血浆置换及免疫抑制剂联合美罗华治疗血栓性血小板减少性紫癜临床观察

Clinical Observation of Plasma Exchange and Immunosuppressive Agents combined with Rituximab in the Treatment of Thrombotic Thrombocytopenic Purpura

目的:分析血栓性血小板减少性紫癜的临床特点、治疗及转归。方法:回归性分析某院2006年1月~2016年4月30例血栓性血小板减少性紫癜患者,分析及比较病因、比较血浆置换及免疫抑制剂(激素或环孢素)联合美罗华治疗疗效、转归、随访情况。结果:30例患者中男性12例,女性18例。30例(100%)均有血小板减少,24例(80%)有神经系统表现,23例(76.67%)有微血管性溶血,11例(36.67%)有发热,12例(40%)有肾脏损害;有5例(16.67%)明确合并自身免疫疾病,3例(10%)合并妊娠,4例(13.33%)合并感染,2例(6.67%)检查为肿瘤,16例(53.33%)不明原因。对照组4例(26.67%)无效死亡,环孢素组1例(16.67%)死亡,美罗华组(100%)全部存活,3组治疗效果之间无明显统计学差异。有效随访22例中1例确诊为肿瘤,5例(22.73%)复发:3例(13.64%)为对照组,2例(9.09%)为环孢素组,均在停用激素或环孢素后复发,复发后血浆置换仍有效;3例妊娠患者终止妊娠后未再复发。结论:早诊断早给予血浆置换是降低TTP高死亡率的关键,血浆置换联合糖皮质激素及美罗华比血浆置换联合激素或环孢素治疗的疗效稍好。该病易复发,维持治疗或控制原发病是关键,美罗华对降低复发率有一定的帮助。

Objective： To analyze the clinical characteristics, treatment and prognosis of thrombotic thrombocytopenic purpura （ITP）. Methods： Regressively analyzed 30 cases of patients in a hospital from January 2006 to April 2006 with thrombotic thrombocytopenic purpura, etiology, plasma exchange and the clinical characteristics, treatment and prognosis of immunosuppressive agents （Cyclosporin A or glucocorticoid） combined with rituximab were compared and analyzed. Results： There were 12 males and 18 females in TTP,30 cases （100%） had thrombocytopenia, 24 cases （80%） had neurological symptoms, 23 cases （ 76.67% ） had microangiopathic hemolytic, 11 cases （36.67%） had fever. 12 cases （40%） with renal damage. 5 cases （16.67%） diagnosed complicated with autoimmune diseases, 3 cases of pregnancy （10%）, 4 cases （13.33%） complicated with infection, 2 cases （6.67%） check for tumor, 16 cases （53.33%） with unknown causes. The control group plasma replacement in 4 cases （26.67%） died, 1 cases of CSA group （ 16.67% ） died, rituximab （100%） group all survived. There was no significant difference between the treatment effect of the three groups. In 22 cases of effectively following-up,1 patient was diagnosed tumor, and the recurrence occurred in 5 cases （ n =22.73%）：3 cases in the control group （ n =13.64%）,2 cases in CSA group （ n =9.09%）, All patients relapse after hormone withdrawal or cyclosporin withdrawal. Plasma exchange is still effective after relapse. 3 cases of pregnancy after termination of pregnancy without recurrence. Conclusion： Early diagnosis of plasma exchange therapy is the key of reducing the mortality of TTP. The effect of plasma exchange combined with glucocorticoid and rituximab than plasmapheresis combined with corticosteroids or cyclosporine treatment is slightly better, but the disease is easy to relapse, maintenance treatment or control of the primary disease is the key. To reduce the recurrence rate, rituximab is of some help.