[Meningoradiculitis with neurologic deficit with a favorable spontaneous course in acquired immunodeficiency syndrome].
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Biomedical subjects
Publications and source records attributed to J C Valcke.
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The authors report the association of a refractory sideroblastic anemia and a "non-excreting" plasma cell myeloma. The anemia was observed 8 years before the onset of the myeloma, which had typical hematological characteristics. Both kappa and gamma light chains were found in the cytoplasm and on the surface of the plasma cells. Pathologic characteristics of this type of myeloma are briefly recalled, as well as the relationships between sideroblastic anemia and kahler's disease, an example of disorders of two lines of hematopoiesis (erythrocytes and leucocytes).
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OBJECTIVE: To study the long term effects of monthly intravenous cyclophosphamide therapy in Wegener's granulomatosis. METHODS: Fourteen consecutive patients with active Wegener's granulomatos treated with a first-line combination of high-dose prednisone and monthly intravenous pulse cyclophosphamide were retrospectively studied. RESULTS: One patient died from septicemia complicating severe leukopenia after the first pulse. At 8 months after instituting intravenous pulse cyclophosphamide therapy, failure was observed in 6 other patients. Between month 16 and 18, 2 other patients relapsed when the time between 2 pulses was lengthened. Five patients developed cyclophosphamide-related side-effects: infection (n = 2), amenorrhea (n = 1), alopecia (n = 2) and vomiting (n = 2). Except for one fatal infection, no major side-effect of intravenous cyclophosphamide therapy was observed. At the end of the study, all patients were off intravenous cyclophosphamide therapy with more than 6 months of followup. The 6 responders were in remission on low-dose prednisone or without treatment. CONCLUSION: A combination of high-dose prednisone and intravenous cyclophosphamide may achieve long-term remission in 42% of patients with Wegener's granulomatosis. Responders to intravenous cyclophosphamide therapy had less extensive disease than non-responders.