Danielle Mazziero Macedo
Full Text Available O xantogranuloma necrobiótico é doença crônica granulomatosa e xantomatosa, caracterizada por pápulas e placas infiltradas, eritematosas e amareladas, preferencialmente localizadas na região periorbital. É comum associar-se com paraproteinemia e risco aumentado para malignidades hematológicas e linfoproliferativas. Sua patogênese permanece desconhecida. Agentes alquilantes, como clorambucil e melfalan, podem ser utilizados no tratamento com sucesso variável. Relata-se um exemplo dessa rara doença em paciente com lesão única e sem paraproteinemia.Necrobiotic xanthogranuloma is a chronic granulomatous and xantomathous disease, characterized by indurated, nontender, yellowish and erythematous nodules and plaques especially located on the periorbital region. It is commonly associated with paraproteinemia and an increased risk for hematological and lymphoproliferative malignancies. Its pathogenesis remains unclear. Alkylating agents, such as chlorambucil and melphalan may be used to treat the disease with variable success. We report a case of this rare disease in a patient with a solitary tumor and without paraproteinemia.
Radl, J.; Glopper, E. de; Berg, P. van den; Zwieten, M.J. van
The influence of thymectomy on the appearance of idiopathic paraproteinemia (IP) during aging was investigated in mice of the C57BL/KaLwRij and CBA/BrARij strains, which under normal conditions develop IP in high and low frequency, respectively. Compared with sham-thymectomized mice, C57BL mice
Ong, F; Hermans, J; Noordijk, E M; De Kieviet, W; Seelen, P J; Wijermans, P W; Kluin-Nelemans, J C
Diagnostic systems for monoclonal gammopathies use bone marrow and X-ray examinations to exclude multiple myeloma (MM). Data from a population-based registry of unselected patients with paraproteinemia indicate that these tests are often done only when MM is suspected. We used 441 randomly selected
Full Text Available Terry Golombick, Terry DiamondDepartment of Endocrinology, St George Hospital, Kogarah, AustraliaAbstract: Plasma cell dyscrasias, most commonly associated with paraproteinemia, are a diverse group of diseases. Monoclonal gammopathy of undefined significance (MGUS can precede multiple myeloma, a progressive neoplastic disease. MGUS occurs in association with a variety of other diseases and currently no treatment is recommended but rather “watchful waiting”. Given that the size of the M-protein is a risk factor for disease progression, early intervention with the aim of reducing the paraprotein load would provide an innovative therapeutic tool. Preliminary results from our pilot study show a drop of between 5% and 30% serum paraprotein in patients taking curcumin compared with patients on placebo. Curcumin is a diferuloylmethane present in extracts of the rhizome of the Curcuma longa plant. As a natural product, this has exciting potential in the treatment of plasma cell dyscrasias.Keywords: plasma cell dyscrasias, MGUS, myeloma, curcumin, paraproteinemia
Radl, J.; Glopper, E.de; Schuit, H.R.E.; Zurcher, C.
Transplantation experiments in the C57BL/KaLwRij mouse model of idiopathic paraproteinemia (IP) showed that an IP-producing clone can be further propagated in young, lethally irradiated mice and also equally as well in nonirradiated recipients by a bone marrow and/or spleen cell transfer. The
Golombick, Terry; Diamond, Terrence H; Badmaev, Vladimir; Manoharan, Arumugam; Ramakrishna, Rajeev
To determine the effect of curcumin on plasma cells and osteoclasts in patients with MGUS. Twenty-six patients with MGUS were recruited into the study and administered 4 grams/day oral curcumin. Blood and urine samples were collected at specified visits after initiating therapy. Full blood count, B2 microglobulin, serum paraprotein, and immunoglobulin electrophoresis (IEPG and EPG) were determined for all patients at each visit. Serum calcium, 25 hydroxyvitamin D3, and bone-specific alkaline phosphatase were determined at baseline only. Urine, as a morning second-void sample, was collected at each visit for urinary N-telopeptide of type I collagen. Our results show that oral curcumin is able to decrease paraprotein load in a select group (i.e., those having a paraprotein level of >20 g/L) of patients with MGUS. Fifty percent (5 of 10) of these patients had a 12% to 30% reduction in their paraprotein levels, while on curcumin therapy. In addition, 27% of patients on curcumin had a >25% decrease in urinary N-telopeptide of type I collagen. Due to the possible progression of MGUS to multiple myeloma, the potential role of curcumin as a therapeutic intervention for MGUS patients warrants further investigation.
