Cases reported "Cryoglobulinemia"

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1/19. hepatitis c virus, autoimmunity and lymphoproliferation.

    We summarize clinical, laboratory and pathologic details regarding a patient who presented with extrahepatic disease manifestations of hepatitis c virus (HCV) infection, including cryoglobulinemic leg ulcers due to cutaneous vasculitis, peripheral sensorimotor neuropathy, and recurrent pulmonary infiltrates. The patient had evidence for B-cell lymphoproliferation, diagnosed as extranodal lymphoma on initial (though not subsequent) bone marrow examination, retroperitoneal lymphadenopathy, and the presence of a Type II IgM6 monoclonal rheumatoid factor which became cryoprecipitable on complexing to IgG. Chronic hepatitis was mild on liver biopsy, though fibrotic changes developed over a three-year period of follow-up. She had consistently normal liver function tests, except for a brief rebound effect on discontinuing interferon-alpha, and preterminally. Symptoms were only partially responsive to trials of corticosteroids, cytotoxic agents, plasmapheresis and interferon, and the patient ultimately died at The Mount Sinai Hospital of sepsis. We review current information regarding the spectrum of extrahepatic HCV infection, including pathogenic factors relevant to its overlapping autoimmune, rheumatic and lymphoproliferative disease manifestations. The exact prevalence of these HCV-related syndromes among the 1% of the world population estimated to be infected by this virus remains to be delineated. Chronicity of infection, and lack of efficacy of currently available therapy in effecting sustained clearance of the virus from the host, have made this an important public health problem that is likely to increase in significance. Possible relationships to non-Hodgkin's lymphoma may present novel opportunities to delineate the basis for oncogenesis in HCV infection.
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2/19. Pre-malignant and malignant lymphoproliferations in an HCV-infected type II mixed cryoglobulinemic patient are sequential phases of an antigen-driven pathological process.

    Type II mixed cryoglobulinemia (MC) is a systemic vasculitis characterized by the presence in the serum of a monoclonal cryoprecipitable IgM with rheumatoid factor (RF) activity. hepatitis c virus (HCV) has been recognized as its major etiologic factor. Because MC frequently evolves into overt B-cell non-Hodgkin's lymphoma (NHL), chronic HCV infection is hypothesized to lead to both benign and malignant lymphoproliferative disease. In this study, we investigated mutations in the V(H) and V(K) genes of the B-cell clone originating the overt B-cell lymphoma in a subject with MC. Mutational patterns were analyzed longitudinally in two bone marrow biopsies obtained at the stage of MC, as well as in multiple involved tissues (bone marrow, liver, and peripheral blood cells) at the stage of overt NHL. Hybridization of variable-diversity-joining (VDJ) PCR products with a probe specific for the neoplastic clone indicated that the lymphoma originated from one of the clones over-stimulated during MC. This clone producing an IgM highly homologous to a protein with RF specificity may explain the MC syndrome in the patient. Moreover, the presence of an IgH ongoing mutation process and the expression of an Ig antigen receptor significantly homologous to an anti-HCV protein support the hypothesis that the MC syndrome and the subsequent evolution to NHL are antigen-driven lymphoproliferative processes possibly sustained by HCV. Furthermore, the marked reduction in intra-clonal diversity in the last bone marrow biopsy obtained at the stage of overt NHL points out a minor dependence of the cells on the antigen-driven mechanism, although an intrinsic propensity of the neoplastic cell to undergo replacement mutations cannot be excluded.
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3/19. Biclonal gammopathy and platelet antibodies in a patient with chronic hepatitis c virus infection and mixed cryoglobulinemia.

    The presence of biclonal gammopathy [immunoglobulin (Ig)Mk IgAlambda] and platelet antibodies in a patient with chronic hepatitis c virus (HCV) infection is reported. A type-II cryoglobulinemia (IgMk and polyclonal IgG) has also been detected. The IgAlambda monoclonal component still persists after removal of the cryoprecipitate. Two populations of atypical lymphoid cells with plasmocytoid features in bone marrow have been detected. They express IgM with k restriction and IgA with lambda restriction, respectively. These cells are CD19 , CD5 , bc12 , and Ki67-. Liver biopsy shows lymphoid infiltrates with features of B follicles. thrombocytopenia with platelet antibodies but without splenomegaly is also present. This case is interesting because two hematologic extrahepatic manifestations simultaneously occur in a patient with HCV infection. Moreover the association between HCV and biclonal gammopathies has never been reported.
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4/19. Molecular study of an IgG1kappa cryoglobulin yielding organized microtubular deposits and glomerulonephritis in the course of chronic lymphocytic leukaemia.

