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Immunodeficiency 46

Disease ID: disease_node_13303

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DbxrefMIM:616740, ORDO:476113, SNOMEDCT_US_2023_09_01:1179288008, UMLS_CUI:C5568133
SubclassofDOID_628, DOID_0050737
Data SourceDOID
SynonymsCID due to TFRC deficiency, IMD46, TFRC-related combined immunodeficiency, combined immunodeficiency due to TFRC deficiency
Doid Labelimmunodeficiency 46
Doid DescriptionA combined T cell and B cell immunodeficiency characterized by hypo- or agammaglobulinemia, normal lymphocyte counts, intermittent neutropenia, intermittent thrombocytopenia, decreased numbers of memory B cells, impaired immunoglobulin class-switching, and decreased proliferative responses of T cells that has_material_basis_in homozygous or compound heterozygous mutation in the TFRC gene on chromosome 3q29.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_13303
Doid IdDOID_0111948
LabelImmunodeficiency 46