Evolving practice: X-linked agammaglobulinemia and lung transplantation

S. Barnes, S. Kotecha, J. A. Douglass, E. Paul, F. Hore-Lacey, R. Stirling, G. I. Snell, G. P. Westall

Research output: Contribution to journalArticleOtherpeer-review

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.

Original languageEnglish
Pages (from-to)1110-1113
Number of pages4
JournalAmerican Journal of Transplantation
Volume15
Issue number4
DOIs
Publication statusPublished - 1 Apr 2015

Keywords

  • clinical research
  • immune modulation
  • immunosuppression
  • immunosuppressive regimens
  • lung transplantation
  • practice
  • pulmonology, rejection: chronic

Cite this

Barnes, S. ; Kotecha, S. ; Douglass, J. A. ; Paul, E. ; Hore-Lacey, F. ; Stirling, R. ; Snell, G. I. ; Westall, G. P. / Evolving practice : X-linked agammaglobulinemia and lung transplantation. In: American Journal of Transplantation. 2015 ; Vol. 15, No. 4. pp. 1110-1113.
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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.",
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Evolving practice : X-linked agammaglobulinemia and lung transplantation. / Barnes, S.; Kotecha, S.; Douglass, J. A.; Paul, E.; Hore-Lacey, F.; Stirling, R.; Snell, G. I.; Westall, G. P.

In: American Journal of Transplantation, Vol. 15, No. 4, 01.04.2015, p. 1110-1113.

Research output: Contribution to journalArticleOtherpeer-review

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AU - Barnes, S.

AU - Kotecha, S.

AU - Douglass, J. A.

AU - Paul, E.

AU - Hore-Lacey, F.

AU - Stirling, R.

AU - Snell, G. I.

AU - Westall, G. P.

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AB - 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.

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