Tsai K S, Chuang C Y, Hsieh K H, Wang T R, Luh K T, Hsu H C
Zhonghua Min Guo Wei Sheng Wu Ji Mian Yi Xue Za Zhi. 1980 Mar;13(1):63-8.
A 17-year-old school boy with recurrent sinopulmonary infection and diarrhea was found to have marked hypogammaglobulinemia. He had no family history of similar disorder. Serum IgG was measured 240 mg/dl, whereas all the other classes of immunoglobulins were undetectable with Mancini's technique. The polymorphonuclear leukocyte function and complement system were normal. Although the numbers of T and B lymphocytes were normal, we demonstrated that the patient's B cells were able to produce only IgG in subnormal amount and unable to secrete other classes of immunoglobulins. No hyperactive suppressor T cell function or inadequate helper T cell function was found. Therefore, the basic immmunopathogeneic mechanism was ascribed to intrinsic B cell defect.
一名17岁的男学生反复出现鼻窦肺部感染和腹泻，被发现有明显的低丙种球蛋白血症。他没有类似疾病的家族史。血清IgG测量值为240mg/dl，而用曼西尼技术检测不到其他所有类别的免疫球蛋白。多形核白细胞功能和补体系统正常。尽管T淋巴细胞和B淋巴细胞数量正常，但我们证明该患者的B细胞仅能产生低于正常量的IgG，且无法分泌其他类别的免疫球蛋白。未发现抑制性T细胞功能亢进或辅助性T细胞功能不足。因此，基本的免疫致病机制归因于内在的B细胞缺陷。
