Fodil Sofiane, Zafrani Lara
Medical Intensive Care Unit, Saint-Louis Hospital, Assistance Publique des Hôpitaux de Paris, University of Paris, 75010 Paris, France.
J Clin Med. 2022 Feb 19;11(4):1103. doi: 10.3390/jcm11041103.
Thrombotic thrombocytopenic purpura (TTP) is a multiorgan disorder. Organ dysfunction occurs as a consequence of widespread microvascular thrombosis, especially in the heart, brain and kidney, causing transient or partial occlusion of vessels, resulting in organ ischemia. Intensive care unit (ICU) admission varies between 40% and 100% of patients with TTP, either because of severe organ failure or in order to initiate emergency plasma exchange (PEx). Severe neurologic manifestations and cardiac involvement have been associated with higher mortality. Acute kidney injury, although usually less severe than that in hemolytic and uremic syndrome, is common during TTP. Initial management in the ICU should always be considered in TTP patients. The current treatment of TTP in the acute phase is based on urgent PEx, combined with corticosteroid therapy, B-cell-targeted immunotherapy, rituximab and inhibition of the interaction between ultra-large Von Willebrand factor multimers and platelets, using caplacizumab, a monoclonal antibody. ICU management permits close monitoring and the rapid introduction of life-sustaining therapies. This review details the epidemiology of TTP in the ICU, organ failures of critically ill patients with TTP, and the initial management of TTP patients in the ICU.
血栓性血小板减少性紫癜（TTP）是一种多器官疾病。器官功能障碍是广泛微血管血栓形成的结果，尤其是在心脏、大脑和肾脏，导致血管短暂或部分阻塞，进而引起器官缺血。40%至100%的TTP患者会入住重症监护病房（ICU），这要么是因为严重器官衰竭，要么是为了开始紧急血浆置换（PEx）。严重的神经系统表现和心脏受累与较高的死亡率相关。急性肾损伤在TTP期间很常见，尽管通常不如溶血尿毒综合征严重。TTP患者在ICU的初始管理应始终被考虑。TTP急性期的当前治疗基于紧急PEx，并联合皮质类固醇治疗、B细胞靶向免疫疗法、利妥昔单抗以及使用单克隆抗体卡泊单抗抑制超大血管性血友病因子多聚体与血小板之间的相互作用。ICU管理允许密切监测并迅速引入维持生命的治疗方法。本综述详细介绍了ICU中TTP的流行病学、TTP重症患者的器官衰竭以及ICU中TTP患者的初始管理。
