were 21 cases with skull, 8 cases of with hypothaamus and stalk hypophysia., 3 cases with cerebral brain.32 cases,21cases with CT, 25 cases with MR examination, both CT and MR data of 14 cases. Aged 3~35 years old, average (16.8±1.03) years. The disease occurs in skull and brain has the following characteristics:(1)The disease usually onset in children and adolescents. (2)The lesion skull bone destruction of bone diploe centered, destroy the edge is clear, rare necrosis, occur in the temporal bone mastoid area easily misdiagnosed as malignant tumor. (3)The brain often occur the hypothalamus, pituitary stalk, visible enlargement of the hypothalamus, pituitary stalk. Cranial nerve degeneration and the occasional parenchymal lesions are changing.Conclusions LCH in skull and brain have certain characteristic imaging findings, combined with the clinical, pathological, beneifcial help to analysis and diagnosis the disease.%目的:探讨颅脑朗格汉斯细胞组织细胞增生症的影像学诊断,以利于临床诊断。方法对我院32例颅脑朗格汉斯细胞组织细胞增生症患者的影像学表现进行回顾性分析,患者均有手术病理结果证实。结果32例,其中发生颅骨21例,下丘脑、垂体柄8例,脑实质3例;32例,CT检查21例,MR检查25例,有CT及MR资料14例。年龄3~35岁,平均(16.8±1.03)岁;本病发生在颅脑有以下特点:(1)发病年龄轻,儿童或青少年多见;(2)病变颅骨骨破坏以骨板障为中心,破坏边缘清楚,少见坏死,发生在颞骨乳突区易误诊恶性肿瘤;(3)脑部常发生下丘脑、垂体柄,可见下丘脑、垂体柄增粗;偶见脑实质病变及

  19. MRI features of Langerhans cell histiocytosis in central nervous system in children%儿童中枢神经系统郎格汉斯细胞组织细胞增生症的MRI表现

    Institute of Scientific and Technical Information of China (English)

    曾洪武; 干芸根; 黄文献; 何玲; 刘鸿圣

    2016-01-01

    Objective To explore the MRI features of Langerhans cell histiocytosis (LCH) in central nervous system (CNS) in children. Methods Clinical and MRI data of 25 cases with LCH in CNS from three children's hospitals between January 2009 and December 2014 were retrospectively studied. All cases were confirmed by surgery or biopsy. All cases underwent non⁃contrast and contrast pituitary and/or cranial MRI examinations. The location, morphology, MRI signal and enhanced patterns of LCH lesions were observed and analyzed. Result A total of 17 cases had hypothalamic-pituitary LCH, with 2 of them complicated with pineal cyst, 2 complicated with brain parenchymal lesions, and one complicated with both pineal cyst and brain parenchymal lesions. MR images showed that neurohypophysis lost its original hyper⁃intensity on T1WI, and nodular or homogeneous thickening was seen in the pituitary stalks. Dura matter was involved in 3 cases, 2 of them had single lesion, and the other one got multiple lesions. Neoplasm in choroid plexus was seen in trigone of left lateral ventricles in one case. Three cases with pineal gland involved demonstrated cystic change. Four cases had gray matter involved, with cerebellar dentate nuclei involvement in 2 cases, and both thalamus and basal ganglia involvement in the other two cases. Three cases showed white matter involvement without obvious Virchow-Robin space enlargement and brain atrophy. Conclusions MR imaging of LCH in CNS shows certain specific characteristics. The diagnosis can be made comprehensively based on both clinic features and other imaging findings.%目的:探讨儿童中枢神经系统郎格汉斯细胞组织细胞增生症(LCH)的MRI表现。方法回顾性分析2009年1月至2014年12月3家儿童医院经手术或穿刺活检证实的中枢神经系统LCH病例25例,所有病例均行垂体和(或)头颅MR平扫及增强扫描。重点观察神经系统受累病灶部位、形态、MRI信号变化及强化情况

  20. 郎格罕氏组织细胞增生症的电镜研究%Electron microscopic studies on Langerhans-cell histiocytosis

    Institute of Scientific and Technical Information of China (English)

    郑晓刚; 陈旭东; 姜少军; 陆珍凤; 周晓军

    2006-01-01

    目的:探讨郎格罕氏组织细胞增生症(LCH)的超微病理特点,提供Birbeck颗粒来源的线索.方法:结合光镜、免疫组化及临床资料,对2例LCH进行了超微结构观察.结果:朗格罕组织细胞胞浆内可见棒状、网球拍状Birbeck颗粒,这些细胞免疫组化CD1a阳性.结论:LCH的诊断须见到典型的郎格罕氏细胞(Langerhans cell,LC)LC细胞,电镜下找到Birbeck颗粒,或CD1a(O10)阳性.Birbeck颗粒可与细胞膜相连续,细胞膜凹陷可在胞质内形成Birbeck颗粒.

  1. Histiocytosis X and Bronchopulmonary Adenocarcinoma: A Rare Coexistence

    Directory of Open Access Journals (Sweden)

    Akýn Kaya

    2002-01-01

    Full Text Available There exists a rarely observed association between pulmonary histiocytosis X and bronchopulmonary cancer. However, the frequency of bronchopulmonary cancer in these patients is higher than in the general population. A 28-year-old patient who currently smokes ten packs of cigarettes a year came to our department of pneumology with complains of cough and hemoptysis. An x-ray of the thorax revealed bilateral cysts and a shadow in the upper part of the right pulmonary field. In addition, a chest tomography showed multiple cysts dispersed throughout the two pulmonary fields and an irregular mass with a diameter of four centimetres in the upper right lobe. Bronchopulmonary adenocarcinoma was diagnosed during a cytologic exam of the bronchial washing. We decided to perform a thoracotomy on the patient, since there was no far metastasis. An upper lobectomy and wedge resection of the upper segment of the lower right lobe, which had been invaded by the tumour, were performed. Histology confirmed the diagnosis of adenocarcinoma. A pulmonary biopsy was carried out on the tumour-free site and showed the presence of histiocytosis X. There is a hypothesis that a neoplasm developed on the pulmonary fibrosis could be an epiphenomenon of bronchopulmonary cancer in patients who smoke and have pulmonary histiocytosis X. It is interesting to note that histiocytosis X and bronchopulmonary cancer were diagnosed at the same time, since the bronchopulmonary cancer may have occurred within a few years following the diagnosis of histiocytosis X, even if she was a smoker. Hemoptysis, which is found in 5% of patients with histiocytosis X, may suggest cancer. This young patient, a smoker, who complained of hemoptysis, is a particularly rare case of the association between pulmonary histiocytosis X and bronchopulmonary cancer whose pathogenesis is not clear cut. It is thus important to note that smoking can have major consequences, even in young people.

  2. 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.

  3. Chitosan microspheres with an extracellular matrix-mimicking nanofibrous structure as cell-carrier building blocks for bottom-up cartilage tissue engineering.

    Science.gov (United States)

    Zhou, Yong; Gao, Huai-Ling; Shen, Li-Li; Pan, Zhao; Mao, Li-Bo; Wu, Tao; He, Jia-Cai; Zou, Duo-Hong; Zhang, Zhi-Yuan; Yu, Shu-Hong

    2016-01-07

    Scaffolds for tissue engineering (TE) which closely mimic the physicochemical properties of the natural extracellular matrix (ECM) have been proven to advantageously favor cell attachment, proliferation, migration and new tissue formation. Recently, as a valuable alternative, a bottom-up TE approach utilizing cell-loaded micrometer-scale modular components as building blocks to reconstruct a new tissue in vitro or in vivo has been proved to demonstrate a number of desirable advantages compared with the traditional bulk scaffold based top-down TE approach. Nevertheless, micro-components with an ECM-mimicking nanofibrous structure are still very scarce and highly desirable. Chitosan (CS), an accessible natural polymer, has demonstrated appealing intrinsic properties and promising application potential for TE, especially the cartilage tissue regeneration. According to this background, we report here the fabrication of chitosan microspheres with an ECM-mimicking nanofibrous structure for the first time based on a physical gelation process. By combining this physical fabrication procedure with microfluidic technology, uniform CS microspheres (CMS) with controlled nanofibrous microstructure and tunable sizes can be facilely obtained. Especially, no potentially toxic or denaturizing chemical crosslinking agent was introduced into the products. Notably, in vitro chondrocyte culture tests revealed that enhanced cell attachment and proliferation were realized, and a macroscopic 3D geometrically shaped cartilage-like composite can be easily constructed with the nanofibrous CMS (NCMS) and chondrocytes, which demonstrate significant application potential of NCMS as the bottom-up cell-carrier components for cartilage tissue engineering.

  4. Spindle Cell Hemangioendothelioma of the Temporal Muscle Resected with Zygomatic Osteotomy: A Case Report of an Unusual Intramuscular Lesion Mimicking Sarcoma

    Directory of Open Access Journals (Sweden)

    Tomohiro Minagawa

    2011-01-01

    Full Text Available Spindle cell hemangioendothelioma (SCH was originally described by Weiss and Enzinger (1986 as a low-grade angiosarcoma resembling both cavernous hemangioma and Kaposi's sarcoma. Recent studies suggest that SCH is a benign neoplasm or reactive lesion accompanying a congenital or acquired vascular malformation. Most SCHs present as one or more nodules affecting the dermis or subcutis of the distal extremities. Few reports describe SCH of the head and neck region; even fewer note intramuscular SCH. Here, we describe a case of SCH involving the temporal muscle mimicking soft tissue sarcoma, who had a successful surgical treatment with a coronal approach and zygomatic osteotomy.

  5. MRI features of neurodegenerative Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Martin-Duverneuil, N.; Guillevin, R.; Chiras, J. [GH Pitie-Salpetriere, 47 Bd de l' Hopital, Department of Neuroradiology, Paris (France); Idbaih, A.; Hoang-Xuan, K. [GH Pitie-Salpetriere, 47 Bd de l' Hopital, Department of Neurology, Paris (France); Donadieu, J. [GH Trousseau, Department of Hematology/Oncology Pediatrics, Paris (France); Genereau, T. [Groupe d' etude des Histiocytoses langerhansiennes, Nantes (France)

    2006-09-15

    CNS complications of LCH include ''space occupying'' lesions corresponding to histiocytic granulomas and ''neurodegenerative'' presentation (ND-LCH) characterized by a progressive cerebellar ataxia. Studies analyzing specifically the MRI presentation of ND-LCH are scarce. We present here the MRIs of 13 patients registered as isolated ND-LCH. Posterior fossa was involved in 12 patients (92%), showing a symmetrical T2 hyperintensity of the cerebellar white matter areas in seven cases with a circumscribed T1 hyperintensity of the dentate nuclei in five cases, definite hyperintense T2 areas in the adjacent pontine tegmentum white matter in nine cases associated with a hyperintensity of the pontine pyramidal tracts in four cases. A cerebellar atrophy was noted in eight cases. The supratentorial region was involved in 11 patients, showing T2 hyperintense lesions in the cerebral white matter in eight cases and a discrete symmetrical T1 hyperintense signal in the globus pallidus in eight patients. A diffuse cortical atrophy was present in three cases and a marked focal atrophy of the corpus callosum in three cases. This series allows us to establish a not previously reported evocative semeiologic MR presentation to precisely orientate to the diagnosis of the pure neurodegenerative form of LCH. (orig.)

  6. Radiological features of thymic langerhans cell histiocytosis.

    Science.gov (United States)

    Lakatos, Karoly; Herbrüggen, Heidrun; Pötschger, Ulrike; Prosch, Helmut; Minkov, Milen

    2013-11-01

    TI was reported in 18/1,264 (1.4%) LCH patients. All nine patients with TI at initial LCH presentation were below 2 years of age and had multisystem LCH (9/242, 4%). Images (sonography, CT, MRI) for central review were available in 15 cases. Characteristic findings of TI were thymus enlargement (67%), few to many cysts (80%), and few to many calcifications (100%). Sonographic and MRI findings were in excellent agreement. We recommend adding sonography of the thymus to the standard for initial clinical evaluation of LCH patients below the age of 2 years.

  7. Growth of children with Langerhans cell histiocytosis

    NARCIS (Netherlands)

    A.C.J. van den Hoek (A. C J); A. Karstens (A.); R.M. Egeler (Maarten); K. Hählen (Karel)

    1995-01-01

    textabstractConclusion: GH deficiency is not a common manifestation of LCH in childhood and GH provocation tests are only indicated when there is a poor or decelerating growth rate. In our patients the number of organs involved and/or the treatment modality did not influence the growth in all but on

  8. Genetics Home Reference: Langerhans cell histiocytosis

    Science.gov (United States)

    ... also frequently occur in the skin, appearing as blisters, reddish bumps, or rashes which can be mild ... be inherited? More about Inheriting Genetic Conditions Diagnosis & Management Genetic Testing (1 link) Genetic Testing Registry: Langerhans ...

