Baines Andrea C, Brodsky Robert A
Division of Hematology, Department of Medicine, Johns Hopkins School of Medicine, Baltimore, MD 21205, USA.
Blood Rev. 2017 Jul;31(4):213-223. doi: 10.1016/j.blre.2017.02.003. Epub 2017 Feb 6.
The complement system is an essential part of the innate immune system that requires careful regulation to ensure responses are appropriately directed against harmful pathogens, while preventing collateral damage to normal host cells and tissues. While deficiency in some components of the complement pathway is associated with increased susceptibility to certain infections, it has also become clear that inappropriate activation of complement is an important contributor to human disease. A number of hematologic disorders are driven by complement, and these disorders may be termed "complementopathies". This includes paroxysmal nocturnal hemoglobinuria (PNH), atypical hemolytic uremic syndrome (aHUS), cold agglutinin disease (CAD) and other related disorders, which will be the focus of this review. A better understanding of the central role of the complement system in the pathophysiology of these disorders may allow for application of therapies directed at blocking the complement cascade.
补体系统是固有免疫系统的重要组成部分，需要精细调节以确保免疫反应能准确针对有害病原体，同时防止对正常宿主细胞和组织造成附带损害。虽然补体途径某些成分的缺陷与对某些感染的易感性增加有关，但补体的不适当激活显然也是人类疾病的一个重要促成因素。许多血液系统疾病是由补体驱动的，这些疾病可被称为“补体病”。这包括阵发性夜间血红蛋白尿（PNH）、非典型溶血性尿毒症综合征（aHUS）、冷凝集素病（CAD）及其他相关疾病，这些将是本综述的重点。更好地理解补体系统在这些疾病病理生理学中的核心作用，可能有助于应用针对阻断补体级联反应的疗法。
