Evolving practice: X-linked agammaglobulinemia and lung transplantation

Abstract

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). This case series examines six patients with X-linked agammaglobulinemia and tracks their post-lung transplant course to highlight the challenges of immunosuppression and infection as well as chronic lung allograft dysfunction in these patients.