Monafo W J, Polmar S H, Neudorf S, Mather A, Filipovich A H
Department of Pediatrics, Washington University School of Medicine, St Louis, MO.
Clin Exp Immunol. 1992 Dec;90(3):390-3. doi: 10.1111/j.1365-2249.1992.tb05856.x.
An unusual form of severe combined immunodeficiency in children from two different families was associated with absence of CD8+ T lymphocytes and normal numbers of CD4+ T lymphocytes that did not respond to stimulation by non-specific mitogens, specific antibodies against T cell receptor or specific antigens. The defect in the CD4+ cells was bypassed by activating agents which are independent of the T cell receptor. The combination of an activation defect and selective depletion of CD8+ T lymphocytes suggests that the defective pathway is important in the differentiation of immature thymocytes as well as the proliferation of mature lymphocytes.
来自两个不同家庭的儿童中出现的一种罕见的严重联合免疫缺陷形式，与CD8⁺ T淋巴细胞缺失以及CD4⁺ T淋巴细胞数量正常但对非特异性丝裂原、抗T细胞受体特异性抗体或特异性抗原刺激无反应有关。CD4⁺细胞的缺陷可被独立于T细胞受体的激活剂绕过。激活缺陷与CD8⁺ T淋巴细胞的选择性耗竭相结合表明，缺陷途径在未成熟胸腺细胞的分化以及成熟淋巴细胞的增殖中起重要作用。