  9. General Information about Langerhans Cell Histiocytosis (LCH)

    Science.gov (United States)

    ... slow growth, and late puberty. Trouble breathing. Central nervous system (CNS) Signs or symptoms of LCH that ... or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against ...

  10. Leukocyte-Mimicking Stem Cell Delivery via In situ Coating of Cells with a Bioactive Hyperbranched Polyglycerol (HPG)

    Science.gov (United States)

    Jeong, Jae Hyun; Schmidt, John J.; Kohman, Richie E.; Zill, Andrew T.; DeVolder, Ross J.; Smith, Cartney E.; Lai, Mei-Hsiu; Shkumatov, Artem; Jensen, Tor W.; Schook, Lawrence G.; Zimmerman, Steven C.; Kong, Hyunjoon

    2013-01-01

    Since stem cells emerged as a new generation of medicine, there are increasing efforts to deliver the stem cells to a target tissue via intravascular injection. However, the therapeutic stem cells lack a capacity to detect and adhere to the target tissue. Therefore, this study presents synthesis of a bioactive hyper-branched polyglycerol (HPG) which can non-invasively associate with stem cells and further guide them to target sites, such as inflamed endothelium. The overall process is analogous to the way in which leukocytes are mobilized to the injured endothelium. PMID:23590123

  11. Leukocyte-mimicking stem cell delivery via in situ coating of cells with a bioactive hyperbranched polyglycerol.

    Science.gov (United States)

    Jeong, Jae Hyun; Schmidt, John J; Kohman, Richie E; Zill, Andrew T; DeVolder, Ross J; Smith, Cartney E; Lai, Mei-Hsiu; Shkumatov, Artem; Jensen, Tor W; Schook, Lawrence G; Zimmerman, Steven C; Kong, Hyunjoon

    2013-06-19

    Since stem cells emerged as a new generation of medicine, there are increasing efforts to deliver stem cells to a target tissue via intravascular injection. However, the therapeutic stem cells lack the capacity to detect and adhere to the target tissue. Therefore, this study presents synthesis of a bioactive hyperbranched polyglycerol (HPG) that can noninvasively associate with stem cells and further guide them to target sites, such as inflamed endothelium. The overall process is analogous to the way in which leukocytes are mobilized to the injured endothelium.

  12. [THE SPONTANEOUS PNEUMOTHORAX INTERPRETATION, DIFFICULTIES DURING HISTIOCYTOSIS X].

    Science.gov (United States)

    Duzhyi, I D; Holubnychyi, S A; Holubnucha, V N; Pustovoy, I A

    2014-01-01

    Since spontaneous pneumothorax can be a complication, and sometimes a manifestation of significant amount of intrathoracic and extrathoracic diseases, verifying the process can be very difficult. One of these diseases is the local (pulmonary) histiocytosis X. Authors cite the etiological diagnosis experience of spontaneous pneumothorax during current pathology. According to their data, spontaneous pneumothorax on the histiocytosis X background found in 3 patients. Complications developed during the disease later stages, and therefore the verification was complex and based on morphological data, consistent with the clinical and radiographic changes.

  13. Oral manifestations of chronic disseminated histiocytosis. A report of 10 cases.

    Science.gov (United States)

    Mínguez, Ignacio; Mínguez, Juan Manuel; Bonet, Jaime; Peñarrocha, Miguel; Sanchis, José María

    2004-01-01

    Chronic disseminated histiocytosis is a systemic disorder resulting from tumor proliferation of Langerhans-type histiocytic cells. The etiology and pathogenesis are not fully clear, though the clinical manifestations are the result of the accumulation and infiltration of these types of cells in organs and tissues. The present study reports 10 patients (6 boys and 4 girls) with chronic disseminated histiocytosis. The patient age at onset of the disease varied from 4 months to 3.2 years (mean 1.7 years). All patients had oral lesions, and in 5 cases these were the first manifestation of the disease. The most frequent alterations were gingival bleeding (7 cases), aphthae measuring over 1 cm in diameter (6 cases), maxillary osteolytic lesions (6 cases), tooth loss due to expulsive folliculitis (5 cases), oral candidiasis (4 cases), orofacial swelling (3 cases), aphthae measuring under 1 cm in diameter (3 cases), and nonspecific oral pain (2 cases). All the oral lesions disappeared with the treatments prescribed, though some patients developed new outbreaks and exacerbations of the disease.

  14. High resolution CT findings of pulmonary Langerhans cell histiocytosis in children%儿童郎格汉斯细胞组织细胞增生症肺部受累的高分辨率CT表现

    Institute of Scientific and Technical Information of China (English)

    李素荣; 闫淯淳; 曹玲; 袁新宇; 郭红伟

    2016-01-01

    Objective To summarize the lung HRCT findings of lung Langerhans cell histiocytosis (LCH) in children. Methods A total of 54 children with lung LCH pathologically proved in our institute from September 2006 to December 2014 were retrospectively reviewed. Patients were subdivided into two subgroups, diffuse type (n=29) and localized type (n=25). In addition, 32 children with LCH but without pulmonary infiltration were selected as control group. HRCT findings of 54 LCH with lung involvement were reviewed and analyzed for distribution pattern. The age of onset, illness duration and pulmonary function were compared between the lung LCH group and the control group. The differences of HRCT findings in LCH patients between diffuse type and localized type were compared with χ2 test. The differences on the onset age, illness duration and pulmonary function between the LCH group with lung involvement and control group were compared with non-parametric test(Mann-Whitney U Test). The differences of pulmonary function between the LCH group with lung involvement and control group were compared with Fisher exact probability test. Results The signs of pulmonary LCH on HRCT included lobular hyperinflation (n=26), interlobular septa thickening (n=11), cystic lesions (n=11), ground⁃glass opacity (n=10), nodules (n=9), and centrilobular nodules (n=6). The signs presented in 21, 8, 7, 9, 4 and 6 cases in diffuse type group respectively and 5, 3, 4, 1, 5, 0 in localized type group. Among them, lobular hyperinflation, ground⁃glass opacity and centrilobular nodules were more prevalent in the diffuse group. The difference was statistically significant (χ2=14.77,2.01,0.55,4.84,0.06 and 3.91, P0.05) and pulmonary function between two groups (P>0.05). Conclusions Lobular hyperinflation, ground glass opacity and centrilobular nodules are the most common manifestations in LCH patients with lung involvement and the distribution is mostly diffuse. Nodules, cystic lesions and interlobular

  15. In vitro investigation of a tissue-engineered cell-tendon complex mimicking the transitional architecture at the ligament-bone interface.

    Science.gov (United States)

    Wang, Zhibing; Zhang, Yuan; Zhu, Jie; Dong, Shiwu; Jiang, Tao; Zhou, Yue; Zhang, Xia

    2015-03-01

    Restoration of the transitional ligament-bone interface is critical for graft-bone integration. We postulated that an allogenic scaffold mimicking the fibrogenic, chondrogenic, and osteogenic transition gradients could physiologically promote ligament-bone incorporation. The aim of this study was to construct and characterize a composite tendon scaffold with a continuous and heterogeneous transition region mimicking a native ligament insertion site. Genetically modified heterogeneous cell populations were seeded within specific regions of decellularized rabbit Achilles tendons to fabricate a stratified scaffold containing three biofunctional regions supporting fibrogenesis, chondrogenesis, and osteogenesis. The observed morphology, architecture, cytocompatibility, and biomechanics of the scaffolds demonstrated their improved bio-physico-chemical properties. The formation of the transitional regions was augmented via enhanced delivery of two transcription factors, sex determining region Y-box 9 and runt-related transcription factor 2, which also triggered early up-regulated expression of cartilage- and bone-relevant markers, according to quantitative PCR and immunoblot analyses. Gradient tissue-specific matrix formation was also confirmed within the predesignated regions via histological staining and immunofluorescence assays. These results suggest that a transitional interface could be replicated on an engineered tendon through stratified tissue integration. The scaffold offers the advantages of a multitissue transition involving controlled cellular interactions and matrix heterogeneity, which can be applied for the regeneration of the ligament-bone interface.

  16. Chitosan microspheres with an extracellular matrix-mimicking nanofibrous structure as cell-carrier building blocks for bottom-up cartilage tissue engineering

    Science.gov (United States)

    Zhou, Yong; Gao, Huai-Ling; Shen, Li-Li; Pan, Zhao; Mao, Li-Bo; Wu, Tao; He, Jia-Cai; Zou, Duo-Hong; Zhang, Zhi-Yuan; Yu, Shu-Hong

    2015-12-01

    Scaffolds for tissue engineering (TE) which closely mimic the physicochemical properties of the natural extracellular matrix (ECM) have been proven to advantageously favor cell attachment, proliferation, migration and new tissue formation. Recently, as a valuable alternative, a bottom-up TE approach utilizing cell-loaded micrometer-scale modular components as building blocks to reconstruct a new tissue in vitro or in vivo has been proved to demonstrate a number of desirable advantages compared with the traditional bulk scaffold based top-down TE approach. Nevertheless, micro-components with an ECM-mimicking nanofibrous structure are still very scarce and highly desirable. Chitosan (CS), an accessible natural polymer, has demonstrated appealing intrinsic properties and promising application potential for TE, especially the cartilage tissue regeneration. According to this background, we report here the fabrication of chitosan microspheres with an ECM-mimicking nanofibrous structure for the first time based on a physical gelation process. By combining this physical fabrication procedure with microfluidic technology, uniform CS microspheres (CMS) with controlled nanofibrous microstructure and tunable sizes can be facilely obtained. Especially, no potentially toxic or denaturizing chemical crosslinking agent was introduced into the products. Notably, in vitro chondrocyte culture tests revealed that enhanced cell attachment and proliferation were realized, and a macroscopic 3D geometrically shaped cartilage-like composite can be easily constructed with the nanofibrous CMS (NCMS) and chondrocytes, which demonstrate significant application potential of NCMS as the bottom-up cell-carrier components for cartilage tissue engineering.Scaffolds for tissue engineering (TE) which closely mimic the physicochemical properties of the natural extracellular matrix (ECM) have been proven to advantageously favor cell attachment, proliferation, migration and new tissue formation

  17. S100-Negative, CD1a-Positive Cutaneous Histiocytosis in a Patient with S100-Positive, CD1a-Positive Pulmonary Histiocytosis.

    Science.gov (United States)

    Mask-Bull, Lisa; Crowson, Neil A; John, Andrew; Mask, Neal A

    2015-08-01

    In the diagnostic approach to histiocytic proliferations, immunohistochemistry may be a source of both confusion and clarification. We present a case of a 60-year-old man with a generalized pruritic eruption that demonstrated positive staining for CD1a, but negative staining for langerin and S100 protein. This immunophenotype is neither representative nor characteristic of any recognized dendritic cell tumor but has been previously described in 3 cases of skin-limited histiocytosis. However, our patient also demonstrated pulmonary histiocytic infiltrates that were positive for both CD1a and S100 proteins. This differing expression of S100 protein witnessed in 2 separate organ systems affords us insight into the pathophysiology of these histiocytic proliferations.

  18. Dendritic cells transfected with scFv from Mab 7.B12 mimicking original antigen gp43 induces protection against experimental Paracoccidioidomycosis.

    Directory of Open Access Journals (Sweden)

    Karen S Ferreira

    Full Text Available Paracoccidioidomycosis (PCM, endemic in Latin America, is a progressive systemic mycosis caused by Paracoccidioides brasiliensis (P. brasiliensis, which primarily attacks lung tissue. Dendritic cells (DCs are able to initiate a response in naïve T cells, and they also participate in Th-cell education. Furthermore, these cells have been used for therapy in several disease models. Here we transfected DCs with a plasmid (pMAC/PS-scFv encoding a single chain variable fragment (scFv of an anti-Id antibody that is capable of mimicking gp43, the main antigenic component of P. brasiliensis. First, Balb/c mice were immunized subcutaneously with pMAC/PS-scFv and, after seven days, scFv protein was presented to the regional lymph nodes cells. Moreover, we showed that the DCs transfected with scFv were capable of efficiently activating proliferation of total lymph node cells and inducing a decrease in lung infection. Therefore, our results suggested that the use of scFv-transfected DCs may be a promising therapy in the paracoccidioidomycosis (PCM model.

