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Article

Genetics of Familial Hemophagocytic Lymphohistiocytosis (HLH).

Hematology/oncology clinics of North America - 1 Jun 2025

Zhang Kejian, Meyer Lauren K, Machowicz Rafal, Coniglio Maria Luisa, Sieni Elena, Nichols Kim E

Abstract excerpt

Familial hemophagocytic lymphohistiocytosis (fHLH) represents a group of rare, inherited immune system disorders characterized by uncontrolled inflammatory responses. fHLH results from genetic mutations that impair CD8 T cell and natural killer cell cytotoxicity. Without treatment, fHLH is commonly fatal, but early diagnosis and treatment, including immunosuppressive therapy, and in many cases, an allogeneic...

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Identifiers

PMID: 40199664
DOI: 10.1016/j.hoc.2025.02.002

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