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hemophagocytic lymphohistiocytosis
Summary
- Synonym
-
- haemophagocytic syndrome
- 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.
- Super Class
- lymphatic system disease
External Links
- Disease Ontology
- DOID:0050120
- Mondo Disease Ontology
- MeSH
- UMLS
- NCI Thesaurus
- ORDO
- OMIM
- GARD
- MGI genotype (from TogoID)
Related Genes
| Gene ID | Gene Symbol | Description | Source |
|---|---|---|---|
| 2523 | FUT1 | fucosyltransferase 1 (H blood group) | |
| 3030 | HADHA | hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha | |
| 4684 | NCAM1 | neural cell adhesion molecule 1 | |
| 23205 | ACSBG1 | acyl-CoA synthetase bubblegum family member 1 | |
| 23583 | SMUG1 | single-strand-selective monofunctional uracil-DNA glycosylase 1 | |
| 27087 | B3GAT1 | beta-1,3-glucuronyltransferase 1 | |
| 27163 | NAAA | N-acylethanolamine acid amidase |
Related Glycoprotein