Cejka, J.; Bollinger, R.O.; Schuit, H.R.E.; Lusher, J.M.; Chang, C.H.; Zuelzer, W.W.
A 12-yr-old boy with acute leukemia was found to have paraproteinemia and Bence-Jones proteinuria. The paraprotein was characterized as immunoglobulin M, type κ and the Bence Jones protein as free κ-chains. Increased amounts of β2-microglobulin were found in the patient’s serum and urine. Electron
Hijmans, W.; Schuit, H.R.E.; Radl, J.; Vossen, J.M.J.J.
In a study of surface immunoglobulins (Ig) on lymphocytes from patients with paraproteinemia (1), we observed that a variable number of plasma cells not only contained intracellular Ig, but also had Ig on their surface, as shown in the vital technique of immunofluorescence. Moreover, in the bone
Anders Bisgaard Jensen
Full Text Available Waldenstrom's macroglobulinamia (WM is a rare malignant lymphoproliferative disorder, characterized by monoclonal IgM paraproteinemia and neoplastic proliferation of malignant lymphoplasmacytoid cells in the bone marrow. Traditionally, WM has been treated with modalities similar to those used in the management of other indolent lymphomas. Just recently, based on impressive clinical trial results in heavily pretreated WM patients, a new Bruton Tyrosine Kinase-inhibitor, Ibrutinib, has been approved for the treatment of this disorder. As the use of Ibrutinib in WM outside clinical trials is still limited, only few clinical reports illustrating treatment side effects are currently available. Here we review the current literature specific on Ibrutinib-associated rash in hematologic patients, and report on an elderly patient with WM, who developed a red maculopapular non-pruritic rash 12 weeks after starting Ibrutinib therapy. Without modifications of the ongoing Ibrutinib schedule, the rash regressed within two weeks of treatment with topical steroidcontaining dermatological compounds.
Full Text Available Scleromyxedema is a rare cutaneous mucinosis, usually presenting with generalized papular eruption and sclerodermoid induration, monoclonal gammopathy and systemic manifestations. An atypical clinical presentation with cutaneous and subcutaneous nodules has been reported rarely. In recent years, intravenous immunoglobulin (IVIg appears to be the therapy of choice for scleromyxedema. Treatment experiences in atypical manifestations with mucinous nodules are limited to sporadic reports. We report the case of male patient with atypical scleromyxedema without underlying paraproteinemia, presenting with generalized papular and sclerodermoid skin eruption and multiple nodular mucinous lesions on the fingers and face as well as on the eyelids, and associated systemic symptoms. Complete regression of all cutaneous lesions and extracutaneous symptoms with sustained remission was achieved by combined treatment with thalidomide and IVIg.
Dolenc-Voljč, M.; Jurčić, V.; Hočevar, A.; Tomšič, M.
