{"title":"Wiskott-Aldrich综合征合并igg4相关硬化疾病1例报告及文献复习","authors":"Seda Altiner, Makbule Seda Bayrak Durmaz","doi":"10.15586/aei.v53i5.1437","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":7536,"journal":{"name":"Allergologia et immunopathologia","volume":"53 5","pages":"138-142"},"PeriodicalIF":2.1000,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Wiskott-Aldrich syndrome complicated with IgG4-related Sclerosing disease: A case report and literature review.\",\"authors\":\"Seda Altiner, Makbule Seda Bayrak Durmaz\",\"doi\":\"10.15586/aei.v53i5.1437\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":7536,\"journal\":{\"name\":\"Allergologia et immunopathologia\",\"volume\":\"53 5\",\"pages\":\"138-142\"},\"PeriodicalIF\":2.1000,\"publicationDate\":\"2025-09-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Allergologia et immunopathologia\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.15586/aei.v53i5.1437\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q3\",\"JCRName\":\"ALLERGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Allergologia et immunopathologia","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.15586/aei.v53i5.1437","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/1/1 0:00:00","PubModel":"eCollection","JCR":"Q3","JCRName":"ALLERGY","Score":null,"Total":0}
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.
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.