Wernet P, Siegal F P, Dickler H, Fu S, Kunkel H G
Proc Natl Acad Sci U S A. 1974 Feb;71(2):531-5. doi: 10.1073/pnas.71.2.531.
Certain patients with immune deficiency were encountered whose peripheral blood lymphocytes included no immunoglobulin-bearing cells. However, other markers of B type lymphocytes were observed; lymphocytes isolated free of macrophages showed the presence of receptors for the Fc fragment of IgG and for the third component of complement. One patient with a syndrome of thymoma and severe hypogammaglobulinemia was studied in special detail. In vitro the patient's cells were able to develop surface Ig in media supplemented with fetal-calf serum or normal human serum; in media supplemented with autologous serum, the cells developed no surface Ig. During culturing antigenic determinants of immunoglobulin became detectable in the medium, and both medium and cell-surface immunoglobulin underwent a shift from specific IgM determinants early in the culture period to IgG and IgA determinants later. Normal lymphocytes and thymocytes activated by concanavalin A repaired the deficiency in the patient's serum. These data support the concept that a factor possibly derived from T cells is missing from this patient's serum and that this factor is required for the maturation of the B cell for immunoglobulin synthesis.A patient with X-linked agammaglobulinemia had a population of circulating lymphocytes with some surface characteristics that appeared similar to those of the B cells from the patient with thymoma. In contrast, however, no Ig synthesis by this patient's cultured cells could be demonstrated. The possible nature of the lymphocytes reacting with aggregated IgG in this case is discussed.
某些免疫缺陷患者的外周血淋巴细胞中未发现携带免疫球蛋白的细胞。然而,观察到了B淋巴细胞的其他标志物;从巨噬细胞中分离出的淋巴细胞显示存在IgG Fc片段和补体第三成分的受体。对一名患有胸腺瘤和严重低丙种球蛋白血症综合征的患者进行了特别详细的研究。在体外,患者的细胞在添加胎牛血清或正常人血清的培养基中能够产生表面免疫球蛋白;在添加自体血清的培养基中,细胞不产生表面免疫球蛋白。在培养过程中,培养基中可检测到免疫球蛋白的抗原决定簇,培养基和细胞表面免疫球蛋白都经历了从培养早期的特异性IgM决定簇到后期的IgG和IgA决定簇的转变。由伴刀豆球蛋白A激活的正常淋巴细胞和胸腺细胞弥补了患者血清中的缺陷。这些数据支持这样一种概念,即该患者血清中可能缺少一种可能来源于T细胞的因子,而这种因子是B细胞成熟进行免疫球蛋白合成所必需的。一名患有X连锁无丙种球蛋白血症的患者有一群循环淋巴细胞,其一些表面特征与胸腺瘤患者的B细胞相似。然而,与此形成对比的是,该患者培养的细胞未能证明有免疫球蛋白合成。本文讨论了在这种情况下与聚集IgG反应的淋巴细胞的可能性质。