| 成人普通变异型免疫缺陷病13例并文献复习 |
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| 引用本文： | 龚胜兰,蒲 银,谢玲俐,杨晓娅,毛 辉. 成人普通变异型免疫缺陷病13例并文献复习[J]. 南方医科大学学报, 2020, 40(8): 1213-1219. DOI: 10.12122/j.issn.1673-4254.2020.08.21 |
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| 作者姓名： | 龚胜兰 蒲 银 谢玲俐 杨晓娅 毛 辉 |
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| 摘 要： | 目的 探讨普通变异型免疫缺陷病的临床特点、免疫学特征、治疗方法和预后。方法 回顾性分析我院 2010.01.01~2019.09.26间诊断为普通变异型免疫缺陷病的13例住院患者的临床资料，包括临床表现、实验室检查、影像学检查、病理检查、治疗等。结果 依据2016年国际专家共识中普通变异型免疫缺陷病的诊断标准共纳入13例患者，发病年龄是24.46±16.82岁，诊断年龄是32.54±14.86岁，诊断延迟中位数5年（IQR：2~15年）。患者主要表现为反复感染，其中反复呼吸道感染 10例 （76.9%），反复腹泻3例（23.1%），3例（23.1%）患者并发自身免疫性疾病，10例（76.9%）患者并发慢性呼吸系统疾病。所有患者IgG、IgA、IgM均降低。10例（76.9%）患者CD4+T细胞比例降低，11例（84.6%）CD8+T细胞比例升高，10例（76.9%）CD4/CD8降 低。58.3%（7/12）的患者补体C3降低，33.3%（4/12）的患者补体C4减低。12例（92.3%）患者在院内给予静脉输注丙种球蛋白治疗，以及相应的对症治疗。1例患者因消化大出血死亡，其余患者好转出院。结论 普通变异型免疫缺陷病临床表现多样，以反复的呼吸道感染最为常见。实验室检查中IgG降低，常伴IgA、IgM降低。目前临床上以免疫球蛋白替代治疗和对并发疾病的对症治疗为主。
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Common variable immune deficiency in adult patients: analysis of 13 cases and literaturereview |
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| Abstract: | Objective To investigate the clinical and immunological characteristics, treatment and prognosis of common variableimmune deficiency (CVID) in adult patients. Methods We retrospectively analyzed the clinical data of 13 adult patientshospitalized in our hospital for CVID diagnosed according to the criteria in International Consensus Document (2016), andanalyzed their clinical manifestations, laboratory test results, imaging findings, pathological examinations and treatments.Results The mean age of onset was 24.46±16.82 years in these patients, who had a mean age of 32.54±14.86 years at diagnosiswith a median diagnostic delay of 5 years (IQR: 2-15 years). The main manifestation of the patients was repeated infections,including repeated respiratory tract infection (10 cases; 76.9% ) and repeated diarrhea (3 cases; 23.1% ). Three (23.1% ) of thepatients had autoimmune disease and 10 (76.9%) had chronic pulmonary disease. IgG, IgA and IgM were decreased in all thepatients. The proportion of CD4+T cells decreased in 10 patients (76.9%), CD8+T cells increased in 11 patients (84.6%), and CD4/CD8 decreased in 10 patients (76.9% ). Complement C3 decreased in 58.3% (7/12) and C4 decreased in 33.3% (4/12) of thepatients. Twelve patients (92.3% ) were treated with intravenous infusion of gamma globulin with symptomatic treatments.One patient died due to massive gastrointestinal hemorrhage, and the other patients showed improve ments after thetreatments and were discharged. Conclusion The clinical manifestations of CVID are diverse, and recurrent respiratory tractinfection is the most common manifestation. Decreased IgG often accompanied by lowered IgA and IgM levels is a commonfinding in laboratory tests. The treatment of CVID currently relies on gamma globulin with symptomatic treatments for thecomplications. |
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