HLH as an additional warning sign of inborn errors of immunity beyond familial-HLH in children: a systematic review

HLH as an additional warning sign of inborn errors of immunity beyond familial-HLH in children: a systematic review

BackgroundHemophagocytic Lymphohistiocytosis (HLH) is a rare and life-threatening condition characterized by a severe impairment of the immune homeostasis. While Familial-HLH (FHL) is a known cause, the involvement of other Inborn Errors of Immunity (IEI) in pediatric-HLH remains understudied.Object...

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Bibliographic Details
Main Authors: Silvia Ricci, Walter Maria Sarli, Lorenzo Lodi, Clementina Canessa, Francesca Lippi, Donata Dini, Marta Ferrari, Laura Pisano, Elena Sieni, Giuseppe Indolfi, Massimo Resti, Chiara Azzari
Format: Article
Language:English
Published: Frontiers Media S.A. 2024-02-01
Series:Frontiers in Immunology
Subjects:
hemophagocytic lymphohistiocytosis
inborn errors of immunity
macrophage activation syndrome
immune deficiency
familial hemophagocytic lymphohistiocytosis
hemophagocytic syndrome
Online Access:https://www.frontiersin.org/articles/10.3389/fimmu.2024.1282804/full

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https://www.frontiersin.org/articles/10.3389/fimmu.2024.1282804/full

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