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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....
Autores principales: | , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer US
2021
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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 |
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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 |
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