Cases reported "Cryoglobulinemia"

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1/62. Diffuse recidivant alveolar hemorrhage in a patient with hepatitis c virus-related mixed cryoglobulinemia.

    A case of diffuse and recidivant alveolar hemorrhage is presented in a patient with hepatitis c virus-related type II mixed cryoglobulinemia with membranoproliferative glomerulonephritis. The patient was a 48-year-old white woman who suffered several outbreaks of pulmonary hemorrhage refractory to treatment with steroids, cyclophosphamide, azathioprine, plasmapheresis and interferon-alpha. The patient also presented persistent increased titers of immune complexes and rheumatoid factor with no histological hepatic alterations. Some considerations about evolution and treatment are given according to the updated physiopathology of this disease.
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ranking = 1
keywords = membranoproliferative, membranoproliferative glomerulonephritis, glomerulonephritis
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2/62. Membranoproliferative glomerulonephritis type I, mixed cryoglobulinemia and lymphoma in the absence of hepatitis c infection.

    Chronic hepatitis c virus infection has been linked to cryoglobulinemia, membranoproliferative glomerulonephritis, and malignant B-cell lymphoproliferation, suggesting a possible pathogenetic link between these disorders. We report a patient with the latter clinical triad in the absence of hepatitis c infection. We postulate that the persistent and dysregulated immunologic activity associated with chronic antigen stimulation, inflammation and/or B-cell malignancy induces nephritogenic autoantibodies, including cryoglobulins, that produce a similar clinical syndrome in genetically susceptible individuals. copyright copyright 1999 S. Karger AG, Basel
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ranking = 1.8335440375414
keywords = membranoproliferative, membranoproliferative glomerulonephritis, glomerulonephritis
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3/62. Recovery from hepatitis c virus-positive cryoglobulinaemic glomerulonephritis after interferon therapy.

    The association between mixed cryoglobulinaemia and chronic hepatitis c virus infection has recently been described. Cryoglobulinaemic glomerulonephritis, a complication of mixed cryoglobulinaemia, is usually treated with immunosuppressive therapy, but, given the presence of viral infection, this therapy is no longer recommended. This report concerns a case of a 30-year-old patient with cryoglobulinaemic glomerulonephritis, refractory to steroid treatment, in whom recovery from hepatitis c virus infection was obtained as well as from cryoglobulinaemic glomerulonephritis after interferon therapy. The clinical symptoms and laboratory tests were normal after prolonged interferon therapy and, 3 years after the end of treatment, the patient is free from disease.
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ranking = 1.4587020656974
keywords = glomerulonephritis
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4/62. The effect of combination therapy with interferon and cryofiltration on mesangial proliferative glomerulonephritis originating from mixed cryoglobulinemia in chronic hepatitis c virus infection.

    Cryofiltration, which has developed from double filtration plasmapheresis (DFPP) with a cooling unit, is an on-line technique to remove cryoglobulin. We report on a patient who suffered from progressive edema and renal insufficiency caused by cryoglobulinemic membranoproliferative glomerulonephritis (MPGN), probably due to chronic hepatitis c virus (HCV) infection. To remove cryoglobulins and terminate the HCV infection, we utilized combination therapy with cryofiltration and interferon-alpha injection with corticosteroids. interferon-alpha was capable of decreasing proteinuria but not diminishing cryoglobulin. Additional cryofiltration could remove cryoglobulin to an undetectable level. This combination therapy was partially successful to reduce proteinuria and prevent the progressive deterioration of renal function. The major adverse effects of this therapy were bleeding and myelosuppression. We conclude that this combination therapy may be effective and should be considered as treatment for cryoglobulinemic MPGN.
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ranking = 1.8335440375414
keywords = membranoproliferative, membranoproliferative glomerulonephritis, glomerulonephritis
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5/62. Membranoproliferative glomerulonephritis and demyelinating neuropathy caused by type II mixed cryoglobulinemia associated with HCV infection.

