Cermáková Z, Hrdliková R, Suláková T, Korístka M, Kovárová P, Hrachovinová I
Blood Center, Faculty Hospital Ostrava, 17. listopadu 1790, 708 52 Ostrava, Czech Republic.
Prague Med Rep. 2009;110(3):239-44.
Thrombotic thrombocytopenic purpura (TTP) was first described by Eli Moschcowitz in 1924. The pathophysiology of this disease is related to unusual, large multimers of von Willebrand factor in microcirculation, that are insufficiently cleaved by ADAMTS13 protease (a disintegrin-like and metalloprotease with thrombospondin type 1motif,13). Congenital TTP/Upshaw-Schulman syndrome is less frequent than acquired one TTP/HUS (haemolytic-ureamic syndrome). Short characteristic of patients with inherited form of TTP is reported as well as their clinical and laboratory features and management of treatment.
血栓性血小板减少性紫癜（TTP）于1924年由伊莱·莫斯科维茨首次描述。该疾病的病理生理学与微循环中异常的、大的血管性血友病因子多聚体有关，这些多聚体不能被ADAMTS13蛋白酶（一种具有血小板反应蛋白基序的解整合素样金属蛋白酶，13）充分裂解。先天性TTP/舒尔曼综合征比获得性TTP/溶血尿毒综合征（HUS）少见。本文报道了遗传性TTP患者的特点、临床和实验室特征以及治疗管理。
