Sampson H A, Walchner A M, Baker P J
J Clin Immunol. 1982 Jan;2(1):39-45. doi: 10.1007/BF00915977.
While deficiency of the terminal components of complement (C3-C8) has classically been associated with recurrent pyogenic infections, it has become apparent that C2 deficiency is also associated with recurrent infections in some individuals. The patient presented here had two major pyogenic infections prior to 1 year of age and was found to lack the second component of complement. Studies of alternative complement pathway and humoral and cellular immunity were found to be within normal limits. Family studies suggest an autosomal codominant pattern of inheritance for the C2 defect, which also corresponded to the inheritance of the HLA A10 B18 haplotype. A review of the literature revealed nine other cases of C2-deficient patients with well-documented recurrent infections. In these patients, Streptococcus pneumoniae is the organism most frequently implicated in infectious processes, whether or not their alternative complement pathway is intact.
虽然传统上认为补体终末成分（C3 - C8）缺乏与复发性化脓性感染有关，但现在已明显看出，C2缺乏在某些个体中也与复发性感染有关。本文报告的患者在1岁前有两次主要的化脓性感染，且被发现缺乏补体的第二成分。对替代补体途径以及体液免疫和细胞免疫的研究发现均在正常范围内。家族研究提示C2缺陷呈常染色体共显性遗传模式，这也与HLA A10 B18单倍型的遗传情况相符。文献回顾发现另外9例有充分记录的C2缺乏且复发性感染的患者。在这些患者中，无论其替代补体途径是否完整，肺炎链球菌都是感染过程中最常涉及的病原体。
