血栓性血小板减少性紫癜12例临床分析

Clinical Analysis of 12 Patients with Thrombotic Thrombocytopenic Purpura

目的为了提高对血栓性血小板减少性紫癜(TTP)的认识,对12例TTP患者的临床表现、实验室特点、治疗和转归等资料进行了回顾性分析。结果在12例TTP患者中,10例有诱发因素(妊娠1例和感染6例,3例为系统性红斑狼疮)。12例患者在起病时或病程中均有不同程度的神经精神症状、coombs试验阴性的溶血性贫血和血小板减低;11例患者有不同程度的不规则发热,以中等度发热为主;10例患者有肾脏损害,均出现蛋白尿,其中7例合并肾功能异常;在12例患者中5例血浆血管性血友病因子裂解酶(ADAMTsl3)活性明显减低(均低于10%)。经过血浆置换、肾上腺糖皮质激素和利妥昔单克隆抗体等治疗后,10例患者完全缓解,但2例半年复发,2例死亡。结论 TTP是由于血小板性微血栓形成而造成全身多系统、多脏器功能障碍的一种血栓性微血管病,临床过程凶险,死亡率极高,早期诊断和以血浆置换为主要手段的早期治疗可大大改善患者的预后。

Objective In order to enhance understanding of thrombotic thrombocytopenic purpura （TFP） ,the clinical features, laboratory characteristics, treatment and outcome of 12 patients with TTP were retrospectively analyzed and investigated. Results The results showed that 10 out of 12 patients with TTP had predisposing factors, such as pregnancy in 1 cases, infection in 6 cases, systemic lupus erythematosus （SLE） in 3 case. Twelve patients all had neuropsychological symptoms, hemolytic anemia with negative--Coombs test, and decreased platelet counts. Eleven patients had irregular fever with different degree. There were 10 patients with kidney damage including proteinuria in 10 cases and renal function abnormalities in 7 cases. The Von Willebrand factor- cleaving protease （ vWF- CP, ADAMTS13 ） activity of 5 cases out of 12 patients significantly decreased （1 ess than 10% ）. After treatment with plasma exchange, glucocorticoid and rituximab so on, 10 ca- ses achieved complete remission, in which 2 cases relapsed in six months. Two patients died at last. Conclusion It is concluded that TFP is a kind of thrombotic mictioangiopathy due to platelet micro- thrombosis involved in multiple systems and multiple organs dysfunction with dangerous clinical process. The mortalily of TTP patients is Very high. Early diagnosis and early treatment with plasma exchange as the main means can greatly improve the prognosis of patients with TTP.