Mitsuya H, Tomino S, Hisamitsu S, Kishimoto S
J Clin Lab Immunol. 1979 Nov;2(4):337-42.
A 28-year-old man with immunodeficiency with hyper IgM was studied. His serum immunoglobulins were characterized by the absence of IgA and low level of IgG associated with high level of IgM. The in vitro pokeweed mitogen (PWM)-induced immunoglobulin synthesis by his peripheral blood lymphocytes was depressed completely for IgA and moderately for IgG although normal numbers and proportions of IgA- and IgG-bearing lymphocytes (on the surface) were demonstrated in the peripheral blood. His T cells could not help IgA production by either normal or his own B cells, whereas they were more efficient helpers for IgM production by his own B cells than were normal T cells. In addition, his B cells produced no IgA and less IgG than normal B cells when co-cultured with normal T cells. This suggests that the failure of IgA specific T helper activity and the maturation arrest of B cells at the stage of the switchover from IgM- to IgG- and/or IgA-producing cells may be the major cause for the hypogammaglobulinaemia in this patient. It is uncertain whether the maturation arrest of B cells is secondary to the T cell defect in helper function for IgA production.
对一名患有高IgM免疫缺陷的28岁男性进行了研究。其血清免疫球蛋白的特征是缺乏IgA，IgG水平低且与高水平的IgM相关。尽管在外周血中显示出正常数量和比例的（表面）携带IgA和IgG的淋巴细胞，但他的外周血淋巴细胞经体外商陆有丝分裂原（PWM）诱导的免疫球蛋白合成中，IgA完全受抑，IgG中度受抑。他的T细胞无论是对正常B细胞还是自身B细胞的IgA产生均无辅助作用，而对于自身B细胞产生IgM而言，它们比正常T细胞是更有效的辅助细胞。此外，当与正常T细胞共培养时，他的B细胞不产生IgA，产生的IgG也比正常B细胞少。这表明IgA特异性T辅助活性的缺失以及B细胞在从产生IgM细胞向产生IgG和/或IgA细胞转换阶段的成熟停滞可能是该患者低丙种球蛋白血症的主要原因。尚不确定B细胞的成熟停滞是否继发于IgA产生辅助功能的T细胞缺陷。