  19. Dendritic cells transfected with scFv from Mab 7.B12 mimicking original antigen gp43 induces protection against experimental Paracoccidioidomycosis.

    Science.gov (United States)

    Ferreira, Karen S; Maranhão, Andrea Q; Garcia, Maria C C; Brígido, Marcelo M; Santos, Suelen S; Lopes, José D; Almeida, Sandro R

    2011-01-07

    Paracoccidioidomycosis (PCM), endemic in Latin America, is a progressive systemic mycosis caused by Paracoccidioides brasiliensis (P. brasiliensis), which primarily attacks lung tissue. Dendritic cells (DCs) are able to initiate a response in naïve T cells, and they also participate in Th-cell education. Furthermore, these cells have been used for therapy in several disease models. Here we transfected DCs with a plasmid (pMAC/PS-scFv) encoding a single chain variable fragment (scFv) of an anti-Id antibody that is capable of mimicking gp43, the main antigenic component of P. brasiliensis. First, Balb/c mice were immunized subcutaneously with pMAC/PS-scFv and, after seven days, scFv protein was presented to the regional lymph nodes cells. Moreover, we showed that the DCs transfected with scFv were capable of efficiently activating proliferation of total lymph node cells and inducing a decrease in lung infection. Therefore, our results suggested that the use of scFv-transfected DCs may be a promising therapy in the paracoccidioidomycosis (PCM) model.

  20. An unusual form of localized papulonodular cutaneous histiocytosis in a 6-month-old boy.

    Science.gov (United States)

    van Haselen, C W; Toonstra, J; den Hengst, C W; van Vloten, W A

    1995-09-01

    We report a 6-month-old boy with an unusual form of cutaneous histiocytosis. The lesions were noticed shortly after birth, and there was no evidence of systemic disease. This histiocytic disorder could not be classified according to the Histiocyte Society classification, and was therefore designated an 'unclassified' group II histiocytic disorder. The clinical picture was characterized by dark-red papulonodules with a tendency to coalesce into plaques. Histologically, the infiltrate was characterized by non-epidermotropic histiocytes showing varying degrees of differentiation, eosinophils and lymphocytes, and by the absence of foamy cells and Touton giant cells. As a most conspicuous feature, electron microscopic examination revealed laminated dense bodies, whereas Birbeck granules and comma-shaped bodies were absent. This further distinguished this uncommon variant from the well-known class II histiocytoses. During a 6-month follow-up period all the lesions showed marked regression.

  1. The acquired radioresistance in HeLa cells under conditions mimicking hypoxia was attenuated by a decreased expression of HIF subunit genes induced by RNA interference

    Energy Technology Data Exchange (ETDEWEB)

    Doi, Nobutaka [Department of Radiological Sciences, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Toyama 930-0194 (Japan); New Products Research & Development, Gene Engineering Division, NIPPON GENE Co., Ltd. (Japan); Ogawa, Ryohei, E-mail: ogawa@med.u-toyama.ac.jp [Department of Radiological Sciences, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Toyama 930-0194 (Japan); Cui, Zheng-Guo [Department of Public Health, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama (Japan); Morii, Akihiro; Watanabe, Akihiko [Department of Urology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama (Japan); Kanayama, Shinji; Yoneda, Yuko [New Products Research & Development, Gene Engineering Division, NIPPON GENE Co., Ltd. (Japan); Kondo, Takashi [Department of Radiological Sciences, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Toyama 930-0194 (Japan)

    2015-05-01

    The cancer cells residing in the hypoxic layer are resistant to radiation and these are ones responsible for cancer recurrence after radiation therapy. One of the reasons why hypoxic cancer cells acquire radioresistance may be attributable to changes in the gene expression profile by the activation of hypoxia inducible factors (HIFs). However, the details underlying this process remain unknown. In this study, we investigated the effects of knockdown of HIF subunit genes to elucidate how HIF subunit genes may be involved in the radioresistance acquired by HeLa cells following exposure to a hypoxia mimic. Interestingly, HIF-1α and HIF-2α seemed mutually complementary for each other when either of them was suppressed. We thus suppressed the expression of both genes simultaneously. To do this, we developed a short hairpin RNA (shRNA) targeting a high homology region between HIF-1α and HIF-2α. It was shown that the expression of the shRNA effectively suppressed the acquisition of radioresistance following the hypoxia mimic. Moreover, it was confirmed that suppression of both subunits resulted in the downregulation of stem cell markers and the suppression of spheroid formation during the hypoxia mimicking-conditions. This shRNA-mediated knockdown method targeting a common region shared by a family of genes may offer a new candidate cancer treatment. - Highlights: • Incubation with CoCl{sub 2} confers radioresistance to HeLa cells. • Both HIF-1α and HIF-2α are involved in the acquisition of radioresistance. • An shRNA to a homology region of HIF-1α and HIF-2α suppressed the radioresistance. • The shRNA decreased cells with stem cell markers and a stem cell phenotype.

  2. Malignant histiocytosis: a clinicopathologic study of 18 consecutive cases.

    Science.gov (United States)

    Rilke, F; Carbone, A; Musumeci, R; Pilotti, S; De Lena, M; Bonadonna, G

    1978-04-30

    The clinical records and histologic material of 18 consecutive patients with malignant histiocytosis were reviewed. The age of the patients ranged from 20 months to 72 years (median 35 years). There were 14 males and 4 females (3.5:1). Lymph node and liver enlargement, fever, and skin nodules were the most common physical findings; and leukocytosis was frequently the most abnormal laboratory test. Seven of 18 patients died, and their survival ranged from 1 to 15 months (median 8 months) after histopathologic diagnosis. The histologic findings on lymph nodes, spleen, liver, bone marrow, and skin were investigated with special reference to both the cellular composition and the pattern of lymph node involvement. Vascular invasion of small perinodal vessels was observed in 4 fatal cases. The absence of capsular invasion and the lack of cohesiveness among atypical proliferating histiocytes of malignant histiocytosis appeared to be inconstant. Sequential lymph node biopsies revealed in later stages the extension of the histiocytic proliferation from the sinuses into the cords and the complete obliteration of the nodal structures. The radiologic investigations yielded numerous pathologic findings that were consistent with the dissemination of the disease. Complete response to initial treatment was achieved in patients that were treated with radiotherapy and/or chemotherapy. Complete response with chemotherapy was achieved only when the treatment included adriamycin. The histologic and clinical features of the present series provide future evidence for the recognition of malignant histiocytosis as a distinct clinical and pathologic entity.

  3. Sinus histiocytosis with massive lymphadenopathy. Is the lymph node enlargement always massive?

    Science.gov (United States)

    Sachdev, Ritesh; Setia, Namrata; Jain, Shyama

    2007-05-01

    Sinus histiocytosis with massive lymphadenopathy (SHML) is a benign proliferating histiocytic disorder, predominantly of lymph nodes with extra-nodal involvement in some cases. It is a self-limiting disease and has a good prognosis; however some patients need steroid therapy. Therein lays a need to differentiate it from other lympho proliferative disorders of poorer prognosis. Clinically, it is suspected only when there is a significant lymph node enlargement in younger age group as the term signifies. Recently, we came across a case where a middle-aged female presented with a subtle swelling in submental region, clinically suspected of reactive or tubercular etiology. However on fine needle aspiration cytology, the smears showed many histiocytes, some of them showing lymphocytes and plasma cells in their cytoplasm, a morphological feature called as "emperipolesis" classically seen in SHML. The present case is reported because of unusual clinical presentation and possibly the need for a revision of existing terminology.

  4. The diagnostic value of {sup 18}F-FDG PET and MRI in paediatric histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Mueller, Wolfgang Peter; Melzer, Henriette Ingrid; Bartenstein, Peter; Pfluger, Thomas [Ludwig-Maximilians-University of Munich, Department of Nuclear Medicine, Munich (Germany); Schmid, Irene [Ludwig-Maximilians-University of Munich, Department of Paediatric Oncology, Munich (Germany); Coppenrath, Eva [Ludwig-Maximilians-University of Munich, Department of Radiology, Munich (Germany)

    2013-03-15

    To analyse the diagnostic value of {sup 18}F-FDG PET and MRI for the evaluation of active lesions in paediatric Langerhans cell histiocytosis. We compared 21 {sup 18}F-FDG PET scans with 21 MRI scans (mean time interval 17 days) in 15 patients (11 male, 4 female, age range 4 months to 19 years) with biopsy-proven histiocytosis. Primary criteria for the lesion-based analysis were signs of vital histiocyte infiltrates (bone marrow oedema and contrast enhancement for MRI; SUV greater than the mean SUV of the right liver lobe for PET). PET and MR images were analysed separately and side-by-side. The results were validated by biopsy or follow-up scans after more than 6 months. Of 53 lesions evaluated, 13 were confirmed by histology and 40 on follow-up investigations. The sensitivity and specificity of PET were 67 % and 76 % and of MRI were 81 % and 47 %, respectively. MRI showed seven false-positive bone lesions after successful chemotherapy. PET showed five false-negative small bone lesions, one false-negative lesion of the skull and three false-negative findings for intracerebral involvement. PET showed one false-positive lesion in the lymphoid tissue of the head and neck region and two false-positive bone lesions after treatment. Combined PET/MR analysis decreased the number of false-negative findings on primary staging, whereas no advantage over PET alone was seen in terms of false-positive or false-negative results on follow-up. Our retrospective analysis suggests a pivotal role of {sup 18}F-FDG PET in lesion follow-up due to a lower number of false-positive findings after chemotherapy. MRI showed a higher sensitivity and is indispensable for primary staging, evaluation of brain involvement and biopsy planning. Combined MRI/PET analysis improved sensitivity by decreasing the false-negative rate during primary staging indicating a future role of simultaneous whole-body PET/MRI for primary investigation of paediatric histiocytosis. (orig.)

  5. Linfoma de células en anillo de sello que simula carcinoma mucosecretante Signet ing cell lymphoma mimicking mucin-producing carcinoma

    Directory of Open Access Journals (Sweden)

    Ana María Saenz de Chirife

    2004-12-01

    Full Text Available El linfoma de células en anillo de sello es una neoplasia poco frecuente, caracterizada por la presensencia de grandes células de citoplasma vacuolado, claro, similar a los encontrados en adeno-carcinomas mucosecretantes. Se ha descripto en ganglios linfáticos y tejidos extralinfáticos. Se presenta el caso de un paciente varón de 59 años con linfoma de células en anillo de sello localizado en pared lateral de orofaringe. Esta localización creemos que no ha sido descripta anteriormente. La histología mostró células en anillo de sello y el inmunofenotipo tumoral fue: vimentina, CD45, CD20, Ig M y cadenas kappa positivos, siendo la fracción de crecimiento tumoral alta (Ki 67:70%. El paciente evolucionó desfavorablemente y falleció a los dos meses del diagnóstico sin recibir tratamiento.Signet ring cell lymphoma is a rare neoplasm caracterized by large, vacuolated and clear cells mimicking mucin-producing adenocarcinoma. It is localized in nodal and extranodal sites. A case of a 59 years old male, with a diffuse lymphoma signet ring cell type localized on oropharingeal mucosa is reported. The histopathology study showed signet ring cells and the immunophenotype was: vimentine(+, CD45(+, CD20(+, Ig M(+, Kappa chain(+ and high index proliferative activity of neoplastic cells (Ki 67:70%. After a review of the literature and previous reports, we could not find a similar case in this anatomic site. The patient had a unfavourable clinical course and died two months after the diagnosis without receiving any treatment.

  6. Necrobiosis lipoidica mimicking cellulitis

    Directory of Open Access Journals (Sweden)

    Joshi A

    1997-01-01

    Full Text Available A 57-year-old obese patient presented with a 5 month history of tender, indurated, erythematous plaques with superficial ulceration on the right shin. The lesions closely mimicked cellulitis but were unresponsive to antibiotics. Though the patient was not a known diabetic, on investigations she was found to be a diabetic. Histology confirmed the diagnosis of necrobiosis lipoidica. This acutely inflammed presentation of necrobiosis lipoidica is extremely rare.

