Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, 02114.
Am J Hematol. 2015 Dec;90(12):1180-6. doi: 10.1002/ajh.24209.
The complement pathway is a cascade of proteases that is involved in immune surveillance and innate immunity, as well as adaptive immunity. Dysfunction of the complement cascade may be mediated by aberrations in the pathways of activation, complement regulatory proteins, or complement deficiencies, and has been linked to a number of hematologic disorders, including paroxysmal noctural hemoglobinuria (PNH), hereditary angioedema (HAE), and atypical hemolytic-uremic syndrome (aHUS). Here, current laboratory tests for disorders of the complement pathway are reviewed, and their utility and limitations in hematologic disorders and systemic diseases are discussed. Current therapeutic advances targeting the complement pathway in treatment of complement-mediated hematologic disorders are also reviewed.
补体途径是一系列蛋白酶，参与免疫监视和先天免疫以及适应性免疫。补体级联功能障碍可能由激活途径、补体调节蛋白或补体缺陷的异常介导，并与许多血液系统疾病有关，包括阵发性夜间血红蛋白尿症（PNH）、遗传性血管性水肿（HAE）和非典型溶血尿毒综合征（aHUS）。本文回顾了补体途径异常的当前实验室检测方法，并讨论了其在血液系统疾病和系统性疾病中的应用及局限性。本文还综述了针对补体介导的血液系统疾病治疗中补体途径的当前治疗进展。
