hemophagocytic lymphohistiocytosis (DOID:0050120)
Alliance: disease page
Synonyms: haemophagocytic syndrome
Alt IDs: DOID:6453, ICD10CM:D76.1, MESH:D051359, NCI:C34792, OMIM:PS267700, ORDO:540, UMLS_CUI:C0024291
Definition: A lymphatic system disease that is characterized by an expansion of the monocyte-macrophage population and intense hemophagocytosis. It can occur de novo, but more often occurs in the setting of another disorder, usually an infection or a malignancy. A clinical picture of fever, hepatosplenomegaly, lymphadenopathy and peripheral pancytopenia. The morphologic hallmark of this syndrome is the phagocytosis of hematopoietic elements by morphologically normal macrophages.