loading...| Preferred Name |
hemophagocytic syndrome |
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| Synonyms |
hemophagocytic syndrome HLH hemophagocytic lymphohistiocytosis FHL familial erythrophagocytic lymphohistiocytosis hemophagocytic disorder familial histiocytic reticulosis haemophagocytic syndrome familial hemophagocytic lymphohistiocytosis |
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| Definitions |
Editor note: NCIT treats hemophagocytic syndrome and Hemophagocytic Lymphohistiocytosis as distinct; we merge here but this should be checked. See https://github.com/NCI-Thesaurus/thesaurus-obo-edition/issues/19 Hemophagocytic syndrome (HPS) is a rare immune disease and a potentially life-threatening disorder characterized by cytokine storm and overwhelming inflammation causing fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. It can be either primary due to a genetic defect (primary hemophagocytic lymphohistiocytosis), or secondary to malignancies, to infections, most commonly with viruses such as Epstein-Barr virus or cytomegalovirus, human immunodeficiency virus, or to autoimmune disorders such as systemic lupus erythematosus or adult-onset Still disease (secondary hemophagocytic lymphohistiocytosis). |
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| ID |
http://purl.obolibrary.org/obo/MONDO_0015540 |
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| comment |
Editor note: NCIT treats hemophagocytic syndrome and Hemophagocytic Lymphohistiocytosis as distinct; we merge here but this should be checked. See https://github.com/NCI-Thesaurus/thesaurus-obo-edition/issues/19 |
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| has characteristic | ||
| has_exact_synonym |
hemophagocytic syndrome HLH hemophagocytic lymphohistiocytosis |
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| has_related_synonym |
FHL familial erythrophagocytic lymphohistiocytosis hemophagocytic disorder familial histiocytic reticulosis haemophagocytic syndrome familial hemophagocytic lymphohistiocytosis |
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| label |
hemophagocytic syndrome |
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| prefixIRI |
MONDO:0015540 |
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| prefLabel |
hemophagocytic syndrome |
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| seeAlso |
https://rarediseases.info.nih.gov/diseases/6589/hemophagocytic-lymphohistiocytosis |
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| textual definition |
Hemophagocytic syndrome (HPS) is a rare immune disease and a potentially life-threatening disorder characterized by cytokine storm and overwhelming inflammation causing fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. It can be either primary due to a genetic defect (primary hemophagocytic lymphohistiocytosis), or secondary to malignancies, to infections, most commonly with viruses such as Epstein-Barr virus or cytomegalovirus, human immunodeficiency virus, or to autoimmune disorders such as systemic lupus erythematosus or adult-onset Still disease (secondary hemophagocytic lymphohistiocytosis). |
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| subClassOf |