Pene Frédéric, Vigneau Cécile, Auburtin Marc, Moreau Delphine, Zahar Jean-Ralph, Coste Joël, Heshmati Farhad, Mira Jean-Paul
Medical Intensive Care Unit Department, Cochin Hospital, Assistance Publique-Hôpitaux de Paris, 27 rue du faubourg Saint-Jacques, 75679 Paris, France.
Intensive Care Med. 2005 Jan;31(1):71-8. doi: 10.1007/s00134-004-2505-0. Epub 2004 Dec 3.
Thrombotic microangiopathies, namely thrombotic thrombocytopenic purpura and hemolytic uremic syndrome, are uncommon microvascular occlusive diseases. Despite the dramatic improvement in the outcome by exogenous plasma supply, either through plasma infusion or through plasma exchange, patients frequently require support in the intensive care unit. In the present study, we evaluated the outcome of a large cohort of patients with severe thrombotic microangiopathies.
A retrospective multicenter study from January 1998 to June 2001.
Fourteen French university hospital medical intensive care units.
Sixty three adult patients with severe thrombotic microangiopathies.
Of the 63 patients, 19 had a clinical presentation of thrombotic thrombocytopenic purpura, 18 had hemolytic uremic syndrome and 26 had combined neurologic and renal failures. Infections were the main etiology associated with thrombotic microangiopathies. The mortality rate was 35%. Of the survivors, all achieved complete remission. Whereas neurologic failure assessed through the Glasgow coma scale was an independent predictor of mortality [HR=0.845 (CI 95%: 0.759-0.940), P=0.002], renal impairment did not appear to be an adverse prognostic factor. The use of plasma exchange was independently associated with survival [HR=0.269 (CI 95%: 0.104-0.691), P=0.006].
Thrombotic microangiopathies with severe organ dysfunctions leading to hospitalization in the intensive care unit are associated with high mortality. Neurologic impairment appears to be the main adverse prognostic factor correlated to mortality, and the study confirms the importance of plasma exchange in the treatment of high-risk patients.
血栓性微血管病，即血栓性血小板减少性紫癜和溶血尿毒综合征，是罕见的微血管闭塞性疾病。尽管通过输注血浆或血浆置换外源性补充血浆可显著改善预后，但患者仍经常需要在重症监护病房接受支持治疗。在本研究中，我们评估了一大群重症血栓性微血管病患者的预后情况。
一项1998年1月至2001年6月的回顾性多中心研究。
法国14家大学医院的医学重症监护病房。
63例患有重症血栓性微血管病的成年患者。
63例患者中，19例临床表现为血栓性血小板减少性紫癜，18例为溶血尿毒综合征，26例合并神经和肾衰竭。感染是与血栓性微血管病相关的主要病因。死亡率为35%。幸存者均实现完全缓解。通过格拉斯哥昏迷量表评估的神经功能衰竭是死亡率的独立预测因素[风险比=0.845（95%置信区间：0.759 - 0.940），P = 0.002]，而肾功能损害似乎不是不良预后因素。血浆置换的使用与生存独立相关[风险比=0.269（95%置信区间：0.104 - 0.691），P = 0.006]。
伴有严重器官功能障碍导致需入住重症监护病房的血栓性微血管病死亡率很高。神经功能损害似乎是与死亡率相关的主要不良预后因素，且该研究证实了血浆置换在高危患者治疗中的重要性。
