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多系统功能障碍后常见变异型免疫缺陷的诊断

The Diagnosis of Common Variable Immunodeficiency After Multisystem Dysfunction.

作者信息

Rasul Taha F, Bergholz Daniel R, Faiz Arfa

机构信息

Department of Infectious Diseases, University of Miami Miller School of Medicine, Miami, USA.

Department of Allergy and Immunology, University of Miami Miller School of Medicine, Miami, USA.

出版信息

Cureus. 2022 Jan 26;14(1):e21624. doi: 10.7759/cureus.21624. eCollection 2022 Jan.

DOI:10.7759/cureus.21624
PMID:35228973
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8874879/
Abstract

Common variable immunodeficiency (CVID) is a primary immunodeficiency caused by the lack of B cell differentiation into plasma cells, thereby leading to decreased serum immunoglobulins. Patients with this condition are predisposed to recurrent infections and are more likely to develop certain cancers and autoimmune diseases. We report the case of a 53-year-old female suffering from recurrent pulmonary infections and a history of non-Hodgkin lymphoma (NHL) who had a poor response to the measles, mumps, and rubella (MMR) and varicella vaccines as a child, and was infected with coronavirus disease 2019 (COVID-19) twice in 2020. Testing of her antibody titers in order to determine suitability for vaccination found an overall decrease in major immunoglobulin classes (IgG, IgM, and IgA) and B cells with normal morphology. The diagnosis of CVID was made, and prompt treatment with intravenous immunoglobulins (IVIG) brought her IgG levels up from 282 to 680 mg/dL within three months. This case highlights the importance for providers to keep immunological dysfunction on their differentials for patients with atypical presentations involving multiple organ systems.

摘要

普通可变免疫缺陷(CVID)是一种原发性免疫缺陷,由B细胞分化为浆细胞的过程受阻引起,进而导致血清免疫球蛋白水平降低。患有这种疾病的患者易反复感染,更有可能患上某些癌症和自身免疫性疾病。我们报告了一例53岁女性病例,该患者反复发生肺部感染,有非霍奇金淋巴瘤(NHL)病史,儿童时期对麻疹、腮腺炎和风疹(MMR)疫苗以及水痘疫苗反应不佳,并于2020年两次感染2019冠状病毒病(COVID-19)。为确定其是否适合接种疫苗而进行的抗体滴度检测发现,主要免疫球蛋白类别(IgG、IgM和IgA)以及形态正常的B细胞总体减少。确诊为CVID后,立即给予静脉注射免疫球蛋白(IVIG)治疗,三个月内她的IgG水平从282mg/dL升至680mg/dL。该病例凸显了对于出现涉及多个器官系统的非典型表现的患者,医疗人员在鉴别诊断时考虑免疫功能障碍的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ffae/8874879/5a379c1d1c9e/cureus-0014-00000021624-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ffae/8874879/5a379c1d1c9e/cureus-0014-00000021624-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ffae/8874879/5a379c1d1c9e/cureus-0014-00000021624-i01.jpg

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本文引用的文献

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Common Variable Immunodeficiency and Autoimmune Diseases: A Retrospective Study of 95 Adult Patients in a Single Tertiary Care Center.普通变异性免疫缺陷和自身免疫性疾病:单中心三级医疗中心 95 例成人患者的回顾性研究。
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观点:常见可变免疫缺陷疾病(CVID)诊断和理解中不断发展的概念。
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Interstitial Lung Disease Frequently Precedes CVID Diagnosis.间质性肺疾病常先于普通变异型免疫缺陷病的诊断出现。
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A High Prevalence of Gastrointestinal Manifestations in Common Variable Immunodeficiency.常见可变免疫缺陷中胃肠道表现的高患病率。
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Two Sides of the Same Coin: Pediatric-Onset and Adult-Onset Common Variable Immune Deficiency.两面一体:儿童期起病和成人期起病的普通变异型免疫缺陷。
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Common Variable Immunodeficiency Disorders (CVID) - Diagnoses of Exclusion, Especially Combined Immune Defects.普通可变免疫缺陷疾病(CVID)——排除性诊断,尤其是联合免疫缺陷。
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Common Variable Immunodeficiency.普通可变免疫缺陷
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Progression of Common Variable Immunodeficiency Interstitial Lung Disease Accompanies Distinct Pulmonary and Laboratory Findings.普通可变免疫缺陷性间质性肺疾病的进展伴有独特的肺部和实验室检查结果。
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