{"title":"BAFF-associated granulomatous lung disease in a patient with GATA2 deficiency","authors":"Motoko Yasutomi-Sakai MD, PhD , Taihei Hayashi MD, PhD , Koji Suzuki MD, PhD , Hayaki Akeda MD , Yumekichi Maeda MD, PhD , Akitoshi Okada MD , Toshihiro Takeda MD , Yasuhiro Watanabe MD , Yoshiaki Imamura MD, PhD , Yusei Ohshima MD, PhD","doi":"10.1016/j.jacig.2024.100336","DOIUrl":null,"url":null,"abstract":"<div><p>A patient with GATA2 deficiency developed corticosteroid-responsive sterile granulomatous lung disease despite monocytopenia. The presence of B-lymphopenia, autoimmunity, an elevated level of serum B-cell–activating factor, and pulmonary plasma cell infiltration, which together suggested an underlying mechanism similar to that of combined variable immunodeficiency lung disease.</p></div>","PeriodicalId":75041,"journal":{"name":"The journal of allergy and clinical immunology. Global","volume":"3 4","pages":"Article 100336"},"PeriodicalIF":0.0000,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2772829324001322/pdfft?md5=9faac18996a29cf60cb46ce01563dc80&pid=1-s2.0-S2772829324001322-main.pdf","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"The journal of allergy and clinical immunology. Global","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S2772829324001322","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
Abstract
A patient with GATA2 deficiency developed corticosteroid-responsive sterile granulomatous lung disease despite monocytopenia. The presence of B-lymphopenia, autoimmunity, an elevated level of serum B-cell–activating factor, and pulmonary plasma cell infiltration, which together suggested an underlying mechanism similar to that of combined variable immunodeficiency lung disease.
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