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Interleukin-2-inducible T-cell kinase deficiency-new patients, new insight?

Lookup NU author(s): 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

© 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.

Publication metadata

Author(s): Ghosh S, Drexler I, Bhatia S, Gennery AR, Borkhardt A

Publication type: Article

Publication status: Published

Journal: Frontiers in Immunology

Year: 2018

Volume: 9

Online publication date: 08/05/2018

Acceptance date: 20/04/2018

Date deposited: 14/08/2018

ISSN (electronic): 1664-3224

Publisher: Frontiers Research Foundation

URL: https://doi.org/10.3389/fimmu.2018.00979

DOI: 10.3389/fimmu.2018.00979

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