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Histological Quantification to Determine Lung Fungal Burden in Experimental Aspergillosis
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BAFF-associated granulomatous lung disease in a patient with GATA2 deficiency
Motoko Yasutomi-Sakai1, Taihei Hayashi1, Koji Suzuki1
1Department of Pediatrics, Faculty of Medical Sciences, University of Fukui, Fukui, Japan.
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.
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