Mariotte Eric, Veyradier Agnès
aService de Réanimation médicale, Hôpital Saint-Louis bService d'Hématologie biologique, Hôpital Lariboisière, Paris, France.
Curr Opin Crit Care. 2015 Dec;21(6):593-601. doi: 10.1097/MCC.0000000000000255.
Thrombotic thrombocytopenic purpura (TTP) is a rare but challenging disease for intensive care specialists. Patients with acute TTP frequently require admission to the intensive care unit because of organ dysfunctions due to the disease or because of the risk of sudden aggravation at the onset of the disease. This review aims at describing recent evolutions in the diagnosis and for the management of TTP for the use of intensive care specialists.
The use of A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats (ADAMTS13) activity along with clinico-biological features to define TTP by most researchers' teams has led to easier interpretation of the literature. The main issues in TTP treatment in 2015 remain the indication and timing of introduction of anti-CD20 antibody rituximab for the treatment of inaugural TTP and the preemptive use of rituximab in asymptomatic patients with decreasing ADAMTS13 activity.
The classification of thrombotic microangiopathies has evolved from a clinical to a pathophysiological definition. TTP is characterized by a severe ADAMTS13 deficiency that can be documented in vitro, along with anti-ADAMTS13 antibodies in most adult cases. Plasmapheresis and immunosuppressive therapy with steroids remain the standard of care for acute inaugural TTP. Anti-CD20 monoclonal antibody rituximab is safe and indicated in relapsing and/or refractory TTP. Its indication in inaugural TTP remains to be evaluated but is nevertheless recommended by experts. Novel therapies for TTP are still in preclinical phases.
血栓性血小板减少性紫癜（TTP）对重症监护专家而言是一种罕见但颇具挑战性的疾病。急性TTP患者常因疾病导致的器官功能障碍或疾病发作时突然加重的风险而需要入住重症监护病房。本综述旨在描述TTP诊断和管理方面的最新进展，以供重症监护专家参考。
大多数研究团队使用具有1型血小板反应蛋白重复序列的解聚素和金属蛋白酶（ADAMTS13）活性以及临床生物学特征来定义TTP，这使得文献解读更加容易。2015年TTP治疗的主要问题仍然是首次发作的TTP治疗中抗CD20抗体利妥昔单抗的使用指征和时机，以及在ADAMTS13活性降低的无症状患者中预防性使用利妥昔单抗。
血栓性微血管病的分类已从临床定义发展为病理生理定义。TTP的特征是严重的ADAMTS13缺乏，这在体外可以得到证实，并且在大多数成人病例中还伴有抗ADAMTS13抗体。血浆置换和类固醇免疫抑制治疗仍然是急性首发TTP的标准治疗方法。抗CD20单克隆抗体利妥昔单抗在复发性和/或难治性TTP中是安全有效的。其在首发TTP中的应用仍有待评估，但专家们仍然推荐使用。TTP的新型疗法仍处于临床前阶段。
