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Summary Literature (0)
DOID:0050120 - hemophagocytic lymphohistiocytosis


Disease Ontology 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.

Synonyms: HPS, Hemophagocytic syndrome, haemophagocytic syndrome,

Xenbase Genes : stxbp2, stx11, unc13d, prf1

Mondo Disease Ontology (on Monarch Initiative):
MONDO:0015540 - hemophagocytic syndrome


Other Model Organisms: AGR, MGI, ZFIN, FlyBase, WormBase, RGD

Ontology Viewers: Disease Ontology, EMBL-EBI, OLSVis tree view, Ontobee
Parent(s): lymphatic system disease (is_a)