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Disease Ontology Browser
hemophagocytic lymphohistiocytosis (DOID:0050120)
Synonyms: haemophagocytic syndrome; Hemophagocytic lymphohistiocytosis, familial, 2; Hemophagocytic syndrome; HPS
Alt IDs: OMIM:267700, OMIM:603552, OMIM:603553, OMIM:608898, OMIM:613101, DOID:6453, ICD10CM:D76.1, MESH:D051359, NCI:C34792, UMLS_CUI:C0024291
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.

Disease References using Mouse Models (7)

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
03/14/2017
MGI 6.08
The Jackson Laboratory