  7. The level and influence factors of health-related quality of life in children with langerhans cell histiocytosis%朗格尔汉斯细胞组织细胞增多症患儿生存质量影响因素的调查分析

    Institute of Scientific and Technical Information of China (English)

    王旭梅; 吴心怡; 王艳红; 朱轩萍

    2012-01-01

    目的 探讨朗格尔汉斯细胞组织细胞增多症(Langerhans Cell Histiocytosis,LCH)患儿的生存质量及其影响因素.方法 采用儿童生存质量普适性核心量表(PedsQL4.0)对就诊于北京儿童医院血液中心的74例LCH患儿进行调查.结果 5~7岁患儿在生理功能、心理社会功能和总功能的得分最低.生理功能的影响因素是体重满意程度,社会功能的影响因素是体重满意程度和在京居住;学校功能的影响因素是性别,总功能的影响因素是体重满意程度.自身体重满意者在总功能领域最高(P<0.05),在生理功能领域得分高于认为自己“胖”的患儿(P<0.05),在社会功能领域得分高于认为自己“瘦”的患儿(P<0.05).结论 LCH患儿的生活质量值得关注.%Objective To investigate the level and influence factors of health-related quality of life(HRQOL) in children with langerhans cell histiocytosis (LCH). Methods Seventy-four LCH children admitted by Beijing Children's hospital were investigated with Pediatric Quality of life Inventory(PedsQIAO). Results Children aged at 5-7 years old had the lowest scores regarding physical functioning, psychosocial functioning and total functioning. The influence factor of HRQOL in physical functioning was satisfaction with body weight, the influence factor of HRQOL in emotional functioning was single child, the influence factors of HRQOL in social functioning were satisfaction with body weight and whether living in Beijing, the influence factor of HRQOL in school functioning was gender, the influence factor of HRQOL in total score was satisfaction with body weight. HRQOL in children who were satisfied with their body weight was significantly higher than that of children who thought they were too fat regarding physical functioning (P<0.05) ,and higher than that of children who thought they were two thin regarding social functioning(P<0.05) ,and higher than that of children who thought they were too

  8. Evolución clínica y radiológica de una mujer con histiocitosis pulmonar de células de Langerhans durante 18 años Clinical and radiological evolution of a woman with pulmonary Langerhans cell histiocytosis during 18 years

    Directory of Open Access Journals (Sweden)

    XIMENA CEA B

    2008-01-01

    Full Text Available La histiocitosis pulmonar de células de Langerhans (HPCL es una enfermedad pulmonar difusa quística poco frecuente. En esta revisión, presentamos un caso de HPCL diagnosticada en una adolescente, no fumadora, que consultó por disnea rápidamente progresiva asociada a dolor pleurítico izquierdo, debido a un neumotorax bilateral. El diagnóstico se confirmó por biopsia quirúrgica. Se indicó tratamiento con corticoides (prednisona durante el primer año. Tras 18 años de seguimiento destaca una importante mejoría clínica, con una evolución radiológica y espirométrica favorable, pero no completa. Los casos que se presentan con neumotorax espontáneo recurrente son raros. La evolución natural de esta enfermedad es variable y su tratamiento aún es controversial, siendo la terapia con inmunosupresores, tal como corticoesteroides y agentes citotóxicos de valor limitado, ya que son escasos los estudios que avalan su eficaciaThe Pulmonary Langerhans cell histiocytosis (PLCH is an uncommon pulmonary diffuse cystic disease. This review presents one case of PLCH diagnosed in a non-smoker female adolescent, who consulted for rapidly progressive dyspnea associated to left pleuritic pain that corresponded to a bilateral pneumothorax. The diagnosis was confirmed by surgical biopsy of the lung. Treatment with corticoids (prednisone was indicated during the first year. After 18 years from diagnosis she presented an important clinical improvement, with a favourable but not complete radiological and spirometric improvement. The cases that make their debut with recurrent spontaneous pneumothorax are infrequent. The natural evolution of this disease is variable and the treatment is still controversial, been the immunosuppressive therapy, as corticoesteroids and cytotoxic agents of limited value, since are few studies that confirm their effectiveness

  9. Mimicking the mechanical properties of the cell cortex by the self-assembly of an actin cortex in vesicles

    Science.gov (United States)

    Luo, Tianzhi; Srivastava, Vasudha; Ren, Yixin; Robinson, Douglas N.

    2014-04-01

    The composite of the actin cytoskeleton and plasma membrane plays important roles in many biological events. Here, we employed the emulsion method to synthesize artificial cells with biomimetic actin cortex in vesicles and characterized their mechanical properties. We demonstrated that the emulsion method provides the flexibility to adjust the lipid composition and protein concentrations in artificial cells to achieve the desired size distribution, internal microstructure, and mechanical properties. Moreover, comparison of the cortical elasticity measured for reconstituted artificial cells to that of real cells, including those manipulated using genetic depletion and pharmacological inhibition, strongly supports that actin cytoskeletal proteins are dominant over lipid molecules in cortical mechanics. Our study indicates that the assembly of biological systems in artificial cells with purified cellular components provides a powerful way to answer biological questions.

  10. Pediatric mast cell sarcoma of temporal bone with novel L799F (2395 C>T) KIT mutation, mimicking histiocytic neoplasm.

    Science.gov (United States)

    Kim, Young S; Wu, Huiqing; Pawlowska, Anna B; Bautista-Quach, Marnelli A; Huang, Qin; Gaal, Karl; Chang, Karen L

    2013-03-01

    Mast cell sarcoma (MCS) is an extremely rare neoplasm with a clinically aggressive course. Because of its rarity, its morphologic and molecular characteristics are still not well defined. We report a case of a 15-year-old girl with MCS of the temporal bone extending into the posterior fossa creating a mass effect. The lesion mimicked a histiocytic neoplasm morphologically, but showed a novel KIT missense mutation, L799F (2395 C>T). The KIT D816V mutation is frequently found in systemic mastocytosis, but it has not been documented in the few reported human MCS cases. However, 1 reported case of MCS has shown a different alteration in the KIT gene. Our case is the first MCS case with L799F mutation, located between the catalytic loop (790 to 797) and the activation loop (810 to 837) of the KIT gene, and only the second case of MCS with KIT mutation documented in the literature. Proximity of the L799F mutation to the enzymatic region of the KIT tyrosine kinase domain may induce resistance to tyrosine kinase inhibitors.

  11. Mimicking the tumor microenvironment to regulate macrophage phenotype and assessing chemotherapeutic efficacy in embedded cancer cell/macrophage spheroid models.

    Science.gov (United States)

    Tevis, Kristie M; Cecchi, Ryan J; Colson, Yolonda L; Grinstaff, Mark W

    2017-03-01

    Tumor associated macrophages (TAMs) are critical stromal components intimately involved with the progression, invasion, and metastasis of cancer cells. To address the need for an in vitro system that mimics the clinical observations of TAM localizations and subsequent functional performance, a cancer cell/macrophage spheroid model is described. The central component of the model is a triple negative breast cancer spheroid embedded in a three-dimensional collagen gel. Macrophages are incorporated in two different ways. The first is a heterospheroid, a spheroid containing both tumor cells and macrophages. The heterospheroid mimics the population of TAMs infiltrated into the tumor mass, thus being exposed to hypoxia and metabolic gradients. In the second model, macrophages are diffusely seeded in the collagen surrounding the spheroid, thus modeling TAMs in the cancer stroma. The inclusion of macrophages as a heterospheroid changes the metabolic profile, indicative of synergistic growth. In contrast, macrophages diffusely seeded in the collagen bear the same profile regardless of the presence of a tumor cell spheroid. The macrophages in the heterospheroid secrete EGF, a cytokine critical to tumor/macrophage co-migration, and an EGF inhibitor decreases the metabolic activity of the heterospheroid, which is not observed in the other systems. The increased secretion of IL-10 indicates that the heterospheroid macrophages follow an M2/TAM differentiation pathway. Lastly, the heterospheroid exhibits resistance to paclitaxel. In summary, the collagen embedded heterospheroid model promotes TAM-like characteristics, and will be of utility in cancer biology and drug discovery.

  12. Acute myocardial infarction mimicking squamous cell lung cancer with bone metastases due to hypercalcemia: a case report

    Institute of Scientific and Technical Information of China (English)

    FANG Chong-feng; XU Geng; CHEN Yang-xin

    2010-01-01

    @@ Acute myocardial infarction (AMI), the most severe coronary artery disease, is one of the most frequent cardiac emergencies, and early diagnosis and treatment are very important to decrease the subsequent cardiac adverse events such as malignant arrhythmia and sudden cardiac death. But in fact, lots of diseases are similar to AMI in clinical practice, of which the most common are myocarditis, pulmonary embolism in department of cardiology. Here we report a case of AMI-like squamous cell lung cancer with bone metastases.

  13. 18F-fluoro-deoxyglucose positron emission tomography/computed tomography scan findings in Rosai-Dorfman disease with IgG4-positive plasma cell infiltration mimicking breast malignancy: a case report and literature review

    Directory of Open Access Journals (Sweden)

    Fu Liping

    2012-11-01

    Full Text Available Abstract Introduction Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare benign disorder characterized histologically by lymphatic sinus dilatation due to histiocyte proliferation. Rosai-Dorfman disease accompanied by IgG4+ plasma cell infiltration is an even rarer situation. To the best of our knowledge, no imaging report of fluoro-deoxyglucose positron emission tomography/computed tomography findings of Rosai-Dorfman disease with IgG4+ plasma cell infiltration has been published, although a series of pathological research has focused on this phenomenon. Case presentation We reviewed the 18F-fluoro-deoxyglucose positron emission tomography/computed tomography scan of a 78-year-old Chinese woman with a solid mass that was found in her right breast during a health checkup. 18F-fluoro-deoxyglucose positron emission tomography/computed tomography showed a hypermetabolic nodule in her right breast and slightly heterogeneous increased fluoro-deoxyglucose uptake of the pulmonary nodules, which were histologically proven to be mammary Rosai-Dorfman disease with IgG4+ plasma cell infiltration and pulmonary amyloidosis, respectively. A literature review was performed to gather information on this rare disease process. Conclusions Although distinguishing benign lymphoplasmacytic proliferation from malignancy may be difficult with 18F-fluoro-deoxyglucose positron emission tomography/computed tomography in light of the pattern and intensity of fluoro-deoxyglucose uptake, our case highlights that whole-body positron emission tomography/computed tomography imaging not only can display the extent of the disease to help complete staging but also can provide functional information about disease activity to guide biopsy.

  14. Spontaneous regression of congenital cutaneous histiocytosis X: report of a case with discussion of nosology and pathogenesis.

    Science.gov (United States)

    Dehner, L P; Bamford, J T; McDonald, E C

    1983-01-01

    Spontaneous regression of histiocytosis X is a rare biologic event as judged by the extensive recorded experience in the literature. We present a case of congenital cutaneous histiocytosis X that resolved without specific therapy in the first week of life. Histologic and ultrastructural studies confirmed the interpretation. This child is an example of so-called pure cutaneous histiocytosis, which, like the other unisystem forms of the disorders, has an excellent prognosis.

  15. Application of whole-body bone scintigraphy in diagnosis and staging of Langerhans cell histiocytosis in infants%全身骨显像在婴幼儿朗格汉斯组织细胞增多症诊断和分期中的应用

    Institute of Scientific and Technical Information of China (English)

    丁献敏; 刘保平; 韩星敏; 程兵; 阮翘

    2011-01-01

    目的 探讨全身骨显像在婴幼儿朗格汉斯组织细胞增多症(LCH)诊断和分期中的应用价值.方法 对20例经手术病理或骨髓穿刺、皮疹压片细胞学确诊为LCH的婴幼儿行全身骨显像检查,对骨显像阳性者加行局部X线或CT检查.利用ROI技术计算全身骨显像阳性病灶的F/N比值并求出位于相同部位病灶的F/N比值均值.结果 20例患儿全身骨显像10例阳性,4例为多发病灶,6例为单发病灶.单纯颅骨放射分布异常3例,颅骨合并其他部位骨3例,肩胛骨2例,肋骨1例,下肢骨1例;单发颅骨病灶的F/N比值均值为0.36,长骨病灶的F/N比值均值为3.52.结论 全身骨显像有助于婴幼儿LCH的诊断及分期.%Objective To assess the application value of whole-body bone scintigraphy in diagnosis and staging of Langerhans cell histiocytosis (LCH) in infants. Methods Whole-body bone scintigraphy was performed in 20 infants of LCH confirmed with surgical pathology or bone marrow aspiration and laboratory data. The infants with positive bone scintigraphy underwent X-ray or CT, and the F/N value of positive lesions and lesions located in the same area were calculated by ROI technology. Results Ten of 20 patients showed positive in bone scans, including 4 with multi-focal lesions and 6 with single lesion. Among 10 patients with positive scintigrams, there were 3 patients with abnormal bone uptakes limited to cranial, 3 with cranial and other parts of abnormal bone uptakes, 2 with scapula abnormal uptakes, as well as rib and lower limb bone involvement (each n= 1). The average ratios of F/N on cranial alone was 0. 36, while focused on long bones was 3. 52. Conclusion Whole-body bone scintigraphy is useful in the diagnosis and staging of LCH in infants.