Scleromyxedema is a rare cutaneous mucinosis, usually presenting with generalized papular eruption and sclerodermoid induration, monoclonal gammopathy and systemic manifestations. An atypical clinical presentation with cutaneous and subcutaneous nodules has been reported rarely. In recent years, intravenous immunoglobulin (IVIg) appears to be the therapy of choice for scleromyxedema. Treatment experiences in atypical manifestations with mucinous nodules are limited to sporadic reports. We report the case of male patient with atypical scleromyxedema without underlying paraproteinemia, presenting with generalized papular and sclerodermoid skin eruption and multiple nodular mucinous lesions on the fingers and face as well as on the eyelids, and associated systemic symptoms. Complete regression of all cutaneous lesions and extracutaneous symptoms with sustained remission was achieved by combined treatment with thalidomide and IVIg. PMID:24348379
Full Text Available Antiphospholipid antibodies (aPL are a heterogeneous group of antibodies that are detected in the serum of patients with a variety of conditions, including autoimmune (systemic lupus erythematosus, infectious (syphilis, AIDS and lymphoproliferative disorders (paraproteinemia, myeloma, lymphocytic leukemias. Thrombosis, thrombocytopenia, recurrent fetal loss and other clinical complications are currently associated with a subgroup of aPL designating the antiphospholipid syndrome. In contrast, aPL from patients with infectious disorders are not associated with any clinical manifestation. These findings led to increased interest in the origin and pathogenesis of aPL. Here we present the clinical features of the antiphospholipid syndrome and review the origin of aPL, the characteristics of experimentally induced aPL and their historical background. Within this context, we discuss the most probable pathogenic mechanisms induced by these antibodies.
Carla Bortolin Fonseca
Full Text Available pyoderma gangrenosum is a rare inflammatory skin condition characterized by progressive and recurrent skin ulceration of destructive course. It is usually associated with rheumatoid arthritis, paraproteinemia, myeloproliferative diseases and inflammatory bowel diseases, especially non-specific ulcerative proctocolitis. In these situations, skin lesions are described as concurrent with the intestinal condition. However, reports on pyoderma gangrenosum preceding intestinal findings are less frequent. The authors describe a case of a woman with febrile condition associated with skin lesions diagnosed by biopsy as pyoderma gangrenosum. Two weeks later, she developed diarrhea, arthralgia and sepsis, being diagnosed as ulcerative proctocolitis. After the administration of the treatment for ulcerative proctocolitis, she showed improvements in sepsis care, remission of diarrhea and regression of skin lesions. This case highlights the importance of considering pyoderma gangrenosum as a manifestation associated with inflammatory bowel disease, regardless of its timing in relation to intestinal symptoms.Pioderma gangrenoso é uma forma de inflamação cutânea, caracterizada por ulceração progressiva e recorrente da pele, com curso destrutivo. Geralmente é associada à artrite reumatoide, paraproteinemia, doenças mieloproliferativas e doença inflamatória intestinal, em especial retocolite ulcerativa inespecífica. Em tais casos, as lesões cutâneas são descritas concomitantes ao quadro intestinal, porém, relatos com descrição de pioderma gangrenoso precedendo achados intestinais são menos frequentes. Os autores relatam caso de mulher com quadro febril associado a lesões cutâneas diagnosticadas por biópsia como pioderma gangrenoso. Duas semanas depois, apresentou diarreia, artralgia e sepse sendo diagnosticada retocolite ulcerativa. Com o tratamento para retocolite ulcerativa apresentou melhora do quadro séptico, remissão da diarreia e
Lymphocytes are divided into 2 major classes: T and B lymphocytes (or cells). T cells are responsible for cell-mediated immune response, and B cells for humoral immune response or antibody formation. The possible immunological complications that might develop as the late manifestation of radiation effects include: lymphoid neoplasms, immune complex diseases, auto-aggressive immune reactions, and other degenerative diseases of immunological nature. The development of lymphoid neoplasma following the exposure to radiation was extensively studied with mice. Radiation-induced immunological compications would not contribute significantly to the life-shortening of exposed individuals. The extensive health survey of adult A-bomb survivors revealed little evidence of immunological complications such as rheumatoid arthritis, kidney diseases, paraproteinemia, etc. The young healthy adults who had received thymic irradiation during infancy for the treatment of enlarged thymus manifested higher incidence of illness with abnormal immunological features. Immune complex diseases, particularly the inter-capillary glomerulosclerosis of kidneys, develop as a result of earlier exposure to high dose of radiation. (Yamashita, S.)
