[Ulceronecrotic purpura revealing Waldenström's macroglobulinemia].
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Biomedical subjects
Publications and source records attributed to J Soubeyrand.
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We report a case of fulminant presentation of thrombotic microangiopathy associated with Hodgkin's lymphoma in an HIV-infected patient. After aggressive therapy with antiretroviral drugs, chemotherapy, and plasmatherapy, complete remission of thrombotic microangiopathy and Hodgkin's lymphoma was observed.
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INTRODUCTION: The objective of this work was to review current data about the physiopathology, clinical features, and treatment of thrombotic thrombocytopenic purpura (Moschowitz's syndrome). CURRENT KNOWLEDGE AND KEY POINTS: Thrombotic thrombocytopenic purpura is a rare disorder characterized by widespread thrombotic injuries of platelets in the microcirculation. Its physiopathology has been elucidated recently. Evidence of a deficiency of Von Willebrand's factor-cleaving protease would be due to either IgG antibodies in the acute form of the disease or constitutional deficiency in the chronic form of the disease. FUTURE PROSPECTS AND PROJECTS: Plasma exchange is the current reference treatment. However, in the light of recent publications, either infusions of concentrates of purified enzyme or more intensive immunosuppressive therapy would be more specific.
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INTRODUCTION: Microscopic colitis describes a subset of patients with chronic watery diarrhea of unknown origin, and normal endoscopic findings and microscopic evidence of an inflammatory infiltrate in the colonic mucosa. We report two cases associated with sicca syndrome. EXEGESIS: A 56-year-old woman and a 76-year-old man presented with a history of lymphocytic colitis associated with sicca syndrome. Drugs or infectious agents were not implicated in the cause of lymphocytic colitis, suggesting that sicca syndrome may be involved in the pathogenesis of microscopic colitis. CONCLUSION: These cases suggest that sicca syndrome should be detected in patients with lymphocytic colitis.
INTRODUCTION: Red cell autoantibodies are very rarely observed in pregnant women. We report one case related to the presence of a lupus anticoagulant. EXEGESIS: A 32-year-old woman had a history of two intrauterine deaths that had occurred at weeks 32 and 33, respectively. Due to the presence of a lupus anticoagulant, prednisone (20 mg/day) and subcutaneous calcium heparin were administered during her third pregnancy. The direct antiglobulin test remained positive throughout the pregnancy; however, hemolysis could not be detected. Caesarean section was done in emergency at week 29, due to fetal bradycardia related to severe fetal hemolysis. CONCLUSION: When autoimmune hemolysis occurs during pregnancy, risks for the fetus increase with the severity of hemolysis in the mother. It is thought that child mortality reflects the existence of anemia, rather than the existence of hemolysis. Our observation suggests that severe fetal hemolysis may occur, despite the absence of hemolysis in the mother.
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OBJECTIVE: An open, uncontrolled trial of ribavirin, an oral guanosine nucleoside analog for treatment of hepatitis C, in patients with hepatitis C virus (HCV) associated cryoglobulinemia intolerant to interferon. METHODS: Five patients with cryoglobulinemia related to HCV infection unresponsive to interferon therapy received oral ribavirin (100 to 1200 mg daily) for 10 to 36 months. RESULTS: Patients treated with ribavirin had prompt decrease in serum aminotransferase levels and marked improvement of manifestations of cryoglobulinemia within a few weeks. Ribavirin did not eradicate HCV RNA from the sera, but a decrease in viral load was observed in 3 patients, from 232 to 86 x 10(5) copies HCV/ml. Relapse occurred within 3 months once therapy was discontinued. The drug was well tolerated, but mild dose related hemolysis was common. CONCLUSION: Ribavirin monotherapy may be effective in patients with symptomatic cryoglobulinemia related to HCV infection, but this effect is not sustained when ribavirin therapy is discontinued.
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