BAFF-associated granulomatous lung disease in a patient with GATA2 deficiency

Autor: 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
Jazyk: angličtina
Rok vydání: 2024
Předmět:
Zdroj: Journal of Allergy and Clinical Immunology: Global, Vol 3, Iss 4, Pp 100336- (2024)
Druh dokumentu: article
ISSN: 2772-8293
DOI: 10.1016/j.jacig.2024.100336
Popis: 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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