Aul C, Scharf R E, Königshausen T, Schneider W
Klin Wochenschr. 1985 Feb 4;63(3):123-32. doi: 10.1007/BF01734250.
Thrombotic thrombocytopenic purpura (TTP) is an uncommon disorder which usually occurs in young adults. It is characterized by a pentad of clinical findings: fever, neurological abnormalities, renal dysfunction, microangiopathic hemolytic anemia and thrombocytopenia. The histological hallmark is the presence of platelet thrombi occluding the microcirculation of multiple organs. The etiology and pathogenesis of disseminated platelet aggregation are uncertain and obviously not uniform in individual patients. Experimental findings suggest that microthrombi may result from intravascular platelet activation or form secondarily at sites of vessel wall damage. The differential diagnosis of TTP includes the hemolytic uremic syndrome in which the microangiopathic changes are exclusively found in the kidneys. When untreated, TTP invariably runs a progressive and fatal course. In recent years, prognosis has been improved by new forms of therapy such as plasmapheresis or infusions of fresh frozen plasma which may lead to recovery in about 80% of patients.
血栓性血小板减少性紫癜（TTP）是一种罕见的疾病，通常发生于年轻人。其临床特征为五联征：发热、神经功能异常、肾功能不全、微血管病性溶血性贫血和血小板减少。组织学特征是存在阻塞多个器官微循环的血小板血栓。弥散性血小板聚集的病因和发病机制尚不确定，且在个体患者中明显不一致。实验结果表明，微血栓可能源于血管内血小板激活，或继发于血管壁损伤部位。TTP的鉴别诊断包括溶血性尿毒症综合征，后者的微血管病性改变仅见于肾脏。若不治疗，TTP必然会呈进行性发展并导致死亡。近年来，通过血浆置换或输注新鲜冷冻血浆等新型治疗方法，预后有所改善，约80%的患者可能康复。
