Metadata | |
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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://ghr.nlm.nih.gov/condition/familial-hemophagocytic-lymphohistiocytosis, http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31801997000500007&lng=pt&nrm=iso |
Xrefs |
SNOMEDCT_US_2023_03_01:190958003 |
Alternateids |
DOID:6453 |
Subsets |
DO_rare_slim NCIthesaurus |
Synonyms |
haemophagocytic syndrome [EXACT] |
Parent Relationships |