血栓性血小板减少性紫癜18例临床分析

目的:了解血栓性血小板减少性紫癜(TTP)的临床特征及治疗,以加深对该病的认识。方法:对我院1998年6月～2005年10月诊断的18例TTP的类型、临床表现、治疗及预后进行回顾性分析。结果:患者多为女性;临床主要表现TTP、微血管性溶血性贫血和神经系统症状;血小板计数和血红蛋白减低;血清乳酸脱氢酶明显升高;凝血功能基本正常;血浆置换和免疫抑制治疗有效;复发率40%,再次治疗有效。结论:TTP是一种女性多见的微血管血栓-出血综合征,血管性血友病因子裂解蛋白酶(ADAMTS13)活性降低参与了TTP的发生,应根据TTP的类型选择不同的治疗方案以提高疗效、减少复发。

The clinical analysis of 18 patients with thrombotic thrombocytopenic purpura

Objective:To study the clinical characteristics and treatment of thrombotic thrombocytopenic purpura(TTP).Method:Clinical and laboratory characteristics of 18 cases with thrombotic thrombocytopenic purpura admitted to our hospital between June 1998 and October 2005 were analyzed retrospectively.Result:There were more female patients than male patients.Characterized by microangiopathic hemolytic anemia,thrombocytopenia,neurological abnormalities and elevated serum LDH value.Clotting studies were usually normal.Plasma exchange therapy and immunosuppressive treatment are valid.The relapse rate in survivors of TTP was 40%.They acquired remission again with the same therapy.Conclusion:TTP is a severe microangiopathy.The incidence of TTP is higher in female.Decrease of activity of a plasma metalloprotease named ADAMTS13 is shown to cause many cases of TTP.Management decision should depend upon the type of TTP in order to achieve complete remission and low relapse rate.