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Immunodeficiency 73B With Defective Neutrophil Chemotaxis And Lymphopenia

Disease ID: disease_node_13334

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DbxrefMIM:618986
SubclassofDOID_0111962, DOID_0050736
Data SourceDOID
SynonymsIMD73B
Doid Labelimmunodeficiency 73b with defective neutrophil chemotaxis and lymphopenia
Doid DescriptionA combined immunodeficiency characterized by onset of recurrent infections in infancy or early childhood and variable immune system abnormalities including B- and T-cell lymphopenia, decreased immunoglobulin subsets, decreased TCR excision circles and dysfunctional T cells, decreased NK cells, neutropenia, and impaired neutrophil chemotaxis that has_material_basis_in heterozygous gain-of-function mutation in the RAC2 gene on chromosome 22q13.1.
Has Material Basis InGENO_0000147
Disease Node Iddisease_node_13334
Doid IdDOID_0112061
LabelImmunodeficiency 73B With Defective Neutrophil Chemotaxis And Lymphopenia