Evolving Practice: X‐Linked Agammaglobulinemia and Lung Transplantation

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).

[1]  G. Verleden,et al.  A new classification system for chronic lung allograft dysfunction. , 2014, The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation.

[2]  E. Mckenzie,et al.  Hypogammaglobulinemia: Incidence, risk factors, and outcomes following pediatric lung transplantation , 2009, Pediatric transplantation.

[3]  F. D'Ovidio,et al.  Immunoglobulin G levels before and after lung transplantation. , 2006, American journal of respiratory and critical care medicine.

[4]  Gerald J Berry,et al.  Revision of the 1996 working formulation for the standardization of nomenclature in the diagnosis of lung rejection. , 2005, The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation.

[5]  J. Sonett,et al.  Risk Factors and Outcomes of Hypogammaglobulinemia after Lung Transplantation , 2005, Transplantation.

[6]  A. Solé,et al.  Lung transplantation in patients with x-linked agammaglobulinemia. , 2003, Transplantation proceedings.

[7]  Marshall Hertz,et al.  Bronchiolitis obliterans syndrome 2001: an update of the diagnostic criteria. , 2002, The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation.

[8]  G. Snell,et al.  The Alfred Hospital Lung Transplant Experience. , 2014, Clinical transplants.

[9]  P. Hopkins,et al.  Bronchiolitis obliterans syndrome, hypogammaglobulinemia, and infectious complications of lung transplantation. , 2013, The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation.