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Lymphohistiocytosis, Hemophagocytic

Disease ID: disease_node_11475

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DbxrefGARD:6589, ICD10CM:D76.1, MESH:D051359, MIM:PS267700, NCI:C34792, ORDO:540, SNOMEDCT_US_2023_03_01:190958003, UMLS_CUI:C0024291
SubclassofDOID_75
Data SourceDOID, MESH
Synonymshaemophagocytic syndrome
Mesh IdD051359
Mesh LabelLymphohistiocytosis, Hemophagocytic
Mesh SubclassofD015616
Doid Labelhemophagocytic lymphohistiocytosis
Doid DescriptionA 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 maligcy. 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].
Has SymptomSYMP_0000521, SYMP_0019142, SYMP_0000047
Disease Node Iddisease_node_11475
Doid IdDOID_0050120
LabelLymphohistiocytosis, Hemophagocytic
Doid Alternate IdsDOID_6453