Burns reports a grant from HEFCE and consulting fees or honoraria from CSL Behring employment by University College London grants/grants pending from NIHR, UCLH III BRC, and GOSH/ICH BRC and travel/accommodations/meeting expenses from Immunodeficiency Canada/IAACI, CSL Behring, Baxalta, and Octagam. Lango Allen reports employment at the University of Cambridge. Good Endowment, and the University of South Florida.ĭisclosure of potential conflict of interest: J.

Supported in part by National Institute of Allergy and Infectious Diseases/National Institutes of Health grant 5K08AI103035 (to J.E.W.), the Jeffrey Modell Foundation (to J.E.W.), the Robert A. Two of 4 patients had a significant decrease, indicating a variable degree of lung function in adult patients with RAG deficiency (see the Methods section and Fig E1, G, in this article's Online Repository).

We performed a retrospective analysis of pulmonary function tests over 2 or more years to assess the decrease in respiratory function. Pulmonary lung function data revealed a median forced vital capacity of 79.55%, diffusing capacity of the lungs for carbon monoxide of 75%, and FEV 1/forced vital capacity ratio of 78%. Patient 1 had peribronchial fibrosis ( Fig 2, B). Germinal center formations in patient 3 were comprised of CD3 + T cells and CD20 + B cells.

Histology of lung biopsy specimens (patients 1 and 3) revealed atypical lymphoid hyperplasia with granulomatous features and giant cell formation ( Fig 2, B). High-resolution computed tomographic imaging of the lung revealed bronchiectasis and granuloma. Progressive pulmonary diseases were also the leading concern for successful hematopoietic stem cell transplantation (HSCT). Diversity, Equity, and Inclusion Initiatives.

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