Blaese R M, Strober W, Waldmann T A
Birth Defects Orig Artic Ser. 1975;11(1):250-4.
The immune system in the Wiskott-Aldrich syndrome is characterized by a host of paradoxes. Elements of the effector limbs of both humoral and cellular immunity such as immunoglobulin synthesis and in vitro lymphocyte proliferative, secretory and killer responses to nonspecific mitogens are intact. Yet, these patients fail to produce specific antibody normally following immunization and they are anergic. This combination of findings is consistent with a defect in the appropriate initiation of specific immune responses, ie a defect in the afferent limb of immunity.
威斯科特-奥尔德里奇综合征的免疫系统具有一系列自相矛盾的特征。体液免疫和细胞免疫效应分支的要素，如免疫球蛋白合成以及体外淋巴细胞对非特异性有丝分裂原的增殖、分泌和杀伤反应均完好无损。然而，这些患者在免疫接种后无法正常产生特异性抗体，且呈无反应性。这些发现的综合结果与特异性免疫反应的适当启动缺陷相一致，即免疫传入分支存在缺陷。
