台湾南部尚无常见变异免疫缺陷（CVID）病例系列报道。台湾南部一家医疗中心对成人 CVID 进行了 20 年回顾。 2003 年 5 月至 2023 年 4 月期间纳入了年龄在 18 岁或以上的患者。确定了 12 名患者，其中 8 名女性/4 名男性，年龄 23 至 68 岁 (38.9 ± 13.4)，延迟 1 至 11 年 (5.0 ± 3.3)。发病后进行诊断。 7 例 (58%) 患者伴有自身免疫性疾病，10 例 (83%) 患者伴有脾肿大，4 例 (25%) 患者伴有淋巴结肿大，2 例 (17%) 患者伴有 B 细胞淋巴瘤。所有患者均接受静脉免疫球蛋白 (IVIg) 输注，并在 2 例中改善自身免疫介导的关节炎。 IgG 谷水平较高（高于 500 mg/dL）的患者比 IgG 谷水平较低的患者有更好的生存率。台湾南部的成人 CVID 具有较高的自身免疫性和淋巴细胞增殖表现的发生率。通过 IVIg 输注进行早期诊断可能会改善演示效果。版权所有 © 2023。由 Elsevier B.V. 出版。

There are no reported case series of common variable immunodeficiency (CVID) from southern Taiwan. A 20-year review was performed in adult CVID from a southern Taiwan medical center. Patients with ages of 18 years or older were enrolled from May, 2003 to April, 2023. Twelve patients were identified, 8 females/4 males aged 23 to 68 (38.9 ± 13.4) with one to 11 years (5.0 ± 3.3) delay of diagnosis after disease onset. There were concomitant autoimmune disorders in 7 (58 %), splenomegaly in 10 (83 %), lymphadenopathy in 4 (25 %) and B-cell lymphoma in 2 (17 %). All received intravenous immunoglobulin (IVIg) infusion with improved autoimmune-mediated arthritis in 2. Patients with higher IgG trough levels (above 500 mg/dL) had a better survival than those with lower IgG trough levels. Adult CVID in southern Taiwan has a high occurrence of autoimmune and lymphoproliferative manifestations. Early diagnosis with IVIg infusion might improve the presentations.Copyright © 2023. Published by Elsevier B.V.