AP-HP, Hôpital Saint-Louis Medical ICU, 1 Avenue Claude Vellefaux, 75010, Paris, France.
Intensive Care Med. 2012 Nov;38(11):1810-7. doi: 10.1007/s00134-012-2638-5. Epub 2012 Jul 14.
Although plasma therapy of thrombotic micro-angiopathies (TMAs) has dramatically improved survival, the outcome remains fatal in up to 15 % of patients. We investigated the causes and risk factors of death in patients with TMA.
Retrospective matched case-control national-registry study of 57 patients who died within 180 days of TMA diagnosis and 48 survivors matched on age, gender, and baseline platelet count and creatinine level. The study period was 1995-2007. Factors associated with mortality were identified using a conditional logistic regression model.
Median time from TMA symptom onset to death was 7 (5-14) days. The leading causes of death were nosocomial infections, myocardial infarction, stroke, and pulmonary embolism. Cases and controls did not differ significantly regarding haemolysis parameters, ADAMTS13 activity, or neurological or gastrointestinal involvement. TMA was more frequently related to HIV or cancer in patients who died. Compared to survivors, non-survivors more often had cardiac involvement at diagnosis (38 vs. 6 %, p = 0.03) and less often received plasma exchange therapy (60 vs. 92 %, p = 0.004). Only two factors were independently associated with mortality by multivariate analysis: cardiac involvement at diagnosis (odds ratio, 5.96; 95 % confidence interval, 1.06-33.4) and plasma exchange therapy (odds ratio, 0.25; 95 % confidence interval, 0.06-0.99).
Our data emphasise the adverse prognostic significance of cardiac abnormalities and support routine plasma exchange in patients with TMA. Given the high risk of cardiac and neurological complications, adequate monitoring should be proposed to these patients in appropriate hospital settings.
尽管血栓性微血管病（TMA）的血浆治疗显著提高了生存率，但仍有多达 15%的患者死亡。我们研究了 TMA 患者死亡的原因和危险因素。
这是一项回顾性配对病例对照的全国性登记研究，纳入了 57 名在 TMA 诊断后 180 天内死亡的患者和 48 名年龄、性别、基线血小板计数和肌酐水平相匹配的幸存者。研究期间为 1995 年至 2007 年。使用条件逻辑回归模型确定与死亡率相关的因素。
从 TMA 症状出现到死亡的中位时间为 7（5-14）天。主要死亡原因是医院获得性感染、心肌梗死、中风和肺栓塞。病例组和对照组在溶血参数、ADAMTS13 活性或神经或胃肠道受累方面无显著差异。与存活者相比，死亡者 TMA 更常与 HIV 或癌症相关。与存活者相比，死亡者在诊断时更常伴有心脏受累（38% vs. 6%，p=0.03），较少接受血浆置换治疗（60% vs. 92%，p=0.004）。多因素分析显示，只有两个因素与死亡率独立相关：诊断时的心脏受累（优势比，5.96；95%置信区间，1.06-33.4）和血浆置换治疗（优势比，0.25；95%置信区间，0.06-0.99）。
我们的数据强调了心脏异常的不良预后意义，并支持对 TMA 患者常规进行血浆置换。鉴于心脏和神经系统并发症的高风险，应在适当的医院环境中向这些患者提出适当的监测建议。