  16. 朗格汉斯细胞组织细胞增生症中TP53蛋白表达及基因突变检测%Detection TP53 Protein Expression and Gene Mutation in Langerhans' Cell Histiocytosis

    Institute of Scientific and Technical Information of China (English)

    孙忠辉; 石梅; 李明; 余红

    2013-01-01

    Objective To determine whether TP53 gene plays a role in the pathogenesis of Langerhans' Cell Histiocytosis (LCH) ,we studied the expression of TP53 protein and mutation of TP53 gene in LCH biopsies. Methods The expression of TP53 protein was detected by immunohistochemical SP method in 38 cases of Langerhans cell histiocytosis. The mutation in TP53 gene exon 2 to 11 was identified by polymerase chain reaction and subsequently conformed by DNA sequencing in the 16 TP53 protein positive biopsies. Results TP53 protein expression was detected in 16/38(42. 11% ) LCH biopsies. In those biopsies, there were no significant difference between the average optical density of multisystem (MS,0. 120 6 ±0.008 8) and single-system (SS,0. 122 1 ±0.0030) respectively(P>0.05). No mutations in the TP53 gene exon 2 to 11 were detected in those positive biopsies. For genetic genotype of the polymorphism of TP53 gene codon 72, there were PRO in 3 cases,ARG in 5 cases, PRO/ARG in 8 cases in LCH group (TP53 protein positive). Compared with 40 normal controls,Chi-square analysis showed there was no significant difference for distribution of the three genotypes( P > 0. 05 ). Then, for the clinical classification, the cases of PRO, ARG and PRO/ ARG were 2,3 ,5 in MS and 1 ,2,3 in SS,respectively. Chi-square analysis also showed no significant differences (P>0. 05). Conclusion The TP53 protein is abnormally expressed in LCH,although there was no significant difference with the clinical classification. There is no TP53 gene mutation in LCH. The TP53 codon 72 genotype distribution was consistent with the normal population and not related with clinical classification. We speculate that the abnormality of TP53 gene expression in LCH may not result from gene mutation but an unknown mechanism.%目的 检测朗格汉斯细胞组织细胞增生症(LCH)中TP53蛋白表达和TP53基因全外显子突变,以探讨TP53在LCH发生发展中的作用机制.方法 应用免疫纽化SP法检测38

  17. Eosinophilic granuloma in the anterior mandible mimicking radicular cyst

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Byung Do; Lee, Wan; Lee, Jun [College of Dentistry, Wonkwang University, Iksan (Korea, Republic of); Son, Hyun Jin [Dept. of Pathology, School of Medicine, Eulji University, Daejeon (Korea, Republic of)

    2013-06-15

    Eosinophilic granuloma is a common expression of Langerhans cell histiocytosis and corresponds with typical bone lesions. The radiographic appearance of eosinophilic granuloma in the jaw is variable and not specific. It may resemble periodontitis, radicular cyst, or malignancies. The purpose of this report is to describe the characteristic radiographic features of eosinophilic granuloma of a 39-year-old male. The lesion in the anterior mandible was first diagnosed as radicular cyst because the radiographic findings were ovoid radiolucent lesion with well-defined border. However, careful interpretation revealed a non-corticated border and floating tooth appearance that were the characteristic radiographic features for the differential diagnosis. Early clinical signs of eosinophilic granuloma can occur in the jaw and a bony destructive lesion might be mistaken for periodontitis or an odontogenic cystic lesion; therefore, careful interpretation of radiographs should be emphasized.

  18. CT imaging findings of thymus or thyroid gland involved in Langerhans cell histiocytosis of pediatric patients%儿童朗格汉斯细胞组织细胞增生症侵及胸腺和甲状腺的CT影像表现

    Institute of Scientific and Technical Information of China (English)

    胡迪; 段晓岷; 曹琪; 彭芸

    2016-01-01

    Objective To investigate the CT imaging findings of thymus and thyroid gland involved in Langerhans cell histiocytosis(LCH) of pediatric patients. Methods CT image findings in pediatric patients with LCH confirmed by pathology and clinical diagnosis from January 2006 to February 2015 were retrospectively analyzed. By radiography, 38 cases' thymus gland were involved and 8 cases' thyroids gland were involved. Results Plain CT scan for thymus gland involved patients revealed 9 cases were shown as hyperplasia, 17 cases were shown as calcification, 9 cases were shown as hyperplasia with calcification, 1 case was shown as cavity, 1 case was shown as hyperplasia with calcification and cavity and 1 case was shown as hyperplasia with calcification and low density. Enhanced CT scan for thyroid gland involved patients showed bilateral or unilateral enlargement of thyroid gland with heterogeneous low density and unobvious enhancement. Conclusions Finely calcifications were the most common CT findings on thymus gland involved in LCH and might without hyperplasia. CT imaging of thyroid LCH exhibited an enlarged or normal size and abnormal density in the thyroid gland with adjacent soft tissues involved.%目的:探讨分析儿童朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)累及胸腺、甲状腺的CT影像表现。方法回顾分析我院2006年1月至2015年2月间经病理及临床确诊LCH累及胸腺和甲状腺患儿治疗前CT影像表现特点。其中累及胸腺者共38例,累及甲状腺者共8例。结果累及胸腺者CT平扫表现为9例单纯肿大,17例不同程度钙化,9例肿大伴不同程度钙化,1例胸腺内可见气腔影,1例肿大伴钙化及气腔,1例肿大伴钙化及低密度灶。累及甲状腺者CT平扫及增强检查表现为双侧甲状腺对称或不对称性肿大,密度不均匀减低,增强病变累及区强化不明显。结论 LCH累及胸腺CT表现以钙化常见,可不伴有胸腺

  19. [Pelvic lymphadenitis after total hip arthroplasty : Mimicking of lymph node metastases in a patient with prostate cancer].

    Science.gov (United States)

    Sevinc, S; Westhoff, C C; Schrader, A J; Olbert, P J; Hofmann, R; Hegele, A

    2010-08-01

    This report describes the case of a 65-year-old patient who underwent radical prostatectomy in our department. Intraoperatively we detected suspicious lymph nodes on the left side. The histopathological examination revealed histiocytosis and foreign body giant cells but no sign of tumor. The enlarged lymph nodes were ascribed to an ipsilateral total hip arthroplasty performed 14 years previously because of progressive coxarthrosis. Lymphadenitis after total hip arthroplasty is frequently observed. Histopathologically and with the use of polarized light microscopy, histiocytosis and wear particles such as titanium, polyethylene, and polyethylene-methylacrylate may be detected. When operating on patients with arthroplasty of a lower limb, particularly those with a total endoprosthesis, the surgeon should bear in mind that changes in lymph node consistency and size do not necessarily indicate tumor involvement or metastases.

  20. The study of BRAF V600E and MAP2K1 gene mutations in Langerhans cell histiocytosis and its significance of clinical findings%朗格汉斯细胞组织细胞增生症中 BRAF V600E 和 MAP2K1基因突变的分析及其临床意义

    Institute of Scientific and Technical Information of China (English)

    曾凯旋; 赵丹珲; 王哲; 闫庆国; 郭双平; 郭英; 刘一雄; 张微晨; 张月华; 李明阳; 李侠; 魏洁; 吕杨

    2016-01-01

    目的:探讨我国朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)中 BRAF V600E 和MAP2K1基因突变发生状况及其临床意义。方法:随机选取35例 LCH 组织标本,采用桑格测序法检测其中BRAF V600E 和 MAP2K1基因突变状况,免疫组化法检测 BRAF V600E 蛋白的表达。分析 BRAF V600E、MAP2K1基因突变与 LCH 临床基本资料(年龄、性别、单/多系统)的关系。结果:在35例 LCH 患者中,男女比例为1.7∶1,82.9%侵及骨组织,97.1%是单系统 LCH(single system LCH,SS -LCH),2.9%是多系统 LCH (multi -system LCH,MS -LCH)。桑格测序法检测 BRAF V600E 基因突变率为17.1%,MAP2K1基因突变率为14.3%,MAP2K1与 BRAF V600E 基因突变有互异性;免疫组化法检测 BRAF V600E 阳性表达率为28.6%,涵盖了桑格测序法测得的突变病例。BRAF V600E 和 MAP2 K1基因突变更多出现在未成年组(35.7%和28.6%),其中 BRAF V600E 突变在未成年人组与成人组间有显著性差异(P =0.028);BRAF V600E 和MAP2K1基因突变对生存的影响无统计学差异(P >0.05)。结论:我国 LCH 患者大部分都是 SS -LCH,主要侵及的部位是骨组织,且预后良好,5年生存率为97.1%。桑格法所测的 BRAF V600E 和 MAP2K1基因突变率均低于西方报道,两者存在互异性,分别为17.1%和14.3%。所有 MAP2K1基因突变都是点突变,没有框内缺失突变,发现一个新的突变位点:c.112 G >A p.E38K;BRAF V600E 和 MAP2K1基因突变主要发生于未成年组中,提示各年龄层中 LCH 的发病机理可能不同,可能 RAS /RAF /MEK/ERK 通路在未成年人 LCH 中发挥更重要的作用;另外这两种突变对 LCH 的生存无影响。%Objective:To study BRAF V600E and MAP2K1 gene mutations in Langerhans cell histiocytosis and analyze the relationship between the

  1. Immunoglobulin G4-Related Disease Mimicking Asthma

    Directory of Open Access Journals (Sweden)

    Hiroshi Sekiguchi

    2013-01-01

    Full Text Available Immunoglobulin (Ig G4-related disease (also known as ‘IgG4-related sclerosing disease’, ‘IgG4-related systemic disease’ or ‘hyper-IgG4-disease’ is a recently recognized systemic fibroinflammatory disease associated with IgG4-positive plasma cells in tissue lesions. IgG4-related disease was initially described as autoimmune pancreatitis, but it is now known to affect virtually any organ. The authors describe a patient presenting with multi-organ manifestations, including airway inflammation mimicking asthma, pulmonary parenchymal infiltrates, intrathoracic lymphadenopathy, submandibular gland swelling and a kidney mass.

  2. Pseudocarcinomatous hyperplasia in anaplastic large cell lymphoma, a mimicker of poorly differentiated squamous cell carcinoma: report of a case and review of the literature.

    Science.gov (United States)

    Price, Alexandra; Miller, Jason H; Junkins-Hopkins, Jacqueline M

    2015-11-01

    Pseudocarcinomatous hyperplasia can occasionally be observed in biopsies of CD30-positive lymphoproliferative disorders. It is important to be cognizant of this association, because epithelial hyperproliferation can overshadow large atypical lymphoid cells, leading to an erroneous diagnosis of squamous cell carcinoma (SCC) or keratoacanthoma. Herein, we present a case of anaplastic large cell lymphoma (ALCL) with pseudocarcinomatous hyperplasia simulating a poorly differentiated carcinoma and review the literature on this subject. Immunohistochemical staining with p63 helped delineate the infiltrating tongues of pseudocarcinomatous hyperplasia from the malignant infiltrate. We present this case to raise awareness of the potential for pseudocarcinomatous hyperplasia to occur in the setting of CD30+ lymphoproliferative disorders. Clinicians and dermatopathologists should consider the possibility of ALCL or lymphomatoid papulosis when examining lesions with features of inflamed SCC, especially if the tumor presents on a site or in a patient that is not typical of SCC.

  3. Synthetic RNAs Mimicking Structural Domains in the Foot-and-Mouth Disease Virus Genome Elicit a Broad Innate Immune Response in Porcine Cells Triggered by RIG-I and TLR Activation.

