1. BAFF-associated granulomatous lung disease in a patient with GATA2 deficiency
- Author
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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, and Yusei Ohshima, MD, PhD
- Subjects
B-cell–activating factor ,common variable immunodeficiency ,GATA 2 ,granuloma ,lung disease ,Immunologic diseases. Allergy ,RC581-607 - 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.
- Published
- 2024
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