IFN-γ and CD25 drive distinct pathological features during hemophagocytic lymphohistiocytosis

Studying the pathogenesis of hemophagocytic lymphohistiocyosis, we found a striking dichotomy between the hematological and inflammatory components of HLH. Hematological features are strictly dependent of IFN-γ, while inflammation is driven by dysregulated IL-2 signaling.

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Bibliographic Details
Published in:Journal of allergy and clinical immunology 2018-12, Vol.143 (6), p.2215-2226.e7
Main Authors: Humblet-Baron, Stéphanie, Franckaert, Dean, Dooley, James, Ailal, Fatima, Bousfiha, Aziz, Deswarte, Caroline, Oleaga-Quintas, Carmen, Casanova, Jean-Laurent, Bustamante, Jacinta, Liston, Adrian
Format: Article
Language:English
Online Access:Get full text
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Summary:Studying the pathogenesis of hemophagocytic lymphohistiocyosis, we found a striking dichotomy between the hematological and inflammatory components of HLH. Hematological features are strictly dependent of IFN-γ, while inflammation is driven by dysregulated IL-2 signaling.
ISSN:0091-6749
1097-6825
DOI:10.1016/j.jaci.2018.10.068