    Science.gov (United States)

    Borrego, Belén; Rodríguez-Pulido, Miguel; Revilla, Concepción; Álvarez, Belén; Sobrino, Francisco; Domínguez, Javier; Sáiz, Margarita

    2015-07-17

    The innate immune system is the first line of defense against viral infections. Exploiting innate responses for antiviral, therapeutic and vaccine adjuvation strategies is being extensively explored. We have previously described, the ability of small in vitro RNA transcripts, mimicking the sequence and structure of different domains in the non-coding regions of the foot-and-mouth disease virus (FMDV) genome (ncRNAs), to trigger a potent and rapid innate immune response. These synthetic non-infectious molecules have proved to have a broad-range antiviral activity and to enhance the immunogenicity of an FMD inactivated vaccine in mice. Here, we have studied the involvement of pattern-recognition receptors (PRRs) in the ncRNA-induced innate response and analyzed the antiviral and cytokine profiles elicited in swine cultured cells, as well as peripheral blood mononuclear cells (PBMCs).

  4. Synthetic RNAs Mimicking Structural Domains in the Foot-and-Mouth Disease Virus Genome Elicit a Broad Innate Immune Response in Porcine Cells Triggered by RIG-I and TLR Activation

    Directory of Open Access Journals (Sweden)

    Belén Borrego

    2015-07-01

    Full Text Available The innate immune system is the first line of defense against viral infections. Exploiting innate responses for antiviral, therapeutic and vaccine adjuvation strategies is being extensively explored. We have previously described, the ability of small in vitro RNA transcripts, mimicking the sequence and structure of different domains in the non-coding regions of the foot-and-mouth disease virus (FMDV genome (ncRNAs, to trigger a potent and rapid innate immune response. These synthetic non-infectious molecules have proved to have a broad-range antiviral activity and to enhance the immunogenicity of an FMD inactivated vaccine in mice. Here, we have studied the involvement of pattern-recognition receptors (PRRs in the ncRNA-induced innate response and analyzed the antiviral and cytokine profiles elicited in swine cultured cells, as well as peripheral blood mononuclear cells (PBMCs.

  5. Synthetic RNAs Mimicking Structural Domains in the Foot-and-Mouth Disease Virus Genome Elicit a Broad Innate Immune Response in Porcine Cells Triggered by RIG-I and TLR Activation

    Science.gov (United States)

    Borrego, Belén; Rodríguez-Pulido, Miguel; Revilla, Concepción; Álvarez, Belén; Sobrino, Francisco; Domínguez, Javier; Sáiz, Margarita

    2015-01-01

    The innate immune system is the first line of defense against viral infections. Exploiting innate responses for antiviral, therapeutic and vaccine adjuvation strategies is being extensively explored. We have previously described, the ability of small in vitro RNA transcripts, mimicking the sequence and structure of different domains in the non-coding regions of the foot-and-mouth disease virus (FMDV) genome (ncRNAs), to trigger a potent and rapid innate immune response. These synthetic non-infectious molecules have proved to have a broad-range antiviral activity and to enhance the immunogenicity of an FMD inactivated vaccine in mice. Here, we have studied the involvement of pattern-recognition receptors (PRRs) in the ncRNA-induced innate response and analyzed the antiviral and cytokine profiles elicited in swine cultured cells, as well as peripheral blood mononuclear cells (PBMCs). PMID:26193305

  6. Xanthogranulomatous cholecystitis mimicking gallbladder cancer.

    Science.gov (United States)

    Ewelukwa, Ofor; Ali, Omair; Akram, Salma

    2014-05-08

    Xanthogranulomatous cholecystitis (XGC) is a benign, uncommon variant of chronic cholecystitis characterised by focal or diffuse destructive inflammatory process of the gallbladder (GB). Macroscopically, it appears like yellowish tumour-like masses in the wall of the GB. This article reports on a 74-year-old woman with XGC mimicking GB cancer.

  7. Clinico-pathologic conference: case 4. Langerhans cell histiocytosis (LCH).

    Science.gov (United States)

    Muramatsu, Takashi; Hall, Gillian L; Hashimoto, Sadamitsu; Miyauchi, Jun; Shimono, Masaki

    2010-12-01

    A 13-month-old Japanese boy presented with painless swelling in a left mandible and cheek. Intraoral examination revealed swelling in the left mandible and hemorrhage of oral mucosa due to biting. CT images revealed a wide osteolytic lesion of the left mandible with floating teeth. Biopsy was carried out and histopathological diagnosis was discussed.

  8. Percutaneous vertebroplasty in osteoporosis, myeloma and Langerhans' cell histiocytosis.

    LENUS (Irish Health Repository)

    Kevane, B

    2012-01-09

    This review aims to assess the effectiveness of percutaneous vertebroplasty as a treatment for the severe refractory pain associated with vertebral fracture, in a group of patients with fractures secondary to either osteoporotic or neoplastic disease. A retrospective review of 20 patients treated with percutaneous vertebroplasty in Cork University Hospital up until March 2007 was carried out and a questionnaire was prepared and distributed. Prior to vertebroplasty, patients had been symptomatic with severe pain for a mean of 20.9 weeks. Of those thirteen whom replied to a postal questionnaire, 12 (92.3%) reported pain relief and this improvement occurred within 7 days in 9 (81.8%). This was associated with decreased analgesic requirements, as determined on chart review. Prior to the procedure only 5 (38.4%) were independently mobile and this figure rose to 10 (76.9%) afterwards, occurring within one week in the majority. Subjective outcomes were better in the group of patients with neoplasm-induced fractures.

  9. Multicystic Hepatocarcinoma Mimicking Liver Abscess

    Directory of Open Access Journals (Sweden)

    Evangelos Falidas

    2013-01-01

    Full Text Available The diagnosis of hepatocellular carcinoma (HCC became easier in relation to the improved radiological examinations; however, the neoplasm may occur under atypical presentations mimicking other benign or malignant processes. Multicystic HCC mimicking a liver abscess associated with septic-type fever and leukocytosis is rare, has a poor prognosis, and poses diagnostic and therapeutic dilemmas. We present the case of an 80-year-old patient, who presented with fever, leukocytosis, and large cystic masses involving right and left lobes of the liver initially considered abscesses and finally diagnosed as HCC after open drainage and liver biopsy. Although the patient died on the tenth postoperative day due to pulmonary oedema, the authors emphasize the high index of suspicion needed in the diagnosis of this unusual presentation of HCC.

  10. Mimicking Seawater For Culturing Marine Bacteria

    DEFF Research Database (Denmark)

    Rygaard, Anita Mac; Sonnenschein, Eva; Gram, Lone

    2015-01-01

    Only about 1% of marine bacteria have been brought into culture using traditional techniques. The purpose of this study was to investigate if mimicking the natural bacterial environment can increase culturability.We used marine substrates containing defined algal polymers or gellan gum as solidif......Only about 1% of marine bacteria have been brought into culture using traditional techniques. The purpose of this study was to investigate if mimicking the natural bacterial environment can increase culturability.We used marine substrates containing defined algal polymers or gellan gum...... as solidifying agents, and enumerated bacteria from seawater and algal exudates. We tested if culturability could be influenced by addition of quorum sensing signals (AHLs). All plates were incubated at 15°C. Bacterial counts (CFU/g) from algal exudates from brown algae were highest on media containing algal...... polymers. In general, bacteria isolated from algal exudates preferred more rich media than bacteria isolated from seawater. Overall, culturability ranged from 0.01 to 0.8% as compared to total cell count. Substitution of agar with gellan gum increased the culturability of seawater bacteria approximately...

  11. Orbital Lymphoma Mimicking Lacrimal Gland Pleomorphic Adenoma

    Directory of Open Access Journals (Sweden)

    Diego Strianese

    2013-09-01

    Full Text Available Purpose: To describe the case of a patient affected by orbital lymphoma mimicking pleomorphic adenoma of the lacrimal gland. Methods: This was a retrospective case report. Results: We present the case of a patient with 15-year history of slowly progressive left proptosis and inferomedial bulbar dislocation who had the presumptive diagnosis of lacrimal gland pleomorphic adenoma based on clinical and radiological features. The patient underwent lateral orbitotomy and lacrimal gland excision. Postoperative histological features were consistent with low-grade B-cell non-Hodgkin lymphoma. Conclusion: The accepted clinico-radiological criteria used for the diagnosis of lacrimal gland fossa lesions might have a certain false-positive rate, even in recent years. The initial surgical approach with the appropriate choice between fine-needle aspiration biopsies, intraoperative biopsies and lacrimal gland excisions might be a challenge.

  12. Decidualized Ovarian Mass Mimicking Malignancy

    Directory of Open Access Journals (Sweden)

    Lufee Wong

    2015-01-01

    Full Text Available Deciduosis classically occurs in the context of known endometriosis in the pelvis, most commonly in the ovaries, but also in the peritoneum. However, ovarian deciduosis outside the context of endometriosis is rare and makes diagnosis difficult, especially as the sonographic appearance suggests a malignant process. We report a case of decidualized ovarian mass in a patient without prior history of endometriosis that mimicked an ovarian malignancy. MRI may be a useful imaging modality to monitor these lesions and guide management. Consultation with a multidisciplinary team accustomed to such conditions will help to tailor the management to each individual.

  13. Tissue-Mimicking Geometrical Constraints Stimulate Tissue-Like Constitution and Activity of Mouse Neonatal and Human-Induced Pluripotent Stem Cell-Derived Cardiac Myocytes

    Directory of Open Access Journals (Sweden)

    Götz Pilarczyk

    2016-01-01

    Full Text Available The present work addresses the question of to what extent a geometrical support acts as a physiological determining template in the setup of artificial cardiac tissue. Surface patterns with alternating concave to convex transitions of cell size dimensions were used to organize and orientate human-induced pluripotent stem cell (hIPSC-derived cardiac myocytes and mouse neonatal cardiac myocytes. The shape of the cells, as well as the organization of the contractile apparatus recapitulates the anisotropic line pattern geometry being derived from tissue geometry motives. The intracellular organization of the contractile apparatus and the cell coupling via gap junctions of cell assemblies growing in a random or organized pattern were examined. Cell spatial and temporal coordinated excitation and contraction has been compared on plain and patterned substrates. While the α-actinin cytoskeletal organization is comparable to terminally-developed native ventricular tissue, connexin-43 expression does not recapitulate gap junction distribution of heart muscle tissue. However, coordinated contractions could be observed. The results of tissue-like cell ensemble organization open new insights into geometry-dependent cell organization, the cultivation of artificial heart tissue from stem cells and the anisotropy-dependent activity of therapeutic compounds.

  14. Xp11 translocation renal cell carcinoma morphologically mimicking clear cell-papillary renal cell carcinoma in an adult patient: report of a case expanding the morphologic spectrum of Xp11 translocation renal cell carcinomas.

    Science.gov (United States)

    Parihar, Asmita; Tickoo, Satish K; Kumar, Sunil; Arora, Vinod Kumar

    2015-05-01

    Xp11 translocation renal cell carcinoma (RCC) is a relatively rare tumor mainly affecting children and adolescents. It shows significant morphological overlap with the 2 most common adult renal tumors, which are the clear cell (conventional) RCC and papillary RCC. We describe case of a young adult female who presented with right flank pain and abdominal mass. Radiological investigations showed features suggestive of renal cell carcinoma in the right kidney. Histopathological findings while suggestive of Xp11 carcinoma, showed significant overlap with the recently described entity clear cell papillary RCC. TFE3 immunohistochemistry confirmed the tumor to be Xp11 translocation RCC. The patient had an aggressive course with lymph node metastasis. In this report, we discuss differential diagnosis and the diagnostic challenges of Xp11 translocation RCC in adults.

  15. Gout: radiographic findings mimicking infection

    Energy Technology Data Exchange (ETDEWEB)

    Rousseau, I.; Raymond-Tremblay, D. [Dept. of Diagnostic Radiology, Centre Hospitalier de l' Univ. de Montreal, Que. (Canada); Cardinal, E. [Dept. of Radiology, Centre Hospitalier de l' Univ. de Montreal, Que. (Canada); Beauregard, C.G. [Dept. of Diagnostic Radiology, Hopital du Sacre-Coeur de Montreal,Que. (Canada); Braunstein, E.M. [Dept. of Radiology, Indiana University Hospital (United States); Saint-Pierre, A. [Rheumatology Unit, Centre Hospitalier de l' Univ. de Montreal, Que. (Canada)

    2001-10-01

    Objective: To describe radiographic features of gout that may mimic infection. Design and patients: We report five patients with acute bacterial gout who presented with clinical as well as radiological findings mimicking acute bacterial septic arthritis or osteomyelitis. Three patients had delay in the appropriate treatment with the final diagnosis being established after needle aspiration and identification of urate crystals under polarized light microscopy. Two patients underwent digit amputation for not responding to antibiotic treatment and had histological findings confirming the diagnosis of gout. Conclusion: It is important for the radiologist to be aware of the radiological manifestations of acute gout that can resemble infection in order to avoid inappropriate diagnosis and delay in adequate treatment. The definitive diagnosis should rely on needle aspiration and a specific search for urate crystals. (orig.)

