Manzanell C, Morell A, Keller H
Schweiz Med Wochenschr. 1985 Oct 5;115(40):1393-401.
The clinical and immunologic findings in a 57-year-old woman with hypogammaglobulinemia are described. The immunodeficiency occurred 11 years after surgical removal of an invasive lymphocytic thymoma. The typical findings of hypogammaglobulinemia as a thymoma-associated clinical syndrome are absence of peripheral blood B-lymphocytes and deficiency of plasma cells in bone marrow. However, in our patient peripheral blood B-lymphocytes and plasma cells in bone marrow were within normal limits, while the total number of peripheral blood mononuclear cells was subnormal. This subnormality was found to be a diminution of peripheral blood T-helper cells. Our patient was anergic to a battery of skin test antigens and her lymphocytes showed a decreased in vitro response to mitogen stimulation. In particular, the response to phytohemagglutinin (PHA) was low, but could be improved by the addition of interleukin-2 to the culture medium. Cocultures between patient B-cells and normal T-cells revealed normal function of patient B-cells. However, cocultures between patient T-cells and normal B-cells showed a significant depression of mitogen-induced immunoglobulin synthesis. These results are suggestive of T-helper cell dysfunction. Consequently, in our case the hypogammaglobulinemia was assumed to be secondary to deficient T-helper cell function.
描述了一名57岁低丙种球蛋白血症女性的临床和免疫学发现。免疫缺陷在侵袭性淋巴细胞性胸腺瘤手术切除11年后出现。作为胸腺瘤相关临床综合征的低丙种球蛋白血症的典型表现是外周血B淋巴细胞缺失和骨髓中浆细胞缺乏。然而，在我们的患者中，外周血B淋巴细胞和骨髓中的浆细胞在正常范围内，而外周血单个核细胞总数低于正常。发现这种低于正常是外周血辅助性T细胞减少。我们的患者对一系列皮肤试验抗原无反应，其淋巴细胞对有丝分裂原刺激的体外反应降低。特别是，对植物血凝素（PHA）的反应较低，但通过在培养基中添加白细胞介素-2可得到改善。患者B细胞与正常T细胞的共培养显示患者B细胞功能正常。然而，患者T细胞与正常B细胞的共培养显示有丝分裂原诱导的免疫球蛋白合成显著降低。这些结果提示辅助性T细胞功能障碍。因此，在我们的病例中，低丙种球蛋白血症被认为继发于辅助性T细胞功能缺陷。
