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Lookup NU author(s): Dr Ben ShillitoeORCiD, Professor Andrew GenneryORCiD
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0).
Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc.PURPOSE OF REVIEW: The clinical outcomes from COVID-19 in monogenic causes of predominant antibody deficiency have pivotal implications for our understanding of the antiviral contribution of humoral immunity. This review summarizes the lessons learned from COVID-19 infection in X-linked agammaglobulinemia (XLA) due to genetic defects in Bruton's tyrosine kinase (BTK). RECENT FINDINGS: Key molecular pathways underlying the development of severe COVID-19 are emerging, highlighting the possible contribution of BTK to hyperinflammation. SARS-CoV-2 specific T-cell responses and complement activation appear insufficient to achieve viral clearance in some B-cell deficient individuals. Whilst appearing efficacious in this group, use of convalescent plasma has been recently associated with the evolution of viral escape variants. Early data suggests individuals with XLA can mount a viral-specific T-cell vaccine response, however, the clinical significance of this is still emerging. SUMMARY: In contrast to reports made early in the pandemic, we show XLA patients remain susceptible to severe disease. Persistent infection was common and is likely to carry a significant symptom burden and risk of novel variant evolution. COVID-19 infection in this vulnerable, antibody deficient group due to genetic, therapeutic or disease causes may require prompt and specific intervention for both patient and societal benefit.
Author(s): Ponsford MJ, Shillitoe BMJ, Humphreys IR, Gennery AR, Jolles S
Publication type: Article
Publication status: Published
Journal: Current Opinion in Allergy and Clinical Immunology
Year: 2021
Volume: 21
Issue: 6
Pages: 525-534
Print publication date: 01/12/2021
Acceptance date: 02/04/2020
Date deposited: 04/01/2022
ISSN (print): 1528-4050
ISSN (electronic): 1473-6322
Publisher: Lippincott Williams & Wilkins
URL: https://doi.org/10.1097/ACI.0000000000000792
DOI: 10.1097/ACI.0000000000000792
PubMed id: 34596095
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