  16. Nucleus-staining with biomolecule-mimicking nitrogen-doped carbon dots prepared by a fast neutralization heat strategy.

    Science.gov (United States)

    Kang, Yan-Fei; Fang, Yang-Wu; Li, Yu-Hao; Li, Wen; Yin, Xue-Bo

    2015-12-11

    Biomolecule-mimicking nitrogen-doped carbon dots (N-Cdots) were synthesized from dopamine by a neutralization heat strategy. Fluorescence imaging of various cells validated their nucleus-staining efficiency. The dopamine-mimicking N-Cdots "trick" nuclear membranes to achieve nuclear localization and imaging.

  17. Malignant granular cell tumor of the abdominal wall mimicking desmoid tumor: A case report with CT imaging findings and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Je Hong; Ahn, Sung Eun; Lee, Dong Ho; Park, Seong Jin; Moon, Sung Kyoung; Lim, Joo Won [Dept. Radiology, Kyung Hee University Hospital, Kyung Hee University School of Medicine, Seoul (Korea, Republic of)

    2016-08-15

    Granular cell tumors (GCTs) are extremely rare mesenchymal neoplasms of Schwann cell origin. Malignant GCTs (MGCTs) comprise 0.5-2% of all GCTs. In the present report, we describe a case of a 66-year-old man with MGCT of the abdominal wall. The patient visited our hospital due to a recently growing palpable soft tissue mass in the abdominal wall. Computed tomography scan revealed a 4.3 × 4.1 × 2.9 cm sized mass arising from the left abdominal wall, which was contemplated as a desmoid tumor before surgical excision. Histopathological examination confirmed MGCT.

  18. Adhesion of Pancreatic Cancer Cells in a Liver-Microvasculature Mimicking Coculture Correlates with Their Propensity to Form Liver-Specific Metastasis In Vivo

    Directory of Open Access Journals (Sweden)

    Mohammad Mahfuz Chowdhury

    2014-01-01

    Full Text Available Organ-specific characteristic of endothelial cells (ECs is crucial for specific adhesion of cancer cells to ECs, which is a key factor in the formation of organ-specific metastasis. In this study, we developed a coculture of TMNK-1 (immortalized liver sinusoidal ECs with 10T1/2 (resembling hepatic stellate cells to augment organ-specific characteristic of TMNK-1 and investigated adhesion of two pancreatic cancer cells (MIA-PaCa-2 and BxPC-3 in the culture. MIA-PaCa-2 and BxPC-3 adhesion in TMNK-1+10T1/2coating culture (TMNK-1 monolayer over 10T1/2 layer on collagen coated surface were similar. However, in TMNK-1+10T1/2gel (coculture on collagen gel surface, MIA-PaCa-2 adhesion was significantly higher than BxPC-3, which was congruent with the reported higher propensity of MIA-PaCa-2 than BxPC-3 to form liver metastasis in vivo. Notably, as compared to BxPC-3, MIA-PaCa-2 adhesion was lower and similar in TMNK-1 only culture on the collagen coated and gel surfaces, respectively. Investigation of the adhesion in the representative human umbilical vein ECs (HUVECs cultures and upon blocking of surface molecules of ECs revealed that MIA-PaCa-2 adhesion was strongly dependent on the organ-specific upregulated characteristics of TMNK-1 in TMNK-1+10T1/2gel culture. Therefore, the developed coculture would be a potential assay for screening novel drugs to inhibit the liver-microvasculature specific adhesion of cancer cells.

  19. Adhesion of pancreatic cancer cells in a liver-microvasculature mimicking coculture correlates with their propensity to form liver-specific metastasis in vivo.

    Science.gov (United States)

    Chowdhury, Mohammad Mahfuz; Danoy, Mathieu; Rahman, Farhana; Shinohara, Marie; Kaneda, Shohei; Shiba, Kiyotaka; Fujita, Naoya; Fujii, Teruo; Sakai, Yasuyuki

    2014-01-01

    Organ-specific characteristic of endothelial cells (ECs) is crucial for specific adhesion of cancer cells to ECs, which is a key factor in the formation of organ-specific metastasis. In this study, we developed a coculture of TMNK-1 (immortalized liver sinusoidal ECs) with 10T1/2 (resembling hepatic stellate cells) to augment organ-specific characteristic of TMNK-1 and investigated adhesion of two pancreatic cancer cells (MIA-PaCa-2 and BxPC-3) in the culture. MIA-PaCa-2 and BxPC-3 adhesion in TMNK-1+10T1/ 2|coating culture (TMNK-1 monolayer over 10T1/2 layer on collagen coated surface) were similar. However, in TMNK-1+10T1/ 2|gel (coculture on collagen gel surface), MIA-PaCa-2 adhesion was significantly higher than BxPC-3, which was congruent with the reported higher propensity of MIA-PaCa-2 than BxPC-3 to form liver metastasis in vivo. Notably, as compared to BxPC-3, MIA-PaCa-2 adhesion was lower and similar in TMNK-1 only culture on the collagen coated and gel surfaces, respectively. Investigation of the adhesion in the representative human umbilical vein ECs (HUVECs) cultures and upon blocking of surface molecules of ECs revealed that MIA-PaCa-2 adhesion was strongly dependent on the organ-specific upregulated characteristics of TMNK-1 in TMNK-1+10T1/ 2|gel culture. Therefore, the developed coculture would be a potential assay for screening novel drugs to inhibit the liver-microvasculature specific adhesion of cancer cells.

  20. Delivery of human mesenchymal adipose-derived stem cells restores multiple urological dysfunctions in a rat model mimicking radical prostatectomy damages through tissue-specific paracrine mechanisms.

    Science.gov (United States)

    Yiou, René; Mahrouf-Yorgov, Meriem; Trébeau, Céline; Zanaty, Marc; Lecointe, Cécile; Souktani, Richard; Zadigue, Patricia; Figeac, Florence; Rodriguez, Anne-Marie

    2016-02-01

    Urinary incontinence (UI) and erectile dysfunction (ED) are the most common functional urological disorders and the main sequels of radical prostatectomy (RP) for prostate cancer. Mesenchymal stem cell (MSC) therapy holds promise for repairing tissue damage due to RP. Because animal studies accurately replicating post-RP clinical UI and ED are lacking, little is known about the mechanisms underlying the urological benefits of MSC in this setting. To determine whether and by which mechanisms MSC can repair damages to both striated urethral sphincter (SUS) and penis in the same animal, we delivered human multipotent adipose stem cells, used as MSC model, in an immunocompetent rat model replicating post-RP UI and ED. In this model, we demonstrated by using noninvasive methods in the same animal from day 7 to day 90 post-RP injury that MSC administration into both the SUS and the penis significantly improved urinary continence and erectile function. The regenerative effects of MSC therapy were not due to transdifferentiation and robust engraftment at injection sites. Rather, our results suggest that MSC benefits in both target organs may involve a paracrine process with not only soluble factor release by the MSC but also activation of the recipient's secretome. These two effects of MSC varied across target tissues and damaged-cell types. In conclusion, our work provides new insights into the regenerative properties of MSC and supports the ability of MSC from a single source to repair multiple types of damage, such as those seen after RP, in the same individual.

  1. Delivery of human mesenchymal adipose-derived stem cells restores multiple urological dysfunctions in a rat model mimicking radical prostatectomy damages through tissue-specific paracrine mechanisms

    DEFF Research Database (Denmark)

    Yiou, René; Mahrouf-Yorgov, Meriem; Trébeau, Céline;

    2016-01-01

    Urinary incontinence (UI) and erectile dysfunction (ED) are the most common functional urological disorders and the main sequels of radical prostatectomy (RP) for prostate cancer. Mesenchymal stem cell (MSC) therapy holds promise for repairing tissue damage due to RP. Because animal studies...... accurately replicating post-RP clinical UI and ED are lacking, little is known about the mechanisms underlying the urological benefits of MSC in this setting. To determine whether and by which mechanisms MSC can repair damages to both striated urethral sphincter (SUS) and penis in the same animal, we...

  2. Humanlike Robots - Synthetically Mimicking Humans

    Science.gov (United States)

    Bar-Cohen, Yoseph

    2012-01-01

    Nature inspired many inventions and the field of technology that is based on the mimicking or inspiration of nature is widely known as Biomimetics and it is increasingly leading to many new capabilities. There are numerous examples of biomimetic successes including the copying of fins for swimming, and the inspiration of the insects and birds flight. More and more commercial implementations of biomimetics are appearing and behaving lifelike and applications are emerging that are important to our daily life. Making humanlike robots is the ultimate challenge to biomimetics and, for many years, it was considered science fiction, but such robots are becoming an engineering reality. Advances in producing such robot are allowing them to perform impressive functions and tasks. The development of such robots involves addressing many challenges and is raising concerns that are related to fear of their application implications and potential ethical issues. In this paper, the state-of-the-art of humanlike robots, potential applications and challenges will be reviewed.

  3. McCune-Albright syndrome mimicking malignancy: an endocrine disease from oncologist's perspective.

    Science.gov (United States)

    Genç, D Bahar; Özkan, M Alp; Büyükgebiz, Atilla

    2012-09-01

    Fibrous dysplasia (FD) is categorized as either monostotic or polyostotic and may occur as a component of McCune-Albright syndrome (MAS). Imaging findings can mimic neoplastic diseases. We present a case of MAS initially suspected to have neoplastic disease. A 9-year-old girl was admitted to pediatric emergency with ataxia. Upon hospitalization, an extradural mass was seen on cranial magnetic resonance imaging (MRI) and the bone survey showed lytic lesions in the long bones. The patient was referred to the pediatric oncology department with a presumptive diagnosis of Langerhans cell histiocytosis or metastatic tumor. Further investigations demonstrated that the patient had MAS and coexisting postinfectious cerebellitis. The findings in this patient demonstrate that the radiographic findings and the clinical presentation of FD and MAS may be similar to those of malignant diseases.

  4. Favourable prognostic features in histiocytosis X: bone involvement and absence of skin disease.

    OpenAIRE

    Broadbent, V

    1986-01-01

    The records of 70 patients presenting to this hospital since 1961 with histiocytosis X confirmed by biopsy examination have been reviewed. The patients were subdivided into three groups: group A, those under 2 years of age at diagnosis; group B, those between 2 and 5 years; and group C, those over 5 years. All eight patients who died (11% overall mortality) were under 2 years of age at diagnosis. Involvement of lung, liver, and bone marrow were confirmed as poor prognostic features. The prese...

  5. Renal Cell Carcinoma Mimicking Adrenal Tumor

    Directory of Open Access Journals (Sweden)

    Mohammad Kazem Moslemi

    2010-01-01

    Full Text Available There are a variety of causes of adrenal pseudotumors on computerized tomography (CT scan, including upper-pole renal mass, gastric diverticulum, prominent splenic lobulation, pancreatic mass, hepatic mass, and periadrenal varices. We present a case of a large subhepatic mass that discrimination of its origin from neighborhood organs was difficult preoperatively. Our patient was a 58 years old man, that three months after an unsuccessful operation in another center for a pseudoadrenal mass underwent a very difficult subcapsular tumorectomy in our center.

  6. Klebsiella pneumoniae pharyngitis mimicking malignancy: a diagnostic dilemma.

    Science.gov (United States)

    Yeh, C-F; Li, W-Y; Hsu, Y-B

    2014-12-01

    Acute pharyngitis is a common disease. However, acute pharyngitis caused by Klebsiella pneumoniae with a gross appearance mimicking hypopharyngeal malignancy has never previously been reported. We report the case of a 57-year-old man with a right hypopharyngeal tumor which was disclosed by fiberoptic laryngoscopy and computed tomography scan. However, both the frozen and final pathologies showed no evidence of malignant cells, and a bacterial culture revealed the growth of K. pneumoniae. The hypopharyngeal lesion completely regressed after 2 weeks of antibiotic treatment. Clinicians should perform biopsy along with tissue culture for tumor-like lesions because infectious agents can lead to lesions with malignancy-like appearance.

