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补体在自身免疫性溶血性贫血和血小板输注无效患者中的作用。

Role of complement in patients with autoimmune hemolytic anemia and platelet transfusion refractoriness.

作者信息

Fontaine Magali J

机构信息

Department of Pathology, University of Maryland School of Medicine, Baltimore, MA, USA.

出版信息

Transfus Clin Biol. 2019 Sep;26(3):152-154. doi: 10.1016/j.tracli.2019.06.232. Epub 2019 Jun 19.

Abstract

The complement is a key player of the innate immune response. It provides defense mechanisms that are not specific, but very efficient at neutralizing any invader, accounting for 4% of the proteins in the peripheral blood. Nevertheless, there is a dark side to the complement system, as it may activate its machinery against healthy cells such as peripheral blood red blood cells and platelets resulting in undesired hemolysis and thrombocytopenia, respectively. Understanding and identifying the role of complement in these settings allow physicians to adjust their diagnostic and therapeutic modalities accordingly. The role of complement in the pathophysiology and management of autoimmune hemolytic anemia and of alloimmune-mediated thrombocytopenia is under investigation and discussed.

摘要

补体是先天性免疫反应的关键参与者。它提供的防御机制是非特异性的,但在中和任何入侵者方面非常有效,占外周血中蛋白质的4%。然而,补体系统也有其不利的一面,因为它可能会激活其机制来攻击健康细胞,如外周血红细胞和血小板,分别导致不良的溶血和血小板减少。了解和确定补体在这些情况下的作用,使医生能够相应地调整他们的诊断和治疗方式。补体在自身免疫性溶血性贫血和同种免疫介导的血小板减少症的病理生理学和管理中的作用正在研究和讨论中。

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