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复发性乙状结肠憩室炎揭示的古德综合征

Good's Syndrome Revealed by Recurrent Sigmoid Diverticulitis.

作者信息

Attout Hassene, Constant Jade, Abdellatif Amal, Barry Guillaume, Halle Olivier

机构信息

Internal Medicine Department, Les Cevennes Hospital, Alès, France.

出版信息

Eur J Case Rep Intern Med. 2025 Jun 5;12(7):005480. doi: 10.12890/2025_005480. eCollection 2025.

DOI:10.12890/2025_005480
PMID:40636243
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12236679/
Abstract

UNLABELLED

Thymus is a primary lymphoid organ whose main function is the education of the T lymphocytes. Thymoma is uncommon neoplasia derived from epithelial cells of the thymus. Patients with thymoma frequently present with autoimmune disorders, mostly myasthenia gravis. Good's syndrome (GS) is an acquired adult-onset combined immunodeficiency typically characterised by the triad of thymoma, hypogammaglobulinaemia and increased susceptibility to recurrent infections. Immunoglobulin replacement is the main therapeutic strategy. Our knowledge of thymoma-associated immunodeficiency is predominantly derived from case reports or small series. We report the case of a patient with recurrent sigmoid diverticulitis that appeared after a thymectomy, who was subsequently found to have severe hypogammaglobulinaemia.

LEARNING POINTS

Good's syndrome is a rare, acquired, adult-onset immunodeficiency.Good's syndrome remains an enigmatic disease.Immunoglobulin replacement is the main therapeutic strategy.

摘要

未标注

胸腺是主要的淋巴器官,其主要功能是对T淋巴细胞进行培育。胸腺瘤是一种源自胸腺上皮细胞的罕见肿瘤。胸腺瘤患者常伴有自身免疫性疾病,多数为重症肌无力。古德综合征(GS)是一种后天获得性成人发病的联合免疫缺陷病,其典型特征为胸腺瘤、低丙种球蛋白血症和反复感染易感性增加三联征。免疫球蛋白替代疗法是主要的治疗策略。我们对胸腺瘤相关免疫缺陷的认识主要来源于病例报告或小样本系列研究。我们报告了一例患者,其在胸腺切除术后出现复发性乙状结肠憩室炎,随后被发现患有严重的低丙种球蛋白血症。

学习要点

古德综合征是一种罕见的、后天获得性成人发病的免疫缺陷病。古德综合征仍然是一种难以捉摸的疾病。免疫球蛋白替代疗法是主要的治疗策略。

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Good's Syndrome Revealed by Recurrent Sigmoid Diverticulitis.复发性乙状结肠憩室炎揭示的古德综合征
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本文引用的文献

1
Good's syndrome: brief overview of an enigmatic immune deficiency.Good 综合征:一种神秘免疫缺陷病的简要概述。
APMIS. 2023 Dec;131(12):698-704. doi: 10.1111/apm.13351. Epub 2023 Sep 20.
2
Good's Syndrome: Time to Move on From Reviewing the Past.Good's 综合征:是时候从回顾过去中走出来了。
Front Immunol. 2022 Jan 12;12:815710. doi: 10.3389/fimmu.2021.815710. eCollection 2021.
3
Clinical and laboratory features of seventy-eight UK patients with Good's syndrome (thymoma and hypogammaglobulinaemia).78 例英国 Good 综合征(胸腺瘤和低丙种球蛋白血症)患者的临床和实验室特征。
Clin Exp Immunol. 2019 Jan;195(1):132-138. doi: 10.1111/cei.13216. Epub 2018 Oct 21.
4
Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective.“好综合征”的预后:胸腺oma相关免疫缺陷的死亡率和发病率展望
Clin Immunol. 2016 Oct;171:12-17. doi: 10.1016/j.clim.2016.07.025. Epub 2016 Aug 4.
5
Thymoma associated with autoimmune diseases: 85 cases and literature review.胸腺瘤合并自身免疫性疾病:85 例病例并文献复习。
Autoimmun Rev. 2016 Jan;15(1):82-92. doi: 10.1016/j.autrev.2015.09.005. Epub 2015 Sep 25.
6
Good syndrome: an adult-onset immunodeficiency remarkable for its high incidence of invasive infections and autoimmune complications.Good 综合征:一种成人发病的免疫缺陷病,其特点是侵袭性感染和自身免疫并发症的发病率高。
Clin Infect Dis. 2015 Jul 15;61(2):e13-9. doi: 10.1093/cid/civ269. Epub 2015 Mar 31.
7
Good's syndrome remains a mystery after 55 years: A systematic review of the scientific evidence.Good 综合征 55 年后仍是未解之谜:系统综述科学证据。
Clin Immunol. 2010 Jun;135(3):347-63. doi: 10.1016/j.clim.2010.01.006. Epub 2010 Feb 10.