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一名年轻女性的古德综合征:一种不寻常的表现。

Good Syndrome in a Young Woman: An Unusual Presentation.

作者信息

Rebelo Sandra D, Ferreira Tiago, Pacheco Teresa, Silva Susana L, Tornada Ana

机构信息

Serviço de Medicina Interna, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte, Lisbon, PRT.

Serviço de Oncologia Médica, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte, Lisbon, PRT.

出版信息

Cureus. 2024 Jan 22;16(1):e52705. doi: 10.7759/cureus.52705. eCollection 2024 Jan.

DOI:10.7759/cureus.52705
PMID:38384652
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10879946/
Abstract

Good Syndrome is a rare disease that comprises the presence of a thymoma, immunodeficiency, and recurrent opportunistic infections. We report the case of a young woman who was diagnosed with Good Syndrome, who had a long-term history of recurrent infections, often due to atypical agents, and who also had a previous history of immunodeficiency and a B1 thymoma invading the large vessels, lung, and pericardium (Masaoka stage IV). She underwent surgical resection of the mediastinal mass, requiring vena cava superior reconstruction due to the extent of invasion, followed by adjuvant radiotherapy and immunoglobulin G supplementation. Despite relative stability in the subsequent years, without serious infections, after three years she had a thymoma recurrence requiring a new therapeutic approach. This case highlights the importance of a thorough investigation of the underlying causes of recurrent infections, which may be the result of an immunodeficiency secondary to malignancy. In young patients, early diagnosis is crucial to avoid disease progression and to reduce mortality rates. To achieve such outcomes, a multidisciplinary team and a comprehensive therapeutic strategy are necessary.

摘要

古德综合征是一种罕见疾病,其特征为存在胸腺瘤、免疫缺陷和复发性机会性感染。我们报告一例被诊断为古德综合征的年轻女性病例,她有长期复发性感染病史,感染常由非典型病原体引起,既往还有免疫缺陷病史以及侵袭大血管、肺和心包的B1型胸腺瘤(马萨oka分期IV期)。她接受了纵隔肿物的手术切除,由于侵袭范围需要进行上腔静脉重建,随后进行辅助放疗和补充免疫球蛋白G。尽管在随后几年病情相对稳定,没有严重感染,但三年后她出现了胸腺瘤复发,需要新的治疗方法。该病例强调了对复发性感染的潜在病因进行全面调查的重要性,复发性感染可能是恶性肿瘤继发免疫缺陷的结果。在年轻患者中,早期诊断对于避免疾病进展和降低死亡率至关重要。为实现这些结果,需要一个多学科团队和综合治疗策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d430/10879946/722b7a6edbaf/cureus-0016-00000052705-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d430/10879946/514c6e7a8f81/cureus-0016-00000052705-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d430/10879946/140c04a4ad82/cureus-0016-00000052705-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d430/10879946/606d2940fc0a/cureus-0016-00000052705-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d430/10879946/722b7a6edbaf/cureus-0016-00000052705-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d430/10879946/514c6e7a8f81/cureus-0016-00000052705-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d430/10879946/140c04a4ad82/cureus-0016-00000052705-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d430/10879946/606d2940fc0a/cureus-0016-00000052705-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d430/10879946/722b7a6edbaf/cureus-0016-00000052705-i04.jpg

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本文引用的文献

1
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.
2
The 2021 WHO Classification of Tumors of the Thymus and Mediastinum: What Is New in Thymic Epithelial, Germ Cell, and Mesenchymal Tumors?2021 年世界卫生组织胸腺和纵隔肿瘤分类:胸腺上皮性、生殖细胞性和间叶性肿瘤有哪些新变化?
J Thorac Oncol. 2022 Feb;17(2):200-213. doi: 10.1016/j.jtho.2021.10.010. Epub 2021 Oct 22.
3
Immunodeficiency and thymoma in Good syndrome: Two sides of the same coin.
Good 综合征伴免疫缺陷和胸腺瘤:同一问题的两个方面。
Immunol Lett. 2021 Mar;231:11-17. doi: 10.1016/j.imlet.2020.12.010. Epub 2021 Jan 5.
4
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.