X-linked dyserythropoietic anemia with abnormal platelets and neutropeniaX-linked anemia with/without neutropenia and/or platelet abnormalities
obo:hasOBONamespace
disease_ontology
n2:IAO_0000115
An anemia characterized by early-onset anemia and bone marrow erythroid hypoplasia with variable neutropenia that has_material_basis_in hemizygous splice-site mutation in GATA1 on chromosome Xp11.23. This mutaion impairs expression of the long isoform of GATA1 but expression of a short form is seen.