    Type II cryoglobulinemia is sometimes observed in patients with HCV infection. It is also well known that some cases with membranoproliferative glomerulonephritis (MPGN) possess cryoglobulinemia, which can induce neuropathy. We treated a 73-year-old woman with hepatitis c virus (HCV) who was admitted to our department because of proteinuria. She was diagnosed as MPGN according to renal biopsy. Her ankle jerk was absent with reduced touch sensation and vibration below the knees; sural nerve biopsy revealed demyelinating neuropathy. Administration of prednisolone resulted in improved proteinuria. cryoglobulinemia associated with HCV infection seemed to induce MPGN and demyelinating neuropathy.
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ranking = 1.8335440375414
keywords = membranoproliferative, membranoproliferative glomerulonephritis, glomerulonephritis
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6/62. splenectomy may improve the glomerulopathy of type II mixed cryoglobulinemia.

    Many patients with type II mixed cryoglobulinemia have been shown to be infected with hapatitis C virus (HCV). Therefore, interferon-alfa has become the first choice of treatment for patients with HCV-associated cryoglobulinemia. However, the disease often relapses after the discontinuation of interferon therapy. The long-term effect of interferon therapy is controversial. Therefore, a more effective therapy needs to be developed. A 62-year-old Japanese woman was admitted to our hospital for the examination of abnormal liver function tests, severe edema, and purpura in her lower extremities. Glomerulopathy secondary to HCV-related cryoglobulinemia was suspected. Her serum creatinine was increased to 2.1 mg/dL. Interferon therapy was considered initially. However, because of pancytopenia caused by liver cirrhosis and splenomegaly, splenectomy was performed in February 1997, before the start of interferon therapy. Renal biopsy specimen taken at the time of the splenectomy showed typical cryoglobulinemic glomerulonephritis. Gradually, after surgery, the patient's thrombocytopenia and anemia improved, her proteinuria and hematuria were decreased, her cryocrit dropped from 15% to 5%, the Ccr increased from 21.1 mL/min to 48.8 mL/min, and the purpura in her lower extremities disappeared. A repeat renal biopsy performed in May 1998 showed marked histological improvement. splenectomy is not widely accepted as a treatment for cryoglobulinemia. Our case suggests the possibility that the monoclonal-IgM component of the type II cryoglobulin may be formed in the spleen. In conclusion, splenectomy may be an effective therapy for cryoglobulinemia in patients with HCV-positive liver cirrhosis and pancytopenia secondary to splenomegaly.
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ranking = 0.20838600938534
keywords = glomerulonephritis
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7/62. Cryofiltration and oral corticosteroids provide successful treatment for an elderly patient with cryoglobulinemic glomerulonephritis associated with hepatitis c virus infection.

    A 75-year-old man was admitted due to nephrotic syndrome, purpura on the legs, which was associated with hepatitis c virus (HCV), and type II mixed cryoglobulinemia. Renal biopsy revealed features of cryoglobulinemic glomerulonephritis. Since the patient was elderly and the HCV genotype was Ib, interferon-alpha for reducing HCV was not indicated. Four sessions of cryofiltration and the administration of corticosteroids improved the proteinuria and renal function strikingly without adverse effects. This case demonstrates that an elderly patient who has nephrotic syndrome caused by cryoglobulinemic glomerulonephritis associated with HCV can be treated safely by cryofiltration with low doses of oral corticosteroids.
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ranking = 1.2503160563121
keywords = glomerulonephritis
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8/62. Complete remission of cryoglobulinemic glomerulonephritis (HCV-positive) after high dose interferon therapy.

    We report the case of a 64-year old woman with hepatitis c virus infection, mixed cryoglobulinemia type II (IgG IgM kappa) and cryoglobulinemic glomerulonephritis. The patient was treated with the standard dose of recombinant interferon alpha-2b (3 million units 3 times a week) for one year, resulting in complete clinical remission and undetectable levels of serum hepatitis c virus rna. AST and ALT normalized and proteinuria decreased from 2.78 to 0.98 g/day. However, a relapse occurred when therapy was stopped. Additional therapy with interferon-alpha (5 million units 3 times a week for 9 months) resulted again in quick and prolonged remission. The clinical course of our patient showed sustained clinical and virologic response after high-dose interferon-alpha treatment confirming the usefulness of interferon alpha in treatment of patients with cryoglobulinemic glomerulonephritis. Our observation is in agreement with others, suggesting that recommended standard dosage and duration of initial treatment with interferon alpha should be re-evaluated. Although our patient had sustained virologic and clinical response after interferon alpha monotherapy, recent studies clearly support combination therapy of interferon alpha and ribavirin for treatment of chronic HCV infections.
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ranking = 1.2503160563121
keywords = glomerulonephritis
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9/62. The benefit of long-term interferon alfa therapy for symptomatic mixed cryoglobulinemia (cutaneous vasculitis/membranoproliferative glomerulonephritis) associated with chronic hepatitis c infection.

