Epstein-Barr virus-driven lymphoproliferation in inborn errors of immunity: a diagnostic and therapeutic challenge.

IF 3.9 3区医学 Q2 IMMUNOLOGY Expert Review of Clinical Immunology Pub Date : 2024-11-01 Epub Date: 2024-07-30 DOI:10.1080/1744666X.2024.2386427

Prabal Barman, Suprit Basu, Taru Goyal, Saniya Sharma, Sangeetha Siniah, Rahul Tyagi, Kaushal Sharma, Ankur K Jindal, Rakesh K Pilania, Pandiarajan Vignesh, Manpreet Dhaliwal, Deepti Suri, Amit Rawat, Surjit Singh

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Abstract

Introduction: Inborn errors of immunity (IEI) are a group of genetically heterogeneous disorders with a wide-ranging clinical phenotype, varying from increased predisposition to infections to dysregulation of the immune system, including autoimmune phenomena, autoinflammatory disorders, lymphoproliferation, and malignancy. Lymphoproliferative disorder (LPD) in IEI refers to the nodal or extra-nodal and persistent or recurrent clonal or non-clonal proliferation of lymphoid cells in the clinical context of an inherited immunodeficiency or immune dysregulation. The Epstein-Barr virus (EBV) plays a significant role in the etiopathogenesis of LPD in IEIs. In patients with specific IEIs, lack of immune surveillance can lead to an uninhibited proliferation of EBV-infected cells that may result in chronic active EBV infection, hemophagocytic lymphohistiocytosis, and LPD, particularly lymphomas.

Areas covered: We intend to discuss the pathogenesis, diagnosis, and treatment modalities directed toward EBV-associated LPD in patients with distinct IEIs.

Expert opinion: EBV-driven lymphoproliferation in IEIs presents a diagnostic and therapeutic problem that necessitates a comprehensive understanding of host-pathogen interactions, immune dysregulation, and personalized treatment approaches. A multidisciplinary approach involving immunologists, hematologists, infectious disease specialists, and geneticists is paramount to addressing the diagnostic and therapeutic challenges posed by this intriguing yet formidable clinical entity.

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先天性免疫错误中由 Epstein-Barr 病毒驱动的淋巴细胞增殖：诊断和治疗的挑战。

导言：先天性免疫错误（IEI）是一组遗传异质性疾病，其临床表型多种多样，从感染易感性增加到免疫系统失调，包括自身免疫现象、自身炎症性疾病、淋巴细胞增生和恶性肿瘤。IEI 中的淋巴细胞增生性疾病（LPD）是指在遗传性免疫缺陷或免疫失调的临床背景下，淋巴细胞结节性或结节外、持续性或复发性克隆性或非克隆性增生。Epstein-Barr 病毒（EBV）在 IEIs 的 LPD 发病机制中起着重要作用。在特定的 IEIs 患者中，免疫监视的缺乏会导致 EBV 感染细胞不受抑制地增殖，从而可能导致慢性活动性 EBV 感染、嗜血细胞性淋巴组织细胞增多症和 LPD，尤其是淋巴瘤：我们将讨论不同 IEI 患者中 EBV 相关 LPD 的发病机制、诊断和治疗方法：EBV驱动的IEIs淋巴细胞增生是一个诊断和治疗问题，需要全面了解宿主与病原体的相互作用、免疫失调和个性化治疗方法。免疫学家、血液学家、传染病专家和遗传学家共同参与的多学科方法对于解决这一有趣而又可怕的临床实体所带来的诊断和治疗难题至关重要。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Expert Review of Clinical Immunology IMMUNOLOGY-

CiteScore

7.60

自引率

2.30%

发文量

221

审稿时长

6-12 weeks

期刊介绍： Expert Review of Clinical Immunology (ISSN 1744-666X) provides expert analysis and commentary regarding the performance of new therapeutic and diagnostic modalities in clinical immunology. Members of the International Editorial Advisory Panel of Expert Review of Clinical Immunology are the forefront of their area of expertise. This panel works with our dedicated editorial team to identify the most important and topical review themes and the corresponding expert(s) most appropriate to provide commentary and analysis. All articles are subject to rigorous peer-review, and the finished reviews provide an essential contribution to decision-making in clinical immunology. Articles focus on the following key areas: • Therapeutic overviews of specific immunologic disorders highlighting optimal therapy and prospects for new medicines • Performance and benefits of newly approved therapeutic agents • New diagnostic approaches • Screening and patient stratification • Pharmacoeconomic studies • New therapeutic indications for existing therapies • Adverse effects, occurrence and reduction • Prospects for medicines in late-stage trials approaching regulatory approval • Novel treatment strategies • Epidemiological studies • Commentary and comparison of treatment guidelines Topics include infection and immunity, inflammation, host defense mechanisms, congenital and acquired immunodeficiencies, anaphylaxis and allergy, systemic immune diseases, organ-specific inflammatory diseases, transplantation immunology, endocrinology and diabetes, cancer immunology, neuroimmunology and hematological diseases.