Familial hemophagocytic lymphohistiocytosis is a lethal disorder of immune regulation. It is a disorder of early childhood characterized by excessive, uncontrolled T-lymphocyte and macrophage activation. Infiltration of the liver, spleen, bone marrow, and central nervous system by activated T cells and macrophages results in a multisystem disorder.
HPLH4, HLH; Familial erythrophagocytic lymphohistiocytosis 4; Familial histiocytic reticulosis 4; Hemophagocytic lymphohistiocytosis
- Phagocytic disorders
- Familial haemophagocytic lymphyohistiocytosis
Phenotypically related immunodeficiencies