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.
BAFF-associated granulomatous lung disease in a patient with GATA2 deficiency.
GATA2 缺陷患者发生 BAFF 相关肉芽肿性肺病
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作者：Yasutomi-Sakai Motoko, Hayashi Taihei, Suzuki Koji, Akeda Hayaki, Maeda Yumekichi, Okada Akitoshi, Takeda Toshihiro, Watanabe Yasuhiro, Imamura Yoshiaki, Ohshima Yusei
| 期刊： | Journal of Allergy and Clinical Immunology Global | 影响因子： | 0.000 |
| 时间： | 2024 | 起止号： | 2024 Aug 28; 3(4):100336 |
| doi： | 10.1016/j.jacig.2024.100336 | 研究方向： | 其它 |
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