| Metadata | |
|---|---|
| ID | DOID:0050120 |
| Name | hemophagocytic lymphohistiocytosis |
| 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. http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31801997000500007&lng=pt&nrm=iso, http://ghr.nlm.nih.gov/condition/familial-hemophagocytic-lymphohistiocytosis |
| Xrefs |
SNOMEDCT_US_2023_03_01:190958003 |
| Alternateids |
DOID:6453 |
| Subsets |
DO_rare_slim NCIthesaurus |
| Synonyms |
haemophagocytic syndrome [EXACT] |
| Parent Relationships | |
| Subclass Logical Relationships |
has symptom some lymphadenopathy has symptom some enlargement of lymph nodes has symptom some hepatosplenomegaly |