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Lookup NU author(s): Professor Andrew GenneryORCiD
This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).
© 2018 Ghosh, Drexler, Bhatia, Gennery and Borkhardt. Patients with primary immunodeficiency can be prone to severe Epstein-Barr virus (EBV) associated immune dysregulation. Individuals with mutations in the interleukin-2-inducible T-cell kinase (ITK) gene experience Hodgkin and non-Hodgkin lymphoma, EBV lymphoproliferative disease, hemophagocytic lymphohistiocytosis, and dysgammaglobulinemia. In this review, we give an update on further reported patients. We believe that current clinical data advocate early definitive treatment by hematopoietic stem cell transplantation, as transplant outcome in primary immunodeficiency disorders in general has gradually improved in recent years. Furthermore, we summarize experimental data in the murine model to provide further insight of pathophysiology in ITK deficiency.
Author(s): Ghosh S, Drexler I, Bhatia S, Gennery AR, Borkhardt A
Publication type: Article
Publication status: Published
Journal: Frontiers in Immunology
Online publication date: 08/05/2018
Acceptance date: 20/04/2018
Date deposited: 14/08/2018
ISSN (electronic): 1664-3224
Publisher: Frontiers Research Foundation
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