title: Unclassified type of congenital dyserythropoietic anaemia (CDA) with prominent peripheral erythroblastosis.
abstract: Two unrelated cases of congenital dyserythropoietic anaemia (CDA) are described. They show striking similarities which could not be attributed to one of the well-known types of CDA or any other congenital disease of the erythroid system. Both patients were followed for many years before and after splenectomy. There was a long-lasting, prominent post-splenectomy erythroblastosis, suggesting impairment of red cell denucleation. The type of heredity is unknown, and the enzymatic or molecular basis of the changes observed is not understood.
mesh_terms: D000328:Adult; D000742:Anemia, Dyserythropoietic, Congenital / Q000097:blood / Q000473:pathology*; D000745:Anemia, Hemolytic, Congenital / Q000473:pathology*; D001853:Bone Marrow / Q000473:pathology; D004900:Erythroblasts / Q000648:ultrastructure; D005260:Female; D006801:Humans; D008854:Microscopy, Electron; D013156:Splenectomy