X-linked agammaglobulinemia (XLA) is a rare primary humoral immunodeficiency syndrome characterized by agammaglobulinemia, recurrent infections and bronchiectasis. Despite the association with end-stage bronchiectasis, the literature on XLA and lung transplantation is extremely limited. We report a series of 6 XLA patients with bronchiectasis who underwent lung transplantation. Short-term outcomes were excellent however long-term outcomes were disappointing with a high incidence of pulmonary sepsis and chronic lung allograft dysfunction (CLAD).
Keywords: clinical research; immune modulation; immunosuppression; immunosuppressive regimens; lung transplantation; practice; pulmonology, rejection: chronic.
© Copyright 2015 The American Society of Transplantation and the American Society of Transplant Surgeons.