  7. Recurrent epiploic appendagitis mimicking appendicitis and cholecystitis

    Science.gov (United States)

    Hearne, Christopher B.; Taboada, Jorge

    2017-01-01

    Epiploic appendagitis (EA) is a rare cause of acute abdominal pain caused by inflammation of an epiploic appendage. It has a nonspecific clinical presentation that may mimic other acute abdominal pathologies on physical exam, such as appendicitis, diverticulitis, or cholecystitis. However, EA is usually benign and self-limiting and can be treated conservatively. We present the case of a patient with two episodes of EA, the first mimicking acute appendicitis and the second mimicking acute cholecystitis. Although recurrence of EA is rare, it should be part of the differential diagnosis of acute, localized abdominal pain. A correct diagnosis of EA will prevent unnecessary hospitalization, antibiotic use, and surgical procedures. PMID:28127129

  8. Mimicking Temperature Through Molecular Machines

    Science.gov (United States)

    Smith, David; Käs, Josef

    2003-03-01

    All eukaryotic cells depend on mechanisms of self-assembly of protein filaments to form a cytoskeleton within the cell. The need for motility and reaction by cells to stimuli additionally requires the existence of pathways which serve to restructure and disassemble cytoskeletal structures. Temperature-driven increases in disorder are the most physically fundamental method for breaking down complex structures, yet would play a destructive role in cellular dynamics. A similar situation is seen on the genetic level with the unfolding of DNA strands for replication and cell division - while temperature-driven unfolding of the strands stands as the most simple pathway, molecular machinery are present to perform the same function without heat-induced damage to the cell (Lodish et al, 2000). We report experimental evidence of a similar mechanism functioning on actin cytoskeletal dynamics, involving collections of the actin-specific molecular motor Myosin II. While crosslink-driven bundling self-assembles complex actomyosin structures (including bundles, asters, and large aggregates) in the near-chemical-equilibrum state, an activation of the motors causes a rapid disassembly of all structures. Such a mechanism is not only harmless to cell function, but occurs on a very rapid timescale which is favorable for quick cytoskeletal dynamics.

  9. Ovarian Heterotopic Pregnancy Clinically Mimicking Endometrioma

    Directory of Open Access Journals (Sweden)

    Nilüfer ONAK KANDEMİR

    2010-01-01

    Full Text Available Heterotopic pregnancy is a very uncommon entity with a difficult preoperative diagnosis. In the present study, we presented an ovarian heterotopic pregnancy case, clinically mimicking endometrioma developed in a 33-year-old female following an in vitro fertilization procedure. The importance of clinical, histopathological, and immunohistochemical examinations in the diagnosis of heterotopic pregnancy are emphasized.

  10. An adult intussusception mimicking early appendicitis

    Institute of Scientific and Technical Information of China (English)

    Wei-Chun Tseng; Cheng-Ting Hsiao; Yu-Cheng Hung

    2012-01-01

    Adult intussusception is rare with variable clinical presentation. We reported a case of adult intussusception presenting with symptoms mimicking acute appendicitis. The patient presented as an abdominal pain from epigastric area with shifting gradually to RLQ. The atypical presentation of adult intussusception remains a diagnostic challenge to the emergency physician.

  11. Acute dystonia mimicking angioedema of the tongue

    DEFF Research Database (Denmark)

    Rasmussen, Eva Rye; Pallesen, Kristine A U; Bygum, Anette

    2013-01-01

    We report a case of acute dystonia of the face, jaw and tongue caused by metoclopramide and mimicking angioedema. The patient had attacks for several years before the correct diagnosis was made and we present the first ever published video footage of an attack. This adverse drug reaction is known...

  12. Toward restrictions on boson stars as black hole mimickers

    Energy Technology Data Exchange (ETDEWEB)

    Guzman, F S, E-mail: guzman@ifm.umich.mx [Instituto de Fisica y Matematicas, Universidad Michoacana de San Nicolas de Hidalgo, Edificio C3, Cd. Universitaria, 58040 Morelia, Michoacan (Mexico)

    2011-09-22

    The status of boson stars as black hole mimickers is presented among other mimickers. We focus on the analysis of the emission spectrum of a simple accretion disk model. We describe the free parameters that allow a boson star to become a black hole mimicker and present an example of a particular astrophysical case.

  13. Malignant Mesothelioma Mimicking Invasive Mammary Carcinoma in a Male Breast

    Directory of Open Access Journals (Sweden)

    Mohamed Mokhtar Desouki

    2015-01-01

    Full Text Available Malignant mesothelioma is an uncommon tumor with strong association with asbestos exposure. Few cases of malignant pleural mesothelioma metastatic to the female breast have been reported. Herein, we presented, for the first time, a case of locally infiltrating malignant pleural mesothelioma forming a mass in the breast of a male as the first pathologically confirmed manifestation of the disease. Breast ultrasound revealed an irregular mass in the right breast which involves the pectoralis muscle. Breast core biopsy revealed a proliferation of neoplastic epithelioid cells mimicking an infiltrating pleomorphic lobular carcinoma. IHC studies showed the cells to be positive for calretinin, CK5/6, WT1, and CK7. The cells were negative for MOC-31, BerEp4, ER, and PR. A final diagnosis of malignant mesothelioma, epithelioid type, was rendered. This case demonstrates the importance of considering a broad differential diagnosis in the setting of atypical presentation with application of a panel of IHC markers.

  14. Multiple skin tumors of indeterminate cells in an adult.

    Science.gov (United States)

    Kolde, G; Bröcker, E B

    1986-10-01

    An adult patient with multiple unusual histiocytic tumors of the skin is described. As shown by immunohistologic study, electron microscopy, and immunoelectron microscopy, the tumors represent circumscribed proliferations of the Langerhans cell-related indeterminate dendritic cells of the skin. This distinct cutaneous histiocytosis may represent a paraneoplastic syndrome.

  15. Cutaneous reactive histiocytosis in dogs: a retrospective evaluation of 32 cases.

    Science.gov (United States)

    Palmeiro, Brian S; Morris, Daniel O; Goldschmidt, Michael H; Mauldin, Elizabeth A

    2007-10-01

    Thirty-two cases of canine cutaneous histiocytosis were retrospectively evaluated. Median age at onset was 4 years. Lesions included nodules and plaques affecting the head/face, trunk and limbs, and erythema, swelling and depigmentation of the nasal planum/nares. Systemic involvement was not ruled out in all cases. All dogs had complete resolution of dermatological lesions after initial treatment (median 45 days). Initial treatment included prednisone +/- antibiotics (12 of 32 dogs), prednisone and tetracycline/niacinamide (four of 32), prednisone and azathioprine (three of 32), tetracycline/niacinamide +/- vitamin E/essential fatty acids (six of 32), antibiotics +/- antihistamines (three of 32), cyclosporine and ketoconazole (one of 32), topical therapy (two of 32), and no treatment (one of 32). Seventeen dogs received maintenance therapy which consisted of tetracycline/niacinamide +/- vitamin E/essential fatty acids (12 of 17), cyclosporine/ketoconazole (two to three times a week) (two of 17), azathioprine daily (one of 17), prednisone/azathioprine (two times a week) (one of 17), and prednisone daily (one of 17). Median follow up was 25 months. Nine dogs had a recurrence of cutaneous histiocytosis (median days to recurrence 130 days), with seven of nine having more than one recurrence. At study completion, six dogs were deceased (no lesions at the time of death) and 26 of 32 were alive with no lesions. Ten of 26 dogs were on maintenance treatment (eight tetracycline/niacinamide, one azathioprine, one vitamin E). Previous dermatological disease and season had no detectable influence on recurrence. Recurrence was significantly more likely in dogs with nasal planum/nares lesions than dogs without these lesions. Tetracycline/niacinamide was an effective treatment option for dogs in this study population.

  16. Neurosyphilis Mimicking Ramsay Hunt Syndrome.

    Science.gov (United States)

    Maeda, Tadashi; Yoshizawa, Sadako; Hirayama, Takehisa; Saga, Tomoo; Tateda, Kazuhiro; Urita, Yoshihisa

    2015-01-01

    A 36-year-old man presented with facial nerve palsy, hearing loss, vertigo and headache. He was initially diagnosed with Ramsay Hunt syndrome and treated with a systemic steroid and valaciclovir; however, his symptoms deteriorated. Serum rapid plasma reagin (RPR) and treponema pallidum hemagglutination tests were positive. Cerebrospinal fluid analysis revealed an elevated white blood cell count and positive RPR, confirming the diagnosis of neurosyphilis. Penicillin G (PCG) was administered, and his facial nerve function and headache improved. However, left-side hearing loss worsened temporarily, which was assumed to be a Jarisch-Herxheimer reaction. Betamethasone was administered along with PCG, and he recovered completely.

  17. Immunohistochemical demonstration of lysozyme in normal, reactive and neoplastic cells of the mononuclear phagocyte system.

    Directory of Open Access Journals (Sweden)

    Motoi,Makoto

    1984-04-01

    Full Text Available Using the peroxidase antiperoxidase (PAP method, lysozyme (LZM was shown to exist in normal, reactive and neoplastic cells belonging to the mononuclear phagocyte system (MPS, but was not detected in histiocytosis X cells. Immunostaining for cytoplasmic LZM by the PAP method is useful for identification of mononuclear phagocytes and for diagnosis of the diseases in which these cells participate.

  18. Spherical Boson Stars as Black Hole mimickers

    CERN Document Server

    Guzman, F S; 10.1103/PhysRevD.80.084023

    2010-01-01

    We present spherically symmetric boson stars as black hole mimickers based on the power spectrum of a simple accretion disk model. The free parameters of the boson star are the mass of the boson and the fourth order self-interaction coefficient in the scalar field potential. We show that even if the mass of the boson is the only free parameter it is possible to find a configuration that mimics the power spectrum of the disk due to a black hole of the same mass. We also show that for each value of the self-interaction a single boson star configuration can mimic a black hole at very different astrophysical scales in terms of the mass of the object and the accretion rate. In order to show that it is possible to distinguish one of our mimickers from a black hole we also study the deflection of light.

  19. Cardiac sarcoidosis mimicking right ventricular dysplasia.

    Science.gov (United States)

    Shiraishi, Jun; Tatsumi, Tetsuya; Shimoo, Kazutoshi; Katsume, Asako; Mani, Hiroki; Kobara, Miyuki; Shirayama, Takeshi; Azuma, Akihiro; Nakagawa, Masao

    2003-02-01

    A 59-year-old woman with skin sarcoidosis was admitted to hospital for assessment of complete atrioventricular block. Cross-sectional echocardiography showed that the apical free wall of the right ventricle was thin and dyskinetic with dilation of the right ventricle. Thallium-201 myocardial imaging revealed a normal distribution. Both gallium-67 and technetium-99m pyrophosphate scintigraphy revealed no abnormal uptake in the myocardium. Right ventriculography showed chamber dilation and dyskinesis of the apical free wall, whereas left ventriculography showed normokinesis, mimicking right ventricular dysplasia. Cardiac sarcoidosis was diagnosed on examination of an endomyocardial biopsy specimen from the right ventricle. A permanent pacemaker was implanted to manage the complete atrioventricular block. After steroid treatment, electrocardiography showed first-degree atrioventricular block and echocardiography revealed an improvement in the right ventricular chamber dilation. Reports of cardiac sarcoidosis mimicking right ventricular dysplasia are extremely rare and as this case shows, right ventricular involvement may be one of its manifestations.

  20. Pulmonary Paragonimiasis Mimicking Tuberculous Pleuritis

    Science.gov (United States)

    Luo, Jian; Wang, Mao-Yun; Liu, Dan; Zhu, Hui; Yang, Sai; Liang, Bin-Miao; Liang, Zong-An

    2016-01-01

    Abstract Pulmonary paragonimiasis is a food-borne zoonosis with a wide variety of radiologic findings, which sometimes can be confused with tuberculosis and carcinoma. Therefore, differential diagnosis is always warranted. A 43-year-old male farmer, with productive cough, blood-tinged sputum and chest pain, as well as patchy consolidation and pleural effusions in chest computer tomography, was misdiagnosed of community-acquired pneumonia and tuberculosis. Complete blood cell count, sputum smear and culture, chest computer tomography, thoracoscopy, and biopsy. The diagnosis of pulmonary paragonimiasis was established due to the finding of Charcot–Leyden crystals in the pleural necrosis, and antibodies against Paragonimus westermani in enzyme-linked immunosorbent assay. Paragonimiasis should be considered as a possibility in the differential diagnosis of tuberculosis. Thoracoscopy is an effective and valuable technology that can help make an accurate diagnosis. PMID:27082624