目的 探讨血栓性血小板减少性紫癜(TTP)的临床实验室特征及治疗转归,旨在提高TTP的诊疗水平.方法 对19例TTP患者的临床资料进行回顾性分析.结果 TTP急性起病,首发症状以皮肤出血与神经症状多见.确诊时血小板减少、微血管病性溶血性贫血、神经异常"三联征"者占94.7%,三联征伴发热及肾脏病变"五联征"者占42.1%.检验指标血清乳酸脱氢酶和破碎红细胞计数分别为1 844(654~6 725)U/L和5.7(2.0~14.4)%,均有所增高.血浆置换(PE)联合糖皮质激素治疗的有效率为85.7%,早期死亡率为14.3%,复发率为33.3%.复发者加用环孢素A和(或)长春新碱后再次完全缓解.结论 TTP女性多见、多无诱因,"三联征"为其突出表现,血清乳酸脱氢酶及破碎红细胞计数升高具有重要提示意义,PE联合糖皮质激素是治疗TTP的有效方法,复发者应选择细胞毒类或烷化剂等免疫抑制剂强化治疗.%Objective To investigate clinical and laboratory features of thrombotic thrombocytopenic purpura (TTP) and its prognosis in order to improve the diagnosis and treatment of TTP. Methods Clinical data of 19 TTP patients were retrospectively analyzed. Results The male to female ratio of TTP patients was 1: 1. 71 with a median age of 45 years. 84. 2% was idi-opathic TTP, which had acute onset and initial symptoms of dermatorrhagia and neurological symptoms. When diagnosed, patients with triad symptoms of thrombocytopenia, microangiopathic hemolytic anemia and neurological abnormality accounted for 94. 7% , and patients with pentad symptoms (triad symptoms combined with fever and renal lesions) accounted for 42. 1 %. Test indicators of serum lactate dehydrogenises and schistocytes of peripheral blood smears were 1 844 (654 ~ 6 725) U/L and 5. 7 (2. 0 ~ 14. 4)% respectively, both increased slightly. The overall response of plasma exchange (PE) combined with corticosteroids was 85. 7% , with early mortality rate of 14. 3% and relapse rate of 33. 3%. The relapsed patients completely remitted after receiving additional cyclosporine A and (or) vincristine. Conclusion TTP is common in female, most lacking causes but with triad symptoms of thrombocytopenia, microangiopathic hemolytic anemia and neurologic abnormalities. The increased serum lactate dehydrogenises and schistocytes counts are of great significance for diagnosis. PE combined with corticosteroids is an effective treatment for TTP, but immunosuppressive therapy with cytotoxic and alkylating agents should be added for relapsed patients.
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