Cargando…

Haematopoietic Stem Cell Transplant for Norovirus-Induced Intestinal Failure in X-linked Agammaglobulinemia

Since the first clinical description in 1952, immunoglobulin replacement therapy remains the mainstay of treatment of patients with X-linked agammaglobulinemia (XLA). However, this therapy only replaces IgG isotype and does not compensate for the loss of Bruton tyrosine kinase in non-B-lymphocytes....

Descripción completa

Detalles Bibliográficos
Autores principales: Shillitoe, Ben M. J., Ponsford, Mark, Slatter, Mary A., Evans, Jennifer, Struik, Siske, Cosgrove, Mike, Doull, Iolo, Jolles, Stephen, Gennery, Andrew R.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer US 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8221090/
https://www.ncbi.nlm.nih.gov/pubmed/34164761
http://dx.doi.org/10.1007/s10875-021-01088-2
_version_ 1783711265458225152
author Shillitoe, Ben M. J.
Ponsford, Mark
Slatter, Mary A.
Evans, Jennifer
Struik, Siske
Cosgrove, Mike
Doull, Iolo
Jolles, Stephen
Gennery, Andrew R.
author_facet Shillitoe, Ben M. J.
Ponsford, Mark
Slatter, Mary A.
Evans, Jennifer
Struik, Siske
Cosgrove, Mike
Doull, Iolo
Jolles, Stephen
Gennery, Andrew R.
author_sort Shillitoe, Ben M. J.
collection PubMed
description Since the first clinical description in 1952, immunoglobulin replacement therapy remains the mainstay of treatment of patients with X-linked agammaglobulinemia (XLA). However, this therapy only replaces IgG isotype and does not compensate for the loss of Bruton tyrosine kinase in non-B-lymphocytes. Patients may still therefore develop complications despite current standard of care. Here, we describe an XLA patient with persistent chronic norovirus infection, refractory to treatment and causing intestinal failure. The patient underwent haematopoietic stem cell transplantation, curing XLA and allowed clearance of norovirus prior to humoral immunoreconstitution, suggesting non-humoral immunodeficiency in these patients.
format Online
Article
Text
id pubmed-8221090
institution National Center for Biotechnology Information
language English
publishDate 2021
publisher Springer US
record_format MEDLINE/PubMed
spelling pubmed-82210902021-06-23 Haematopoietic Stem Cell Transplant for Norovirus-Induced Intestinal Failure in X-linked Agammaglobulinemia Shillitoe, Ben M. J. Ponsford, Mark Slatter, Mary A. Evans, Jennifer Struik, Siske Cosgrove, Mike Doull, Iolo Jolles, Stephen Gennery, Andrew R. J Clin Immunol Original Article Since the first clinical description in 1952, immunoglobulin replacement therapy remains the mainstay of treatment of patients with X-linked agammaglobulinemia (XLA). However, this therapy only replaces IgG isotype and does not compensate for the loss of Bruton tyrosine kinase in non-B-lymphocytes. Patients may still therefore develop complications despite current standard of care. Here, we describe an XLA patient with persistent chronic norovirus infection, refractory to treatment and causing intestinal failure. The patient underwent haematopoietic stem cell transplantation, curing XLA and allowed clearance of norovirus prior to humoral immunoreconstitution, suggesting non-humoral immunodeficiency in these patients. Springer US 2021-06-23 2021 /pmc/articles/PMC8221090/ /pubmed/34164761 http://dx.doi.org/10.1007/s10875-021-01088-2 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Original Article
Shillitoe, Ben M. J.
Ponsford, Mark
Slatter, Mary A.
Evans, Jennifer
Struik, Siske
Cosgrove, Mike
Doull, Iolo
Jolles, Stephen
Gennery, Andrew R.
Haematopoietic Stem Cell Transplant for Norovirus-Induced Intestinal Failure in X-linked Agammaglobulinemia
title Haematopoietic Stem Cell Transplant for Norovirus-Induced Intestinal Failure in X-linked Agammaglobulinemia
title_full Haematopoietic Stem Cell Transplant for Norovirus-Induced Intestinal Failure in X-linked Agammaglobulinemia
title_fullStr Haematopoietic Stem Cell Transplant for Norovirus-Induced Intestinal Failure in X-linked Agammaglobulinemia
title_full_unstemmed Haematopoietic Stem Cell Transplant for Norovirus-Induced Intestinal Failure in X-linked Agammaglobulinemia
title_short Haematopoietic Stem Cell Transplant for Norovirus-Induced Intestinal Failure in X-linked Agammaglobulinemia
title_sort haematopoietic stem cell transplant for norovirus-induced intestinal failure in x-linked agammaglobulinemia
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8221090/
https://www.ncbi.nlm.nih.gov/pubmed/34164761
http://dx.doi.org/10.1007/s10875-021-01088-2
work_keys_str_mv AT shillitoebenmj haematopoieticstemcelltransplantfornorovirusinducedintestinalfailureinxlinkedagammaglobulinemia
AT ponsfordmark haematopoieticstemcelltransplantfornorovirusinducedintestinalfailureinxlinkedagammaglobulinemia
AT slattermarya haematopoieticstemcelltransplantfornorovirusinducedintestinalfailureinxlinkedagammaglobulinemia
AT evansjennifer haematopoieticstemcelltransplantfornorovirusinducedintestinalfailureinxlinkedagammaglobulinemia
AT struiksiske haematopoieticstemcelltransplantfornorovirusinducedintestinalfailureinxlinkedagammaglobulinemia
AT cosgrovemike haematopoieticstemcelltransplantfornorovirusinducedintestinalfailureinxlinkedagammaglobulinemia
AT doulliolo haematopoieticstemcelltransplantfornorovirusinducedintestinalfailureinxlinkedagammaglobulinemia
AT jollesstephen haematopoieticstemcelltransplantfornorovirusinducedintestinalfailureinxlinkedagammaglobulinemia
AT genneryandrewr haematopoieticstemcelltransplantfornorovirusinducedintestinalfailureinxlinkedagammaglobulinemia