Preferred Name | hemophagocytic lymphohistiocytosis | |
Synonyms |
haemophagocytic syndrome |
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Definitions |
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. Xref MGI. MESH:C537250 added from NeuroDevNet [WAK]. |
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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 |
ORDO:540 SNOMEDCT_US_2021_09_01:190958003 UMLS_CUI:C0024291 NCI:C34792 OMIM:PS267700 MESH:D051359 ICD10CM:D76.1 GARD:6589 |
|
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. Xref MGI. MESH:C537250 added from NeuroDevNet [WAK]. |
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has_alternative_id |
DOID:6453 |
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has_exact_synonym |
haemophagocytic syndrome |
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has_obo_namespace |
disease_ontology |
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has_symptom |
http://purl.obolibrary.org/obo/SYMP_0019142 |
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IAO_0000115 |
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. |
|
id |
DOID:0050120 |
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in_subset | ||
label |
hemophagocytic lymphohistiocytosis |
|
notation |
DOID:0050120 |
|
note |
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. Xref MGI. MESH:C537250 added from NeuroDevNet [WAK]. |
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prefLabel |
hemophagocytic lymphohistiocytosis |
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subClassOf |