Functional and genetic testing in adults with HLH reveals an inflammatory profile rather than a cytotoxicity defect

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory condition. Primary HLH occurs early in life as a result of monogenic biallelic mutations affecting lymphocyte cytotoxicity. Secondary HLH occurs mostly in adults secondary to infection, lymphoma, or rheumatic disease. I...

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Veröffentlicht in:Blood
Hauptverfasser: Carvelli, Julien, Piperoglou, Christelle, Farnarier, Catherine, Vely, Frédéric, Mazodier, Karin, Audonnet, Sandra, Nitschke, Patrick, Bole-Feysot, Christine, Boucekine, Mohamed, Cambon, Audrey, Hamidou, Mohamed, Harle, Jean-Robert, de Saint Basile, Geneviève, Kaplanski, Gilles
Format: Artigo
Sprache:Inglês
Veröffentlicht: American Society of Hematology 2020
Schlagworte:
Clinical Trials and Observations
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC7530375/
https://ncbi.nlm.nih.gov/pubmed/32356861
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1182/blood.2019003664
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