    The efficacy of long-term interferon therapy for chronic hepatitis c infection with symptomatic mixed cryoglobulinemia has not clearly been defined. We describe a patient with chronic hepatitis c, symptomatic mixed cryoglobulinemia (cutaneous vasculitis), and membranoproliferative glomerulonephritis (MPGN) who responded clinically, biochemically, and virologically to a 1-year course of interferon therapy. Interferon side effects were minimal. Relapse occurred when interferon was discontinued, and suppressive maintenance interferon therapy was required for clinical, biochemical, and virologic remission. During the 5th year of maintenance therapy, she developed potential side effects that necessitated discontinuation of interferon treatment. After treatment stoppage, a clinical, biochemical, and virologic remission was maintained for more than 1 year. However, the potential side effects, which included eye irritation, arthralgias, myalgias, fatigue, insomnia, memory loss, and depression, persisted. Ophthalmologic, rheumatologic, and neurologic evaluations were nondiagnostic. Psychometric testing revealed dementia and mood disorder. Because the disabling symptoms persisted after 9 months, a health-related quality of life assessment was carried out with the SF-36 survey. Compared with healthy individuals and patients with chronic hepatitis c, our case had a lower health-related quality of life assessment on six out of seven scales and on four out of seven scales of the SF-36 survey, respectively. This case report indicates that long-term maintenance interferon therapy was effective in the treatment of symptomatic mixed cryoglobulinemia and its renal complications and resulted in a clinical, biochemical, and virologic sustained response. It is postulated that the side effects of long-term interferon therapy in this setting may be problematic.
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ranking = 5
keywords = membranoproliferative, membranoproliferative glomerulonephritis, glomerulonephritis
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10/62. interferon-alpha in combination with ribavirin as initial treatment for hepatitis c virus-associated cryoglobulinemic membranoproliferative glomerulonephritis.

    Mixed cryoglobulinemia (MC) and glomerulonephritis are the most important extrahepatic manifestations of chronic hepatitis c virus (HCV) infection. In HCV-infected patients with MC, renal involvement worsens the overall prognosis because of a high incidence of infection or cardiovascular disease. The relationship between MC and HCV infection has prompted the use of antiviral therapy. Two patients with chronic HCV infection, type-II MC and membranoproliferative glomerulonephritis (MPGN), presenting as nephrotic syndrome were treated with interferon (IFN)-alpha (3 MU 3 times per week) and ribavirin (15 mg/kg daily) for 6 months. Laboratory tests included measurement of anti-HCV antibodies, HCV rna, and HCV genotyping, and characterization of circulating cryoglobulins. A pretreatment renal biopsy was performed, and the histopathologic lesions were scored according to the index of disease activity. viremia and cryoglobulinemia were suppressed in both patients. However, a complete remission of proteinuria was observed in 1 patient only. The evaluation of the renal biopsy specimens revealed a mild MPGN (activity score: 5/24) in the patient with remission of proteinuria and a severe MPGN (activity score: 15/24) in the patient who maintained a nephrotic-range proteinuria. Although a fully satisfactory treatment is not yet available, we feel that a reasonable therapeutic strategy for HCV-infected patients with MC nephritis could be as follows: (1) antiviral treatment alone for patients with a low-grade kidney involvement, and (2) a short-term course of steroids and cytotoxic drugs followed by antiviral therapy for acute exacerbations and/or rapidly progressive GN.
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ranking = 5.2083860093853
keywords = membranoproliferative, membranoproliferative glomerulonephritis, glomerulonephritis
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