Radaszkiewicz T, Eibl M, Jarisch R, Lachmann D
Virchows Arch A Pathol Anat Histol. 1975;366(4):341-51. doi: 10.1007/BF00433893.
A case of hereditary incomplete, combined immunodeficiency is reported. The patient, a 20-week-old boy, suffered from severe, generalized tuberculosis after BCG-vaccination. A conspicuous discrepancy was found between the normal number of lymphocytes in the peripheral blood and the severe morphological changes in the organs of the lymphoreticular system (deficient development of thymic structures; absence of lymphocytes in the thymus and the thymus-dependent areas of the periphery as well as in the bursa-dependent structures). A partial primary defect of the stem cells and a secondary insufficiency of the functions with incomplete differentiation due to an insufficiency of the primary lymphatic organs are discussed as possible causative factors. The inability to develop epitheloid cells in connection with the tuberculous infection is interpreted in part as a sequence of a T-cell insufficiency.
报告了一例遗传性不完全性联合免疫缺陷病例。该患者为一名20周大的男孩,在接种卡介苗后患上了严重的全身性结核病。在外周血淋巴细胞数量正常与淋巴网状系统器官严重形态学改变(胸腺结构发育不全;胸腺及外周胸腺依赖区以及法氏囊依赖结构中无淋巴细胞)之间发现了明显差异。讨论了干细胞的部分原发性缺陷以及由于初级淋巴器官功能不全导致的不完全分化的继发性功能不足作为可能的致病因素。与结核感染相关的无法形成上皮样细胞部分被解释为T细胞功能不全的结果。