Distinct severity of HLH in both human and murine mutants with complete loss of cytotoxic effector PRF1, RAB27A, and STX11

Inherited defects of granule-dependent cytotoxicity led to the life-threatening immune disorder hemophagocytic lymphohistiocytosis (HLH), characterized by uncontrolled CD8 T-cell and macrophage activation. In a cohort of HLH patients with genetic abnormalities expected to result in the complete abse...

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Detalhes bibliográficos
Main Authors: Sepulveda, Fernando E., Debeurme, Franck, Ménasché, Gaël, Kurowska, Mathieu, Côte, Marjorie, Schmid, Jana Pachlopnik, Fischer, Alain, de Saint Basile, Geneviève
Formato: Artigo
Idioma:Inglês
Publicado em: 2013
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3824285/
https://ncbi.nlm.nih.gov/pubmed/23160464
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1182/blood-2012-07-440339
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