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环孢素诱导一名患有家族性血小板减少症和肠病的患者长期缓解。

Prolonged remission induced by cyclosporine in a patient with familial thrombocytopenia and enteropathy.

作者信息

Beck M N, Kuchler H, Roulet M, Beck D

机构信息

Department of Pediatrics, University Hospital, Lausanne, Switzerland.

出版信息

Pediatr Hematol Oncol. 1995 May-Jun;12(3):289-93. doi: 10.3109/08880019509029571.

Abstract

A 21-year-old patient, since the age of 16, presented a familial type of chronic autoimmune thrombocytopenia that responded only partially to various types of immunosuppressive treatment. His prolonged survival, compared to his decreased siblings, was complicated by the appearance of a severe enteropathy. High doses of corticosteroids induced a type 1 diabetes as a major side effect. The introduction of cyclosporine resulted in both a continuous complete clinical remission and a partial hematological remission and allowed the discontinuation of all other medication for 18 months. In uncommon complex autoimmune disease, cyclosporine may represent a safe and effective alternate therapy when other immunosuppressive agents have failed.

摘要

一名21岁患者,自16岁起患有家族性慢性自身免疫性血小板减少症,对各种类型的免疫抑制治疗仅部分有效。与他患病的兄弟姐妹相比,他的长期存活因严重肠道疾病的出现而变得复杂。高剂量皮质类固醇导致1型糖尿病这一主要副作用。环孢素的使用导致临床持续完全缓解和部分血液学缓解,并使所有其他药物停用18个月。在罕见的复杂自身免疫性疾病中,当其他免疫抑制剂治疗失败时,环孢素可能是一种安全有效的替代疗法。

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