Stem cell transplantation as treatment for major histocompatibility class I deficiency

Major histocompatibility class I deficiency, due to genetic lesions in TAP1, TAP2, TAPBP, or B2M, manifests with recurrent sinopulmonary infections and granulomatous skin ulceration, is predominately treated antimicrobial prophylaxis chest physiotherapy. One previous report of hematopoietic stem cell transplantation has been described the literature, demonstrating cure immune defect without significant graft-versus-host disease. In this report, we expand literature on HSCT MHC-I deficiency follow-up original patient, maintained resolution normal function regression rash 15 years post-transplant, describe a further patient mycobacterial disease whose transplant course was complicated by severe