Wiskott-Aldrich综合征合并igg4相关硬化疾病1例报告及文献复习

IF 2.1 4区 医学 Q3 ALLERGY
Allergologia et immunopathologia Pub Date : 2025-09-01 eCollection Date: 2025-01-01 DOI:10.15586/aei.v53i5.1437
Seda Altiner, Makbule Seda Bayrak Durmaz
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引用次数: 0

摘要

Wiskott-Aldrich综合征(WAS)是一种以湿疹、微血小板减少症和复发性感染为特征的x连锁免疫缺陷。免疫球蛋白g4相关疾病(IgG4-RD)是一种累及多器官的纤维炎性疾病。我们报告一位34岁男性WAS患者发展为颈部淋巴结病变及腮腺肿大。最初的活组织检查不确定,影像学提示多形性腺瘤。鉴于持续的宫颈淋巴结病和潜在的免疫缺陷,淋巴瘤也被考虑在鉴别诊断。然而,切除的唾腺和淋巴结的组织病理学检查显示淋巴浆细胞和嗜酸性粒细胞浸润,大量igg4阳性浆细胞,层状纤维化和闭塞性静脉炎,与IgG4-RD一致。患者对强的松治疗反应良好。本病例强调了考虑IgG4-RD在免疫缺陷个体淋巴增生性病变鉴别诊断中的重要性,并强调了切除组织标本的组织病理学评估的诊断价值。据我们所知,这是一种罕见的WAS和IgG4-RD共存的情况,以前没有文献报道过。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Wiskott-Aldrich syndrome complicated with IgG4-related Sclerosing disease: A case report and literature review.

Wiskott-Aldrich Syndrome (WAS) is an X-linked immunodeficiency characterized by eczema, microthrombocytopenia, and recurrent infections. Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory disorder involving various organs. We present a 34-year-old male with WAS who developed cervical lymphadenopathy and parotid gland swelling. Initial biopsies were inconclusive and imaging suggested pleomorphic adenoma. Given the persistent cervical lymphadenopathy and the underlying immunodeficiency, lymphoma was also considered in the differential diagnosis. However, histopathological examination of excised salivary gland and lymph nodes revealed lymphoplasmacytic and eosinophilic infiltrates, numerous IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis, consistent with IgG4-RD. The patient responded well to prednisone therapy. This case emphasizes the importance of considering IgG4-RD in the differential diagnosis of lymphoproliferative lesions in immunodeficient individuals and highlights the diagnostic value of histopathological evaluation in excisional tissue specimens. To our knowledge, this represents a rare coexistence of WAS and IgG4-RD not previously reported in the literature.

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来源期刊
CiteScore
3.70
自引率
0.00%
发文量
131
审稿时长
6-12 weeks
期刊介绍: Founded in 1972 by Professor A. Oehling, Allergologia et Immunopathologia is a forum for those working in the field of pediatric asthma, allergy and immunology. Manuscripts related to clinical, epidemiological and experimental allergy and immunopathology related to childhood will be considered for publication. Allergologia et Immunopathologia is the official journal of the Spanish Society of Pediatric Allergy and Clinical Immunology (SEICAP) and also of the Latin American Society of Immunodeficiencies (LASID). It has and independent international Editorial Committee which submits received papers for peer-reviewing by international experts. The journal accepts original and review articles from all over the world, together with consensus statements from the aforementioned societies. Occasionally, the opinion of an expert on a burning topic is published in the "Point of View" section. Letters to the Editor on previously published papers are welcomed. Allergologia et Immunopathologia publishes 6 issues per year and is included in the major databases such as Pubmed, Scopus, Web of Knowledge, etc.
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