    glomerulonephritis with organized microtubular monoclonal immunoglobulin deposits (GOMMID) and glomerulonephritis related to type I cryoglobulin are well-known but rare complications of B cell derived chronic lymphocytic leukaemia. In these disorders, monoclonal Ig have never been studied at the molecular level. We conducted a pathological and molecular analysis in a patient with chronic lymphocytic leukaemia, glomerulonephritis and a single circulating monoclonal Ig. Unusual IgG1kappa kidney deposits were observed. The heavy and light chain variable region sequences of that cryoprecipitating monoclonal Ig were characterized. light microscopy revealed glomerulonephritis typical of cryoglobulinaemia, with neutrophil and macrophage infiltration, endocapillary hyperplasia and few protein thrombi. Electron microscopic study clearly evidenced numerous subepithelial mixed granular and organized deposits with a unique microtubular organization, reminiscent of the GOMMID. The Ig molecule sequence revealed alterations of charge and hydrophobicity potentially promoting a crystal-like aggregation and the aggregation of microtubules.This description suggests that common mechanisms are involved in various forms of precipitation and/or deposition of complete Ig molecules, with a variable extent of organization and with a possible overlap between pathological patterns of either glomerulonephritis with microtubular deposits or type I cryoglobulinic glomerulonephritis.
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5/19. Renal involvement in monoclonal (type I) cryoglobulinemia: two cases associated with IgG3 kappa cryoglobulin.

    Renal involvement has been described rarely in monoclonal (type I) cryoglobulinemia, although this complication is frequent among patients with mixed (type II or III) cryoglobulin. We report two patients with glomerulonephritis and monoclonal IgGkappa cryoglobulin. Both patients presented with nephrotic syndrome, microscopic hematuria, and impaired renal function. hepatitis c serology was negative, bone marrow aspiration was normal, and the renal biopsy specimen showed membranoproliferative glomerulonephritis with glomerular subendothelial deposits of monoclonal IgGkappa. In both cases, circulating cryoglobulin and monotypic tissue deposits were found to be IgG3kappa, suggesting that this isotype may have a particular propensity to cause this type of membranoproliferative glomerulonephritis. Although 18 cases of type I cryoglobulinemia with biopsy-proven glomerulonephritis have been reported to date, this is the first characterization of immunoglobulin heavy-chain isotype in this disease.
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6/19. Long-term effects of anti-CD20 monoclonal antibody treatment of cryoglobulinaemic glomerulonephritis.

    BACKGROUND: Type II mixed cryoglobulinaemia (MC) is a systemic vasculitis, associated in most cases with hepatitis c virus (HCV) infection, and sustained by proliferation of oligoclonal cells. Systemic B-cell depletion and clinical remission can be achieved in non-Hodgkin lymphoma by a human/mouse chimeric monoclonal antibody that specifically reacts with the CD20 antigen (Rituximab). Similar effects could be expected in type II MC. methods: Six patients, mean age 64.2 years (range: 37-76 years), with HCV infection genotype 2a2c (three cases) or 1b (three cases) and symptomatic type-II MC with systemic manifestations, including renal involvement (five cases) and bone marrow clonal restriction (three cases), were considered eligible for Rituximab therapy. Rituximab was administered intravenously at a dose of 375 mg/m(2) on days 1, 8, 15 and 22. Two more doses were administered 1 and 2 months later. No other immunosuppressive drugs were added. Response was evaluated by assessing the changes in clinical signs, symptoms and laboratory parameters for < or = 18 months. RESULTS: Levels of proteinuria, erythrocyte sedimentation rate and cryocrit significantly decreased at 2, 6 and 12 months. rheumatoid factor and IgM significantly decreased at 6 months whereas C4 values significantly increased at 2 and 6 months. HCV viral load and immunoglobulin g remained stable. Bone marrow abnormalities were found to reverse to normal in all three positive cases. Constitutional symptoms (skin ulcers, purpura, arthralgia, weakness, paraesthesia and fever) disappeared or improved. No acute or delayed side effects were observed. CONCLUSIONS: Rituximab appears to be a safe and effective therapeutic option in symptomatic patients with HCV-associated MC glomerulonephritis and signs of systemic vasculitis.
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7/19. Pure red-cell aplasia in a peritoneal dialysis patient with HCV-related cryoglobulinemia in the absence of neutralizing antierythropoietin antibodies.

