| 释义 |
congenital dyserythropoietic anemia con·gen·i·tal dys·e·ryth·ro·poi·et·ic a·ne·mi·aa group of anemias characterized by ineffective erythropoiesis, bone marrow erythroblastic multinuclearity, and secondary hemochromatosis. Three types are described: type I [MIM224120], macrocytic, megaloblastic anemia with erythroblastic internuclear chromatin bridges; type II, [MIM*224100], normoblastic anemia with multinucleated erythroblasts; type III, macrocytic anemia with erythroblastic multinuclearity and gigantoblasts [MIM*105600]. Both types I and II are autosomal recessively inherited, type III is of autosomal dominant inheritance.congenital dyserythropoietic anemia Pediatric Hematology A group of inherited defects of erythropoiesis Clinical Lifelong mild-to-moderate anemia and ineffective erythropoiesis |