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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://ghr.nlm.nih.gov/condition/familial-hemophagocytic-lymphohistiocytosis, http://www.mayoclinicproceedings.com/inside.asp?AID=57&UID=, http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31801997000500007&lng=pt&nrm=iso
Xrefs

GARD:6589

ICD10CM:D76.1

MESH:D051359

NCI2004_11_17:C35439

NCI:C34792

OMIM:PS267700

ORDO:540

UMLS_CUI:C0024291

Alternateids

DOID:6453

Subsets

NCIthesaurus

Synonyms

haemophagocytic syndrome [EXACT]

Parent Relationships

is_a lymphatic system disease

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