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Evolving Practice: X‐Linked Agammaglobulinemia and Lung Transplantation
Author(s) -
Barnes S.,
Kotecha S.,
Douglass J. A.,
Paul E.,
HoreLacey F.,
Stirling R.,
Snell G. I.,
Westall G. P.
Publication year - 2015
Publication title -
american journal of transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.89
H-Index - 188
eISSN - 1600-6143
pISSN - 1600-6135
DOI - 10.1111/ajt.13084
Subject(s) - bronchiectasis , medicine , x linked agammaglobulinemia , lung transplantation , immunodeficiency , primary immunodeficiency , lung , common variable immunodeficiency , transplantation , sepsis , incidence (geometry) , immunology , immune system , antibody , physics , receptor , tyrosine kinase , optics , bruton's tyrosine kinase
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).