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| Term | hemophagocytic lymphohistiocytosis | ID (Ontology) | DOID:0050120 (Human Disease) |
| 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. | ||
| Also Known As | "haemophagocytic syndrome" | ||
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immune system disease |__lymphatic system disease |__hemophagocytic lymphohistiocytosis 3 rec. |__familial hemophagocytic lymphohistiocytosis 1 |__familial hemophagocytic lymphohistiocytosis 2 |__familial hemophagocytic lymphohistiocytosis 3 1 rec. |__familial hemophagocytic lymphohistiocytosis 4 1 rec. |__familial hemophagocytic lymphohistiocytosis 5 1 rec. |
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| Is a | lymphatic system disease | ||
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GARD:6589 ICD10CM:D76.1 MESH:D051359 MIM:PS267700 NCI:C34792 ORDO:540 SNOMEDCT_US_2023_03_01:190958003 UMLS_CUI:C0024291 |
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