Roberts A W, Gillett E A, Fleming S J
Department of Hematology, Royal Brisbane Hospital, Queensland, Australia.
J Clin Apher. 1991;6(3):150-4. doi: 10.1002/jca.2920060305.
A retrospective analysis was made of 14 consecutive adults with hemolytic uremic syndrome/thrombotic thrombocytopenic purpura (HUS/TTP) who were treated with plasma exchange (PE). In six patients the disease was primary, whilst in eight HUS/TTP was considered secondary to an associated condition. Thirteen patients had renal involvement, six had central nervous system symptoms or signs, three had fever, and one had myocardial damage. Twelve patients (86%) recovered. Four of these relapsed, but responded to further treatment. Two patients failed to respond and died. Hematological response occurred rapidly in survivors: thrombocytopenia resolved after a median of four exchanges, and hemolysis after a median of six. Four patients had a complete recovery, seven had residual mild end organ damage, and one had severe renal impairment. PE was an effective treatment for both primary and secondary HUS/TTP. The platelet count proved to be the earliest indicator of clinical outcome. Continuing follow-up of survivors is required because of the risk of relapse and the high incidence of end organ damage.
对连续14例接受血浆置换（PE）治疗的溶血性尿毒症综合征/血栓性血小板减少性紫癜（HUS/TTP）成年患者进行了回顾性分析。6例患者的疾病为原发性，而8例HUS/TTP被认为继发于相关疾病。13例患者有肾脏受累，6例有中枢神经系统症状或体征，3例有发热，1例有心肌损伤。12例患者（86%）康复。其中4例复发，但对进一步治疗有反应。2例患者无反应并死亡。幸存者的血液学反应迅速：血小板减少症在中位4次置换后得到缓解，溶血在中位6次置换后得到缓解。4例患者完全康复，7例有残留的轻度终末器官损伤，1例有严重肾功能损害。PE对原发性和继发性HUS/TTP均为有效治疗方法。血小板计数被证明是临床结果的最早指标。由于存在复发风险和终末器官损伤的高发生率，需要对幸存者进行持续随访。
