/familial hemophagocytic lymphohistiocytosis type 1

familial hemophagocytic lymphohistiocytosis type 1

Rare Disease

MONDO:0009974

Also Known As

familial hemophagocytic lymphohistiocytosisFHL1HLH1HPLH1familial HLHfamilial hemophagocytic lymphohistiocytosis type 1Erythrophagocytic lymphohistiocytosis, familialHlh1Hplh1familial hemophagocytic lymphohistiocytosis 1hemophagocytic lymphohistiocytosis, familialhemophagocytic lymphohistiocytosis, familial, 1hemophagocytic reticulosis, familialreticulosis, familial histiocytic

Definition

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