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

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

Journal of Allergy and Clinical Immunology: Global (Nov 2024)

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

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

Affiliations

Motoko Yasutomi-Sakai, MD, PhD: Department of Pediatrics, Faculty of Medical Sciences, University of Fukui, Fukui, Japan; Corresponding author: Motoko Yasutomi-Sakai, MD, PhD, Department of Pediatrics, Faculty of Medical Sciences, University of Fukui, 23-3 Shimoaizuki, Matsuoka Eiheiji-cho Yoshida-gun, Fukui 9101193, Japan.
Taihei Hayashi, MD, PhD: Department of Pediatrics, Faculty of Medical Sciences, University of Fukui, Fukui, Japan
Koji Suzuki, MD, PhD: Department of Pediatrics, Faculty of Medical Sciences, University of Fukui, Fukui, Japan
Hayaki Akeda, MD: Department of Pediatrics, Faculty of Medical Sciences, University of Fukui, Fukui, Japan
Yumekichi Maeda, MD, PhD: Department of Pediatrics, Faculty of Medical Sciences, University of Fukui, Fukui, Japan
Akitoshi Okada, MD: Division of Thoracic Surgery, Second Department of Surgery, University of Fukui, Fukui, Japan
Toshihiro Takeda, MD: Third Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Fukui, Japan
Yasuhiro Watanabe, MD: Department of Pediatrics, Fukui Red Cross Hospital, Fukui, Japan
Yoshiaki Imamura, MD, PhD: Division of Diagnostic Pathology/Surgical Pathology, University of Fukui Hospital, Fukui, Japan
Yusei Ohshima, MD, PhD: Department of Pediatrics, Faculty of Medical Sciences, University of Fukui, Fukui, Japan

Journal volume & issue: Vol. 3, no. 4
p. 100336

Abstract

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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 in Journal of Allergy and Clinical Immunology: Global

ISSN: 2772-8293 (Online)
Publisher: Elsevier
Country of publisher: United States
LCC subjects: Medicine: Internal medicine: Specialties of internal medicine: Immunologic diseases. Allergy
Website: https://www.sciencedirect.com/journal/journal-of-allergy-and-clinical-immunology-global

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Abstract

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