一名 GATA2 缺乏症患者的 BAFF 相关肉芽肿性肺病

The journal of allergy and clinical immunology. Global Pub Date : 2024-08-28 DOI:10.1016/j.jacig.2024.100336

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

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引用次数: 0

摘要

一名 GATA2 缺乏症患者尽管出现了单核细胞减少，但却患上了皮质类固醇反应性无菌肉芽肿肺病。患者出现 B 淋巴细胞减少、自身免疫、血清 B 细胞激活因子水平升高和肺浆细胞浸润，这些因素共同提示其潜在机制类似于合并变异性免疫缺陷肺病。

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

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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来源期刊

The journal of allergy and clinical immunology. Global Immunology, Allergology and Rheumatology

CiteScore

0.70

自引率

0.00%

发文量

审稿时长

92 days