Conlon P J, Howell D N, Macik G, Kovalik E C, Smith S R
Department of Medicine, Duke University Medical Center, Durham, NC 27710, USA.
Nephrol Dial Transplant. 1995;10(7):1189-93.
Thrombotic thrombocytopenic purpura/Hemolytic uremic syndrome (TTP/HUS) is generally regarded to be a rare disease. The present study was undertaken to identify presenting features, prognostic variables, pathological features and outcome associated with TTP/HUS. The present study is a retrospective chart review of 68 patients treated with plasmapheresis for TTP/HUS at a single tertiary referral medical institution from 1980-1992. The annual number of patients with TTP/HUS treated with plasmapheresis increased from an average of one case per year in 1980 to nine cases per a year in 1992. The in-hospital mortality for patients presenting with TTP/HUS was 25%. Forty four percent of patients presented with an elevated serum creatinine, and 16% required hemodialysis support. Of the seven patients who survived and required hemodialysis support only two patients continued on dialysis. None of the patients presenting with a normal serum creatinine required dialysis at any time in their course. Patient age, sex, presenting platelet count, white blood cell count, hemoglobin level and presence of neurological disease were not significantly associated with death or need for dialysis. The histopathological features of TTP/HUS (fibrin/platelet thrombi in renal vessels and glomeruli, fibrinoid necrosis of vessel walls) were found in all five cases autopsied. The incidence of TTP/HUS may be increasing. Alternative possibilities for the increased frequency of cases seen include greater diagnostic suspicion and referral bias. Despite the use of plasmapheresis, mortality during the initial hospital admission was almost 25%. In retrospect prognosis could not be predicted based on admission biochemical or clinical variables. The majority of patients who developed acute renal failure and survived to hospital discharge recovered renal function and became independent of dialysis.
血栓性血小板减少性紫癜/溶血性尿毒症综合征（TTP/HUS）通常被认为是一种罕见疾病。本研究旨在确定与TTP/HUS相关的临床表现、预后变量、病理特征及结局。本研究是一项回顾性图表审查，涉及1980年至1992年在一家单一的三级转诊医疗机构接受血浆置换治疗的68例TTP/HUS患者。接受血浆置换治疗的TTP/HUS患者年数量从1980年的平均每年1例增加到1992年的每年9例。TTP/HUS患者的住院死亡率为25%。44%的患者血清肌酐升高，16%的患者需要血液透析支持。在存活且需要血液透析支持的7例患者中，只有2例继续接受透析。血清肌酐正常的患者在病程中的任何时候均无需透析。患者的年龄、性别、就诊时血小板计数、白细胞计数、血红蛋白水平及神经疾病的存在与死亡或透析需求均无显著相关性。在所有5例尸检病例中均发现了TTP/HUS的组织病理学特征（肾血管和肾小球内的纤维蛋白/血小板血栓，血管壁的纤维蛋白样坏死）。TTP/HUS的发病率可能正在上升。所观察到的病例数增加的其他可能性包括诊断怀疑度提高和转诊偏倚。尽管使用了血浆置换，但首次住院期间的死亡率仍接近25%。回顾性分析发现，无法根据入院时的生化或临床变量预测预后。大多数发生急性肾衰竭且存活至出院的患者肾功能恢复，不再依赖透析。
