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Publication — IRIC

Combined IFN-γ and JAK inhibition to treat hemophagocytic lymphohistiocytosis in mice.

Familial hemophagocytic lymphohistiocytosis is a life-threatening hyperinflammatory disease caused by genetic defects in the granule-mediated cytotoxic pathway. Success of hematopoietic cell transplantation, the only cure, is correlated with the extent of disease control before transplantation. Unfortunately, disease refractoriness and toxicities to standard chemotherapy-based regimens are fatal in a fraction of patients. Novel targeted immunotherapies, such as IFN-γ blocking antibodies or ruxolitinib, a Janus kinase (JAK) 1/2 inhibitor, are promising but only partially effective at controlling disease.

Publication date
January 1, 2023
Principal Investigators
Joly JA, Vallée A, Bourdin B, Bourbonnais S, Patey N, Gaboury L, Théorêt Y, Decaluwe H
PubMed reference
J Allergy Clin Immunol 2023;151(1):247-259.e7
PubMed ID
Cytokines and Adaptive Immunity Laboratory, Sainte-Justine University Hospital Research Center, Université de Montréal, Montréal, Québec, Canada.