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Ghosh, S.* ; Drexler, I.* ; Bhatia, S.* ; Adler, H. ; Gennery, A.R.* ; Borkhardt, A.*

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

Front. Immunol. 9:979 (2018)
Verlagsversion DOI PMC
Open Access Gold
Creative Commons Lizenzvertrag
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 () 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.
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Publikationstyp Artikel: Journalartikel
Dokumenttyp Review
Schlagwörter Epstein–barr Virus-related Malignancies ; Combined Immunodeficiency ; Interleukin-2-inducible T-cell Kinase ; Lymphoproliferative Disorders ; Primary Immunodeficiency
Sprache englisch
Veröffentlichungsjahr 2018
HGF-Berichtsjahr 2018
ISSN (print) / ISBN 1664-3224
e-ISSN 1664-3224
Zeitschrift Frontiers in Immunology
Quellenangaben Band: 9, Heft: , Seiten: , Artikelnummer: 979 Supplement: ,
Verlag Frontiers
Begutachtungsstatus Peer reviewed
Institut(e) Research Unit Lung Repair and Regeneration (LRR)
Institute of Lung Health and Immunity (LHI)
POF Topic(s) 30202 - Environmental Health
80000 - German Center for Lung Research
Forschungsfeld(er) Lung Research
PSP-Element(e) G-503100-005
G-501800-311
DOI 10.3389/fimmu.2018.00979
PubMed ID 29867957
Erfassungsdatum 2018-10-29
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