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Familial Erythrocytosis 1

Disease ID: disease_node_15334

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DbxrefICD10CM:D75.0, MIM:133100, ORDO:90042
SubclassofDOID_0050736, DOID_10780
Data SourceDOID
SynonymsECYT1, autosomal dominant benign erythrocytosis, primary familial and congenital polycythemia
Doid Labelfamilial erythrocytosis 1
Doid DescriptionA primary polycythemia that has_material_basis_in mutation in the gene encoding the erythropoietin receptor. It is characterized by increased serum red blood cell mass and hemoglobin concentration, hypersensitivity of erythroid progenitors to EPO, and low serum levels of EPO.
Has Material Basis InGENO_0000147
Disease Node Iddisease_node_15334
Doid IdDOID_0060652
LabelFamilial Erythrocytosis 1