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

Lookup NU author(s): Dr Ben Shillitoe, Professor Mary Slatter, Professor Andrew GenneryORCiD

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This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).

Abstract

© 2021, The Author(s). 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.

Publication metadata

Author(s): Shillitoe BMJ, Ponsford M, Slatter MA, Evans J, Struik S, Cosgrove M, Doull I, Jolles S, Gennery AR

Publication type: Article

Publication status: Published

Journal: Journal of Clinical Immunology

Year: 2021

Volume: 41

Pages: 1574-1581

Print publication date: 01/10/2021

Online publication date: 23/06/2021

Acceptance date: 14/06/2021

Date deposited: 08/07/2021

ISSN (print): 0271-9142

ISSN (electronic): 1573-2592

Publisher: Springer Nature

URL: https://doi.org/10.1007/s10875-021-01088-2

DOI: 10.1007/s10875-021-01088-2

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