Pereira A, Mazzara R, Monteagudo J, Sanz C, Puig L, Martínez A, Ordinas A, Castillo R
Service of Hemotherapy and Hemostasis, Hospital Clínico, Barcelona, Spain.
Ann Hematol. 1995 Jun;70(6):319-23. doi: 10.1007/BF01696619.
The aim of this study was to investigate pretreatment prognostic factors that could be useful in predicting the response to plasma exchange in thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS). Thirty-two patients with TTP/HUS, treated with plasma exchange at our institution from 1980 to 1994, were studied. The main clinical and laboratory data at the beginning of plasma exchanges were analyzed by the Cox stepwise logistic regression, applied to either treatment failure or death. Seventeen (53%) patients attained a complete remission and 22 (69%) survived (five in advanced renal failure and long-term hemodialysis). Longer delay in initiating plasma exchanges, presence of stupor or coma, and higher creatinine levels at the beginning of plasma exchanges were independent predictors of treatment failure. Stupor or coma at the beginning of plasma exchanges was the only predictor of mortality from unremitted TTP/HUS. Hemoglobin levels, platelet count, and LDH activity, traditionally envisaged as markers of disease activity, neither correlated with previous duration of TTP/HUS nor had any prognostic value. Early diagnosis of TTP/HUS and prompt initiation of intensive plasma exchange emerged from this study as the most effective interventions for improving the prognosis of TTP/HUS patients.
本研究的目的是调查在血栓性血小板减少性紫癜/溶血尿毒综合征（TTP/HUS）中，有助于预测血浆置换反应的预处理预后因素。对1980年至1994年在我院接受血浆置换治疗的32例TTP/HUS患者进行了研究。通过Cox逐步逻辑回归分析血浆置换开始时的主要临床和实验室数据，将其应用于治疗失败或死亡情况。17例（53%）患者实现完全缓解，22例（69%）存活（5例处于晚期肾衰竭并长期接受血液透析）。开始血浆置换的时间延迟较长、存在木僵或昏迷以及血浆置换开始时肌酐水平较高是治疗失败的独立预测因素。血浆置换开始时的木僵或昏迷是TTP/HUS未缓解导致死亡的唯一预测因素。传统上被视为疾病活动标志物的血红蛋白水平、血小板计数和乳酸脱氢酶活性，既与TTP/HUS之前的病程无关，也没有任何预后价值。本研究表明，TTP/HUS的早期诊断和及时开始强化血浆置换是改善TTP/HUS患者预后的最有效干预措施。
