Expert statement on the ICU management of patients with thrombotic thrombocytopenic purpura
Expert statement on the ICU management of patients with thrombotic thrombocytopenic purpura
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DOI：
10.1007/s00134-019-05736-5
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发表时间：
2019-11-01
影响因子：
38.9
通讯作者：
Van de Louw, Andry
中科院分区：
文献类型：
--
作者：
Azoulay, Elie;Bauer, Philippe R.;Van de Louw, Andry
血栓性血小板减少性紫癜（TTP）如果不及时治疗，90%的患者是致命的，必须早期诊断以优化患者结局。然而，TTP的发生率非常低，这是制定循证临床实践建议的一个障碍，各中心生存率的差异很大，部分原因可能是由于指导不足导致的管理策略差异。因此，我们制定了一份专家声明，为TTP危重患者的管理提供值得信赖的指导。由于很难在文献中找到强有力的证据，因此无法报告专家之间就大多数项目达成共识的情况。鉴于TTP治疗的最新进展，例如利妥昔单抗和最近获得许可的药物caplacizumab的使用，如果管理策略的其他组成部分遵循标准化模式，则其益处将最大化，因此该专家声明是及时的。最后，未回答的问题被确定为未来的研究主题TTP。
Thrombotic thrombocytopenic purpura (TTP) is fatal in 90% of patients if left untreated and must be diagnosed early to optimize patient outcomes. However, the very low incidence of TTP is an obstacle to the development of evidence-based clinical practice recommendations, and the very wide variability in survival rates across centers may be partly ascribable to differences in management strategies due to insufficient guidance. We therefore developed an expert statement to provide trustworthy guidance about the management of critically ill patients with TTP. As strong evidence was difficult to find in the literature, consensus building among experts could not be reported for most of the items. This expert statement is timely given the recent advances in the treatment of TTP, such as the use of rituximab and of the recently licensed drug caplacizumab, whose benefits will be maximized if the other components of the management strategy follow a standardized pattern. Finally, unanswered questions are identified as topics of future research on TTP.