血栓性血小板减少性紫癜14例临床分析  

Clinical Analysis of 14 Patients with Thrombotic Thrombocytopenic Purpura

作　　者：王艳[1] 张敬宇[1] 牛志云[1] 林凤茹[1] 周建辉[2] 

机构地区：[1]河北医科大学第二医院血液科、河北省血液病重点实验室,河北石家庄050000 [2]解放军总医院肾科,北京100853

出　　处：《中国实验血液学杂志》2014年第2期407-411,共5页Journal of Experimental Hematology

摘　　要：为了提高对血栓性血小板减少性紫癜(TTP)的认识,对14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。结果表明:在14例TTP患者中,7例有诱发因素(妊娠4例和感染3例;1例为系统性红斑狼疮,1例为造血干细胞移植后患者)。14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;8例患者有不同程度的不规则发热,以中等度发热为主;8例患者有肾脏损害,均出现蛋白尿,其中4例合并肾功能异常;在14例患者中13例血浆血管性血友病因子裂解酶(ADAMTS13)活性明显减低(均低于10%),其中12例患者同时存在ADAMTS13抑制物。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,12例患者完全缓解,但8例2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。In order to enhance the understanding of thrombotic thrombocytopenic purpura （TIP）, the clinical features, laboratory characteristics, treatment and outcome of 14 patients with TTP were retrospectively analyzed and investigated. The results showed that 7 out of 14 patients with TTP had predisposing factors, such as pregnancy in 4 cases, infection in 3 cases, systemic lupus erythematosus （SLE） in 1 case and hematopoietic stem cell transplantation （HSCT） in 1 case. Fourteen patients all had neuropsychological symptoms, hemolytic anemia with negative-Coombs test, and decreased platelet counts. Eight patients had irregular fever with different degree. There were 8 patients with kidney damage including proteinuria in 8 cases and renal function abnormalities in 4 cases. The von Willebrand factor- cleaving protease （VWF-CP, ADAMTS13 ） activity of 13 cases out of 14 patients significantly decreased （less than 10% ）. At same time, plasma ADAMTS13 inhibitors were detected in 12 cases out of these 13 patients with decreased ADAMTS13 activity. After treatment with plasma exchange, glucocorticoid and rituximab so on, 12 cases achieved complete remission, in which 8 cases relapsed in two years. Two patients died at last, in which one case was secondary to HSCT. It is concluded that TTP is a kind of thrombotic microangiopathy due to platelet microthrombosis involved in multiple systems and multiple organs dysfunction with dangerous clinical process. The mortality 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.

关 键 词：血栓性血小板减少性紫癜 血管性血友病因子裂解酶 血浆置换 

分 类 号：R554.6[医药卫生—血液循环系统疾病]