Tsudo M, Moriguchi T
Internal Medicine, Kyoto-Katsura Hospital.
Rinsho Ketsueki. 1996 Nov;37(11):1289-92.
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.
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。
