Good’s Syndrome, a Rare form of Acquired Immunodeficiency Associated with Thymomas

Author:

Tamburello Antonio,Castelnovo Laura,Faggioli Paola,Bompane Daniela,Brando Bruno,Gatti Arianna,Roncoroni Lucia,Di Marco Biancamaria,Mazzone Antonino

Abstract

Good’s syndrome (GS) or thymomaassociated immunodeficiency is a rare clinical entity that should be ruled out in patients with thymoma who develop severe, recurrent bacterial infections and opportunistic viral and fungal infections. There are no treatment protocols established, hence, early recognition is imperative to avoid complications. We report the case of a 42-year-old female, known for a previous thymectomy for giant thymoma who has suffered for a long time from recurrent pulmonary and urinary tract infections and cold sores. In March 2016 she referred to our unit complaining of fever, cough, chest pain, and cold sores due to Herpes simplex virus (HSV), confirmed serologically as HSV-1. Chest X-ray showed left pneumonia due to Streptococcus pneumoniae. She started antibiotics (amoxicillin/clavulanic acid associated with azithromycin) with gradual improvement. Given her history she was studied for an underlying immunodeficiency: IgG, IgA, and IgM were significantly low or absent, as well as all IgG subclasses; blood and bone marrow aspirate leucocyte immunophenotyping showed complete absence of B lymphocytes and reduced CD4+ T cells. In light of: i) thymoma; ii) B lymphocyte deficit; iii) hypogammaglobulinemia; iv) recurrent infections, GS was diagnosed and pre-emptive immunoglobulin treatment, associated with HSV and Pneumocystis jiroveci prophylaxis (Acyclovir for HSV and Sulfamethoxazole- Trimethoprim for P. jiroveci) were started. Since then the patient has no longer presented any infectious episodes.

Publisher

MDPI AG

Subject

General Computer Science

Reference12 articles.

1. Characteristics of Good’s Syndrome in China: a systematic review;Dong;Chin Med J,2017

2. Agammaglobulinemia – a provocative experiment of nature;Good;Bull University Minnesota,1954

3. Case report and literature review on Good's syndrome, a form of acquired immunodeficiency associated with thymomas;Joven;Hawaii J Med Public Health,2013

4. Review: Good’s syndrome remains a mystery after 55 years: a systematic review of the scientific evidence;Kelesidis;Clin Immunol,2010

5. Durable hypogammaglobulinemia associated with thymoma (Good syndrome);Kitamura;Intern Med,2009

同舟云学术

1.学者识别学者识别

2.学术分析学术分析

3.人才评估人才评估

"同舟云学术"是以全球学者为主线,采集、加工和组织学术论文而形成的新型学术文献查询和分析系统,可以对全球学者进行文献检索和人才价值评估。用户可以通过关注某些学科领域的顶尖人物而持续追踪该领域的学科进展和研究前沿。经过近期的数据扩容,当前同舟云学术共收录了国内外主流学术期刊6万余种,收集的期刊论文及会议论文总量共计约1.5亿篇,并以每天添加12000余篇中外论文的速度递增。我们也可以为用户提供个性化、定制化的学者数据。欢迎来电咨询!咨询电话:010-8811{复制后删除}0370

www.globalauthorid.com

TOP

Copyright © 2019-2024 北京同舟云网络信息技术有限公司
京公网安备11010802033243号  京ICP备18003416号-3