IMPROVED SURVIVAL IN THROMBOTIC THROMBOCYTOPENIC PURPURA HEMOLYTIC UREMIC SYNDROME - CLINICAL-EXPERIENCE IN 108 PATIENTS
IMPROVED SURVIVAL IN THROMBOTIC THROMBOCYTOPENIC PURPURA HEMOLYTIC UREMIC SYNDROME - CLINICAL-EXPERIENCE IN 108 PATIENTS
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DOI：
10.1056/nejm199108083250605
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发表时间：
1991-08-08
影响因子：
158.5
通讯作者：
KICKLER, TS
中科院分区：
文献类型：
--
作者：
BELL, WR;BRAINE, HG;KICKLER, TS
背景和方法。血栓性血小板减少性紫癜-溶血性尿毒综合征（TTP-HUS）以微血管病性溶血性贫血、血小板减少、发热、中枢神经系统异常和肾功能不全为特征。在早期的报告中，死亡率接近100%。1979年，针对诊断为TTP-HUS的约翰霍普金斯医院收治的患者引入了治疗方案。治疗方案包括泼尼松200 mg/d，对于症状轻微且无中枢神经系统症状的患者，以及泼尼松加血浆置换，对于临床症状迅速恶化且单用泼尼松48小时后仍未改善的患者，以及出现中枢神经系统症状且红细胞压积值和血小板计数迅速下降的患者。共有108名患者接受了治疗，91%的患者存活。单用泼尼松对30例轻度TTP-HUS患者有效（2例复发和2例死亡）。对78例合并TTP-HUS患者采用血浆置换加强的松治疗，67例复发，8例死亡。36例接受维持性血浆输注的患者中有22例复发。对于血浆置换反应较差的患者，脾切除术或阿司匹林和双嘧达莫治疗均无效。71名受试者中无一例O 157：H7大肠杆菌培养阳性。9%的患者妊娠，无一例分娩TTP-HUS婴儿。TTP-HUS患者可获得91%存活率的有效治疗。
Background and Methods. Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, central nervous system abnormalities, and renal dysfunction. In early reports the mortality approached 100 percent. A treatment protocol was introduced in 1979 for patients admitted to Johns Hopkins Hospital with the diagnosis of TTP-HUS. Treatment regimens included 200 mg of prednisone a day, for patients with minimal symptoms and no central nervous system symptoms, and prednisone plus plasma exchange, for patients with rapid clinical deterioration who did not improve after 48 hours of prednisone alone and for patients presenting with central nervous system symptoms and rapidly declining hematocrit values and platelet counts.Results. A total of 108 patients were treated, and 91 percent survived. Prednisone alone was judged to be effective in 30 patients with mild TTP-HUS (2 relapses and 2 deaths). Plasma exchange plus prednisone was given to 78 patients with complicated TTP-HUS, resulting in 67 relapses and 8 deaths. Relapses occurred in 22 of 36 patients given maintenance plasma infusions. Neither splenectomy nor treatment with aspirin and dipyridamole was effective in those with a poor response to plasma exchange. None of the 71 patients tested had positive cultures for O157:H7 Escherichia coli. Nine percent of the patients were pregnant, and none gave birth to infants with TTP-HUS.Conclusions. Effective treatment with 91 percent survival is available for patients with TTP-HUS.