DO_rare_slim NCIthesaurus has symptom hemophagocytic lymphohistiocytosis ORDO:540 ICD10CM:D76.1 MESH:D051359 MIM:PS267700 DOID:0050120 disease_ontology DOID:6453 NCI:C34792 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. SNOMEDCT_US_2025_09_01:190958003 GARD:6589 UMLS_CUI:C0024291 Xref MGI. MESH:C537250 added from NeuroDevNet [WAK]. haemophagocytic syndrome lymphatic system disease hepatosplenomegaly enlargement of lymph nodes lymphadenopathy