Low-dose cyclosporin for severe aplastic anemia.
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Biomedical subjects
Publications and source records attributed to B C Mehta.
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A randomized double-blind placebo-controlled crossover trial of danazol was carried out in 19 cases of hemophilia A. Danazol was given for 3 months at a dose of 150 mg/day to patients under 15 years of age, and 300 mg/day to older patients. The basal factor VIII:C level was 8.3 +/- 5.6% (mean +/- SD), and after 3 months of danazol treatment was 15.3 +/- 11.0% (p = 0.02). Six patients (basal factor VIII:C 2-22%) showed a 1.36- to 2.87-fold elevation of factor VIII:C levels after danazol. 0/2, 1/4 and 5/13 cases of severe, moderate and mild disease, respectively, responded. Decreases in the number of bleeding episodes and cryoprecipitate requirement were seen in the responders. No adverse reactions to danazol were encountered. Danazol appears to raise the factor VIII:C levels in selected cases of hemophilia A.
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Aplastic crisis as a result of parvovirus infection is seen in a number of haematologic disorders characterized by decreased red cell survival, and leg ulceration due to unknown causes is seen in a number of haemolytic anaemias. Neither of the two has been reported in a case of sideroblastic anemia. We report one case with each of these complications in association with sideroblastic anaemia.
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High dose intravenous gamma globulin has been used in the therapy of immune thrombocytopenia with variable success. Nine non-splenectomized patients with immune thrombocytopenia (8 ITP, 1 Evans syndrome) were treated with IV IgG. One patient with ITP and the case of Evans syndrome had chronic disease, and the others had acute ITP. All patients had been pretreated with steroids with variable response. The patient with chronic ITP and the one with Evans syndrome responded completely to IgG; remission has lasted for 18 months in both. Two patients with acute ITP responded fully to IgG, but one relapsed after 8 months and the other was lost to follow-up after 1 month. Two patients with acute ITP had partial response to IgG, which could be maintained off steroids in one and on a low dose of steroids in the other. Three patients with acute ITP had no response at all to IgG. High dose IV IgG is useful in selected cases of acute as well as chronic ITP, and previous response to steroids increases the likelihood of response. Splenectomy is not a prerequisite for response.
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Acquired inhibitors of blood coagulation factors occur spontaneously or secondarily to various diseases or drug administration. A clinically significant factor VII inhibitor is very rare. We report a patient developing factor VII inhibitor possibly as a reaction to penicillin administration; it gave rise to fatal haemorrhage.
145 patients were diagnosed to have hereditary spherocytosis (HS) over a period of 25 years. Jaundice (66.9%), fever (65.5%), weakness (44.8%), and abdominal pain (35.8%) were the commonest complaints. 94.5% had splenomegaly (JP-17 cm) and 71.7% had hepatomegaly (JP-6 cm). Spherocytes were detected in the peripheral smears of all patients at presentation on careful examination. 67 patients had been investigated elsewhere and spherocytes missed in 86.6%. Gall stones were seen in 20 of the 54 patients investigated. Family history suggestive of HS was available in only 16.6% of cases, whereas examination and investigations revealed HS in almost all families. Splenectomy was done in all symptomatic patients. In the 39 patients followed up for 1-9 years after splenectomy.
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