Castillo, Jorge J; Jurczyszyn, Artur; Brozova, Lucie; Crusoe, Edvan; Czepiel, Jacek; Davila, Julio; Dispenzieri, Angela; Eveillard, Marion; Fiala, Mark A; Ghobrial, Irene M; Gozzetti, Alessandro; Gustine, Joshua N; Hajek, Roman; Hungria, Vania; Jarkovsky, Jiri; Jayabalan, David; Laubach, Jacob P; Lewicka, Barbara; Maisnar, Vladimir; Manasanch, Elisabet E; Moreau, Philippe; Morgan, Elizabeth A; Nahi, Hareth; Niesvizky, Ruben; Paba-Prada, Claudia; Pika, Tomas; Pour, Ludek; Reagan, John L; Richardson, Paul G; Shah, Jatin; Spicka, Ivan; Vij, Ravi; Waszczuk-Gajda, Anna; Gertz, Morie A
IgM myeloma is a rare hematologic malignancy for which the clinicopathological features and patient outcomes have not been extensively studied. We carried out a multicenter retrospective study in patients with diagnosis of IgM myeloma defined by >10% marrow involvement by monoclonal plasma cells, presence of an IgM monoclonal paraproteinemia of any size, and anemia, renal dysfunction, hypercalcemia, lytic lesions and/or t(11;14) identified by FISH. A total of 134 patients from 20 centers were included in this analysis. The median age at diagnosis was 65.5 years with a male predominance (68%). Anemia, renal dysfunction, elevated calcium and skeletal lytic lesions were found in 37, 43, 19, and 70%, respectively. The median serum IgM level was 2,895 mg dL -1 with 19% of patients presenting with levels >6,000 mg dL -1 . International Staging System (ISS) stages 1, 2, and 3 were seen in 40 (33%), 54 (44%), and 29 (24%) of patients, respectively. The malignant cells expressed CD20 (58%) and cyclin D1 (67%), and t(11;14) was the most common cytogenetic finding (39%). The median overall survival (OS) was 61 months. Higher ISS score was associated with worse survival (P = 0.02). Patients with IgM myeloma present with similar characteristics and outcomes as patients with more common myeloma subtypes. © 2017 Wiley Periodicals, Inc.
Seaux, Liesbeth; Van Houcke, Sofie; Dumoulin, Els; Fiers, Tom; Lecocq, Elke; Delanghe, Joris R
Analytical interferences have been described due to the presence of various exogenous UV-absorbing substances in serum. Iodine-based X-ray contrast agents and various antibiotics have been reported to interfere with interpretation of serum protein pherograms, resulting in false diagnosis of paraproteinemia. In the present study, we have explored the possibility of measuring UV absorbance at two distinct wavelengths (210 and 246 nm) to distinguish between true and false paraproteins on a Helena V8 clinical electrophoresis instrument. This study demonstrates that most substances potentially interfering with serum protein electrophoresis show UV-absorption spectra that are distinct from those of serum proteins. Scanning at 246 nm allows detection of all described interfering agents. Comparing pherograms recorded at both wavelengths (210 and 246 nm) enables to distinguish paraproteins from UV-absorbing substances. In case of a true paraprotein, the peak with an electrophoretic mobility in the gamma-region decreases, whereas the X-ray contrast media and antibiotics show an increased absorption when compared to the basic setting (210 nm). The finding of iodine-containing contrast media interfering with serum protein electrophoresis is not uncommon. In a clinical series, interference induced by contrast media was reported in 54 cases (of 13 237 analyses), corresponding with a prevalence of 0.4%. In the same series, 1631 true paraproteins (12.3%) were detected. Implementation of the proposed algorithm may significantly improve the interpretation of routine electrophoresis results. However, attention should still be paid to possible interference due to presence of atypical proteins fractions (e.g., tumor markers, C3). © 2014 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.