Severe adult hemophagocytic lymphohistiocytosis (HLHa) correlates with HLH-related gene variants.

doi:10.1016/j.jaci.2023.07.023

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Article

Severe adult hemophagocytic lymphohistiocytosis (HLHa) correlates with HLH-related gene variants.

The Journal of allergy and clinical immunology - 1 Jan 2024

Bloch Coralie, Jais Jean Philippe, Gil Marine, Boubaya Marouane, Lepelletier Yves, Bader-Meunier Brigitte, Mahlaoui Nizar, Garcelon Nicolas, Lambotte Olivier, Launay David, Larroche Claire, Lazaro Estibaliz, Liffermann Francois, Lortholary Olivier, Michel Marc, Michot Jean-Marie, Morel Pierre, Cheminant Morgane, Suarez Felipe, Terriou Louis, Urbanski Geoffrey, Viallard Jean-Francois, Alcais Alexandre, Fischer Alain, de Saint Basile Geneviève, Hermine Olivier

Abstract excerpt

BACKGROUND: The contribution of genetic factors to the severity of adult hemophagocytic lymphohistiocytosis (HLHa) remains unclear. OBJECTIVE: We sought to assess a potential link between HLHa outcomes and HLH-related gene variants. METHODS: Clinical characteristics of 130 HLHa patients (age ≥ 18 years and HScore ≥ 169) and genotype of 8 HLH-related genes (LYST, PRF1, UNC13-D, STX11, STXBP2, RAB27A, XIAP, and...

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PMID: 37678575
DOI: 10.1016/j.jaci.2023.07.023

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