获得性血栓性血小板减少性紫癜临床分析 - 内科急危重症杂志.doc

非典型血栓性血小板减少性紫癜临床分析 深圳市南山区人民医院 卢博 刘泽林* 王欣 金梦迪 深圳 518052 摘要 目的。方法回顾性分析我院收治的7例非典型TTP患者的临床资料。结果7例TTP患者，初诊时均无典型五联征，但均具备血小板减少及微血管病性溶血二联症。诊断确立后予血浆置换联合糖皮质激素，。1例死亡。2例复发患者予利妥昔单抗治疗，均完全缓解结论TTP患者早期快速诊断、一经确立诊断立即应用血浆置换对降低TTP患者的死亡率至关重要，联合糖皮质激素及CD20单抗治疗可以进一步降低TTP患者的死亡率及复发率。 R558 　　　文献标识码　A Clinical Analysis of atypical Thrombotic Thrombocytopenic Purpura LU Bo, Jin Mengdi, Wang Danyu, Wang Xin, Cui Haiyan, Liu Zelin. Shenzhen Nanshan people’s Hospital, Shenzhen 518052, China Abstract Objective: To make a further understanding of diagnose and treatment of TTP. Methods : A retrospective survey of 7 atypical TTP patients confirmed in our hospital. Results : No one of the 7 cases have typical five clinical manifestation at initial stage, but all have thrombocytopenia and microangiopathic hemolytic. The ratio of schistocytes on peripheral blood smear was between 2.0% - 6.7%, with a median ratio of 5%. These patients were treated with a combination therapy of plasma exchange and steroids immidiately once the diagnosis was established. 6 cases had response to treatment, 1 cases was died. Two cases of relapsed patients had been treated with rituximab, both were complete remission. Conclusion: Early detection, rapid diagnosis and immediate treatment with plasma exchange is crucial to reduce mortality in patients with TTP. combination with steroids and CD20 monoclonal antibody may further reduce mortality and relapse rate. Key words atypical thrombotic thrombocytopenic purpura clinical characteristic plasma exchange rituximab 血栓性血小板减少性紫癜（Thrombotic thrombocytopenic purpura，TTP）是一种以血小板减少和微血管病性溶血性贫血为特点的血液学急症发病率低、诊断疑难，死亡率高血栓性微血管病（Thrombotic Microangiopathies TMA）TTP分为遗传性和获得性两种。遗传性TTP是ADAMTS13基因突变导致血管性血友病因子裂解酶活性降低或缺乏所致，常在感染、应激或妊娠等诱发因素作用下发病[1]。获得性TTP根据有无原发病分为原发性和继发性。原发性TTP无明显诱因，多因患者体内存在抗ADAMTS13自身抗体（抑制物），导致ADAMTS13活性降低或缺乏[2]，是主要临床类型。继发性TTP常因感染、药物、肿瘤、自身免疫性疾病、造血干细胞移植等因素诱发，发病机制复杂，预后不佳[3]。TTP患者的早期诊断及时治疗对TTP的预后具有决定性作用。本研究通过回顾性分析7例TTP患者，总结其临床特点，为临床早期诊断、及时治疗提供帮助。资料与方法 临床资料：我院于2010年至2014年共收治7例TTP患者，其中男性为2