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古德综合征与新型冠状病毒肺炎:病例报告及文献综述

Good's syndrome and COVID-19: case report and literature review.

作者信息

Berzenji Lawek, Yogeswaran Suresh Krishan, Snoeckx Annemiek, Van Schil Paul E, Wener Reinier, Hendriks Jeroen M H

机构信息

Department of Thoracic and Vascular Surgery, Antwerp University Hospital, Edegem, Belgium.

Department of Radiology, Antwerp University Hospital, Edegem, Belgium.

出版信息

Mediastinum. 2022 Sep 9;7:5. doi: 10.21037/med-22-12. eCollection 2023.

Abstract

BACKGROUND

Good's syndrome (GS) is an adult-onset acquired immunodeficiency, in which patients present with thymoma and hypogammaglobulinemia (HGG). GS is characterized by low to absent peripheral B cells and impaired T-cell mediated immunity, often resulting in various (opportunistic) infections and concurrent autoimmune disorders. In this case report, we present a case of a patient with GS and coronavirus disease 2019 (COVID-19) infection after surgical removal of a thymoma. The simultaneous occurence of these two entities is extremely rare.

CASE DESCRIPTION

A 55-year-old man presented with oral lichen planus and cutaneous lesions. Additional symptoms included a weight loss of 5 kilograms in the last six months. Computed tomography (CT) and positron emission tomography (PET) of the chest showed a large anterior mediastinal mass with a maximum diameter of 10 centimetres. A core needle biopsy was performed, which led to a pathological diagnosis of thymoma type AB. In addition to these earlier findings, laboratory analysis revealed HGG. The combination of a thymoma and HGG led to a diagnosis of GS. Induction chemotherapy with cisplatin-etoposide was started, however, the patient developed COVID-19 after 2 cycles. Treatment with remdesivir was initiated and, subsequently, a thymectomy via sternotomy was performed. Final pathology confirmed a thymoma type AB of 14 centimetres, fully encapsulated, and without invasion. Resection margins were negative and the tumour was classified as pT1aN0, R0 resection. The patient has received immunoglobulin treatments every 4 weeks for his GS and has not developed any new infections since the start of this therapy.

CONCLUSIONS

Patients with GS are prone to developing (pulmonary) infections. Clinicians should be aware of the possible clinical effects of COVID-19 infections in this patient population.

摘要

背景

古德综合征(GS)是一种成人起病的获得性免疫缺陷病,患者表现为胸腺瘤和低丙种球蛋白血症(HGG)。GS的特征是外周血B细胞减少或缺乏以及T细胞介导的免疫功能受损,常导致各种(机会性)感染和并发自身免疫性疾病。在本病例报告中,我们介绍了一例在手术切除胸腺瘤后感染2019冠状病毒病(COVID-19)的GS患者。这两种疾病同时出现极为罕见。

病例描述

一名55岁男性出现口腔扁平苔藓和皮肤病变。其他症状包括在过去六个月内体重减轻了5公斤。胸部计算机断层扫描(CT)和正电子发射断层扫描(PET)显示前纵隔有一个最大直径为10厘米的大肿块。进行了粗针活检,病理诊断为AB型胸腺瘤。除了这些早期发现外,实验室分析显示存在HGG。胸腺瘤和HGG的结合导致了GS的诊断。开始使用顺铂-依托泊苷进行诱导化疗,然而,患者在2个周期后感染了COVID-19。开始使用瑞德西韦治疗,随后通过胸骨切开术进行了胸腺切除术。最终病理证实为14厘米的AB型胸腺瘤,完全包膜完整,无侵袭。切除边缘阴性,肿瘤分类为pT1aN0,R0切除。该患者因GS每4周接受一次免疫球蛋白治疗,自该治疗开始以来未发生任何新的感染。

结论

GS患者易发生(肺部)感染。临床医生应意识到COVID-19感染对该患者群体可能产生的临床影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d25/10011861/5f2e17d1d28b/med-07-5-f1.jpg

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