loading...| Preferred Name |
hemophagocytic lymphohistiocytosis |
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| Synonyms |
haemophagocytic syndrome |
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| Definitions |
Xref MGI. MESH:C537250 added from NeuroDevNet [WAK]. 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. |
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| ID |
http://purl.obolibrary.org/obo/DOID_0050120 |
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| comment |
Xref MGI. MESH:C537250 added from NeuroDevNet [WAK]. |
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| database_cross_reference |
MESH:D051359 SNOMEDCT_US_2023_03_01:190958003 ICD10CM:D76.1 OMIM:PS267700 UMLS_CUI:C0024291 GARD:6589 NCI:C34792 ORDO:540 |
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| has exact synonym |
haemophagocytic syndrome |
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| has symptom |
http://purl.obolibrary.org/obo/SYMP_0019142 |
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| has_alternative_id |
DOID:6453 |
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| has_obo_namespace |
disease_ontology |
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| id |
DOID:0050120 |
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| in_subset | ||
| label |
hemophagocytic lymphohistiocytosis |
|
| notation |
DOID:0050120 |
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| prefLabel |
hemophagocytic lymphohistiocytosis |
|
| textual 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. |
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| subClassOf |