Hypogammaglobulinemia and anemia 18 years after thymoma resection
Hypogammaglobulinemia and anemia 18 years after thymoma resection
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DOI：
10.1016/s0091-6749(97)70283-5
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发表时间：
1997-12-01
影响因子：
14.2
通讯作者：
Richmond, GW
中科院分区：
文献类型：
--
作者：
Raschal, S;Siegel, JN;Richmond, GW
免疫缺陷伴胸腺瘤(Good‘s综合征)是一种罕见的情况，发生在7%到13%的成人起病的低丙种球蛋白血症患者中。在80%的病例中，低丙种球蛋白血症是在胸腺瘤确诊后5年内发现的。一名70岁的男性在胸腺瘤切除18年后被发现患有低丙种球蛋白血症。细胞免疫表型显示未检测到B细胞，CD4(+)细胞减少，CD8(+)细胞增加。CD4(+)和CD8(+)亚群均表达CD38(+)DR+细胞和CD45RO(+)细胞。CD8(+)CD28(+)细胞明显减少。患者外周血单核细胞可诱导产生的细胞因子显示IL-2、IL-10和干扰素-γ的产生减少。这些数据表明，Good综合征患者激活了具有细胞因子分泌失调的记忆T细胞。
Immunodeficiency with a thymoma (Good's syndrome) is a rare condition occurring in 7% to 13% of patients with adult-onset hypogammaglobulinemia. In 80% of cases, hypogammaglobulinemia is detected within 5 years of the identification of the thymoma. A 70-year-old man was found to have hypogammaglobulinemia 18 years after a thymoma had been resected. Cellular immunophenotyping revealed there were no detectable B cells, decreased CD4(+) cells, and increased CD8(+) cells. Both CD4(+) and CD8(+) subsets expressed increased populations of CD38(+) DR+ cells and CD45RO(+) cells. The CD8(+) CD28(+) population was markedly reduced. Inducible cytokine production by the patient's peripheral blood mononuclear cells revealed decreased IL-2, IL-10, and interferon-gamma production. These data suggest that patients with Good's syndrome have activated memory T cells that have dysregulated cytokine production.