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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 and Gennery, Andrew R. 2021. Haematopoietic stem cell transplant for norovirus-induced intestinal failure in X-linked agammaglobulinemia. Journal of Clinical Immunology 41 , 1574 -1581. 10.1007/s10875-021-01088-2

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Abstract

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.

Item Type: Article
Date Type: Publication
Status: Published
Schools: Medicine
Additional Information: This article is licensed under a Creative Commons Attribution 4.0 International License
Publisher: Springer
ISSN: 0271-9142
Date of First Compliant Deposit: 6 July 2021
Date of Acceptance: 14 June 2021
Last Modified: 11 Oct 2021 14:02
URI: http://orca.cardiff.ac.uk/id/eprint/142335

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