Familial hemophagocytic lymphohistiocytosis

Orpha code: 540OMIM code: 618998

Definition

Familial Hemophagocytic lymphohistiocytosis (FHL) is a rare primary immunodeficiency characterized by a macrophage activation syndrome (see this term) with an onset usually occurring within a few months or less common several years after birth.

Disease data
Classification

Disease

Synonyms
Familial HLH
Rodzinna HLH
ORPHA code
540
OMIM code
618998
ICD10 code
D76.1
ICD11 code
4A01.23

No additional description.

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