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[环孢素治疗难治性埃文斯综合征的疗效]

[Effective cyclosporine therapy to resistant-Evans syndrome].

作者信息

Tsudo M, Moriguchi T

机构信息

Internal Medicine, Kyoto-Katsura Hospital.

出版信息

Rinsho Ketsueki. 1996 Nov;37(11):1289-92.

PMID:8960663
Abstract

Evans syndrome is a rare disease defined as autoimmune hemolytic anemia plus immune thrombocytopenia. We describe a 57-year-old man with Evans syndrome whose thrombocytopenia was refractory to conventional therapy, including prednisolone, danazol, azathiopurine, cyclophosphamide, vincristine, gamma-globulin and splenectomy. The patient was then treated with three cycles of pulsed high-dose dexamethasone (40 mg/day for 4 sequential days every 4 weeks) followed by cyclosporine A therapy (300 mg/day). The platelet counts dramatically increased from 2,000/microliter to 200,000/microliter. Although thrombocytopenia appeared again with the tapering of cyclosporine A, the platelet counts recovered by re-increase of cyclosporine A, and have remained above 100,000/microliter for more than 14 months. Treatment with cyclosporine A can provide an effective therapeutic choice for refractory Evans syndrome.

摘要

伊文斯综合征是一种罕见疾病,定义为自身免疫性溶血性贫血合并免疫性血小板减少症。我们描述了一名患有伊文斯综合征的57岁男性,其血小板减少症对包括泼尼松龙、达那唑、硫唑嘌呤、环磷酰胺、长春新碱、γ-球蛋白和脾切除术在内的传统治疗均无效。该患者随后接受了三个周期的脉冲式大剂量地塞米松治疗(每4周连续4天,每天40毫克),随后接受环孢素A治疗(每天300毫克)。血小板计数从2000/微升急剧增加到200000/微升。尽管随着环孢素A逐渐减量血小板减少症再次出现,但通过再次增加环孢素A,血小板计数得以恢复,并且在超过14个月的时间里一直保持在100000/微升以上。环孢素A治疗可为难治性伊文斯综合征提供一种有效的治疗选择。

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