| Preferred Name |
hemophagocytic lymphohistiocytosis |
| Synonyms |
haemophagocytic syndrome |
| Definitions |
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. Xref MGI. MESH:C537250 added from NeuroDevNet [WAK]. |
| ID |
http://purl.obolibrary.org/obo/DOID_0050120 |
| comment |
Xref MGI. MESH:C537250 added from NeuroDevNet [WAK]. |
| database_cross_reference |
GARD:6589 NCI:C34792 MESH:D051359 UMLS_CUI:C0024291 OMIM:PS267700 ICD10CM:D76.1 SNOMEDCT_US_2022_09_01:190958003 ORDO:540 |
| 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. |
| has exact synonym |
haemophagocytic syndrome |
| has symptom |
http://purl.obolibrary.org/obo/SYMP_0000521 |
| has_alternative_id |
DOID:6453 |
| has_obo_namespace |
disease_ontology |
| id |
DOID:0050120 |
| in_subset | |
| label |
hemophagocytic lymphohistiocytosis |
| notation |
DOID:0050120 |
| prefLabel |
hemophagocytic lymphohistiocytosis |
| subClassOf |