Fontán G, García Rodriguez M C, Carrasco S, Zabay J M, de la Concha E G
Immunology Service, Ciudad Sanitaria La Paz, Madrid, Spain.
Clin Immunol Immunopathol. 1988 Mar;46(3):432-41. doi: 10.1016/0090-1229(88)90062-1.
Two cases of severe combined immunodeficiency (SCID) with normal numbers of T cells are reported. Studies of T-cell subsets showed an absence of TQ1+ lymphocytes and a very low percentage of CD4+ cells in Patient 2. Functional studies of T cells from this patient showed a normal suppressor activity. Patient 1 had normal percentages of T-cell subsets and his lymphocytes showed helper and suppressor activities but to a lesser degree than normal controls. Both cases stressed the heterogeneity of SCID in which T cells could be present and retain some of their functional activities.
报告了两例T细胞数量正常的重症联合免疫缺陷(SCID)病例。T细胞亚群研究显示,患者2缺乏TQ1+淋巴细胞,CD4+细胞百分比极低。对该患者T细胞的功能研究显示其抑制活性正常。患者1的T细胞亚群百分比正常,其淋巴细胞表现出辅助和抑制活性,但程度低于正常对照。这两个病例都强调了SCID的异质性,即T细胞可能存在并保留一些功能活性。