Meri S, Jarva H
Department of Bacteriology and Immunology, Haartman Institute, Helsinki University Central Hospital, Finland.
Vox Sang. 1998;74 Suppl 2:291-302. doi: 10.1111/j.1423-0410.1998.tb05434.x.
Because of its strong potential for generating inflammation and causing tissue destruction the complement system has to be kept strictly under control. Cells of the host need special protection against the cytolytic complement system. This paper will describe how inappropriate activation of complement in the fluid phase is prevented and how viable human blood cells defend themselves against being destroyed and cleared away by the complement system. Since disturbances in complement regulation occasionally result in disease a brief reference will be made to two of the syndromes caused by complement regulator deficiency, hereditary angioedema (HAE) and paroxysmal nocturnal hemoglobinuria (PNH).
由于补体系统具有引发炎症和导致组织破坏的强大潜力，因此必须严格加以控制。宿主细胞需要针对溶细胞性补体系统进行特殊保护。本文将描述如何防止补体在液相中不适当激活，以及活的人类血细胞如何抵御补体系统的破坏和清除。由于补体调节紊乱偶尔会导致疾病，因此将简要提及由补体调节因子缺乏引起的两种综合征，即遗传性血管性水肿（HAE）和阵发性夜间血红蛋白尿（PNH）。
