Wang Yan, Zhang Jing-Yu, Niu Zhi-Yun, Lin Feng-Ru, Zhou Jian-Hui
Department of Hematology, The Second Hospital of Hebei Medical University, Hebei Province Key Laboratory of Blood Disease, Shijiazhuang 050000, Hebei Province, China.
Department of Hematology, The Second Hospital of Hebei Medical University, Hebei Province Key Laboratory of Blood Disease, Shijiazhuang 050000, Hebei Province, China. E-mail:
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2014 Apr;22(2):407-11. doi: 10.7534/j.issn.1009-2137.2014.02.026.
In order to enhance the understanding of thrombotic thrombocytopenic purpura (TTP), 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.
为提高对血栓性血小板减少性紫癜（TTP）的认识，回顾性分析和研究14例TTP患者的临床特点、实验室特征、治疗及转归。结果显示，14例TTP患者中7例有诱发因素，其中妊娠4例、感染3例、系统性红斑狼疮（SLE）1例、造血干细胞移植（HSCT）1例。14例患者均有神经精神症状、Coombs试验阴性的溶血性贫血及血小板计数减少。8例患者有不同程度的不规则发热。8例患者有肾脏损害，其中蛋白尿8例，肾功能异常4例。14例患者中13例的血管性血友病因子裂解蛋白酶（VWF-CP，ADAMTS13）活性显著降低（低于10%）。同时，在这13例ADAMTS13活性降低的患者中，12例检测到血浆ADAMTS13抑制剂。经血浆置换、糖皮质激素、利妥昔单抗等治疗后，12例患者达到完全缓解，其中8例在两年内复发。最终2例患者死亡，其中1例继发于HSCT。结论：TTP是一种因血小板微血栓形成导致多系统、多器官功能障碍的血栓性微血管病，临床过程凶险，TTP患者死亡率很高。早期诊断并以血浆置换为主要手段进行早期治疗可大大改善TTP患者的预后。