    Pure red-cell aplasia (PRCA) in recombinant human erythropoietin (rHuEpo) treated patients is a matter of growing concern. In most cases, neutralizing anti-EPO antibodies have been detected in patient serum and held responsible for the development of PRCA. We describe a 68-year-old white woman suffering from HCV-related cryoglobulinemia and chronic kidney disease on renal replacement therapy with peritoneal dialysis. Five months after the introduction of epoetin-b therapy she developed a PRCA, as shown by the bone marrow aspirate. Cryocrit rose from 5% to 15% at this time, reticulocyte count fell, while white blood cells and platelets remained within normal values. Epoetin-b therapy was discontinued and steroid treatment was started. The test for anti-erythropoietin antibodies was negative. Hemoglobin and reticulocytes progressively rose and steroid therapy was tapered and eventually stopped, when the cryocrit was 3%. We propose that a relapse in the HCV-related cryoglobulinemia might be held responsible for the erythropoietic marrow failure.
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8/19. A case of type I cryoglobulinemia associated with a monoclonal gammopathy of undetermined significance (MGUS).

    A 79-year-old Japanese male who had been suffering from renal insufficiency of unknown causes visited us with reddish papules and purpura on both forearms of a month's duration. A skin biopsy was performed from the right forearm, and the histopathology showed an obstruction of superficial blood vessels with eosinophilic amorphous materials suggesting cryoglobulins. Although further investigations revealed the presence of monoclonal immunoglobulin (Ig) G-kappa type I cryoglobulin, a bone marrow biopsy demonstrated that there was 7.6% proliferation of plasmacytoid cells, and the serum level of Ig G was less than 3 g/dl. The diagnosis was type I cryoglobulinemia associated with monoclonal gammopathy of undetermined significance as the possible cause of chronic renal failure. Therefore, purpuric changes on extremities of the elderly, especially those complaining of renal failure should, be taken seriously and not regarded simply as an age-related phenomenon.
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9/19. Alveolar hemorrhage in cryoglobulinemia--an indicator of poor prognosis.

    OBJECTIVE: Alveolar vasculitis is an unusual event in the course of cryoglobulinemia (CG). The inflammatory process involving the alveolar capillary walls may result in severe alveolar hemorrhage and consequently lead to a grave outcome. The objective of this study was to evaluate the occurrence of this unusual finding in CG. methods: We reviewed the records of all patients with CG who developed acute alveolitis, registered their associated clinical and laboratory parameters and evaluated the possible impact these parameters may have on their prognosis. In addition we scanned the medline for similar cases. RESULTS: Of the 125 patients with CG who were hospitalized in our medical center during the last 23 years, 4 (3.2%) developed alveolar hemorrhage. All patients exhibited extreme fatigue, fever with clinical and radiological evidence of alveolitis. Of the 4 new cases, 1 had type II CG and 3 had type III CG. Of our 4 patients, 3 developed concomitant acute renal failure necessitating hemodialysis. A literature survey resulted in 6 additional cases. All 10 patients experienced acute respiratory insufficiency and eight had at least one episode of hemoptysis. In the other 2 patients the bronchoalveolar lavage (BAL) fluid contained hemosiderin laden macrophages. Five of the 10 patients had concomitant hepatitis C virus (HCV) infection; 2 patients were seen prior to modern identification of the HCV; however, liver abnormalities were not described. Of the 10 patients 5 patients had type II CG and 5 others had type III. Of the 7 patients in whom outcome was available, 6 died from their illness. Acute renal failure or exacerbation of antecedent glomerular disease occurred in 8 patients. CONCLUSIONS: Alveolitis is a rare manifestation of CG, presenting as an overwhelming systemic illness and portends a poor prognosis with a high mortality rate.
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10/19. Crystalline deposits in ascites in a case of cryoglobulinemia.

    Crystalline inclusions were observed on routine cytologic studies of ascitic fluid from a patient with exudative ascites of undetermined cause. These inclusions were polymorphic, but frequently appeared as slender needles. They were seen most often in histiocytes and, less frequently, in mesothelial cells and plasma cells. Extracellular crystals were also seen. The crystals were also present in biopsy specimens of peritoneum, liver and bone marrow. Special studies by polarizing light microscopy, cytochemistry, electron microscopy and immunocytochemistry suggested that the crystals were most probably immunoglobulins synthesized by plasma cells. Some were excreted extracellularly and phagocytized by histiocytes. Further studies indicated that the patient had an indolent plasma-cell dyscrasia, resulting in excessive production of a monoclonal immunoglobulin. The immunoglobulin may have crystallized and become deposited in tissues, inciting chronic inflammatory changes. Massive deposits of crystals in the peritoneum, with the resultant inflammatory reaction, was possibly the cause of the formation of ascites.
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