Congenital Dyserythropoietic Anemias

Chapter 446 Congenital Dyserythropoietic Anemias




The congenital dyserythropoietic anemias (CDA) are a heterogeneous class of genetic disorders characterized by unique morphologic abnormalities in marrow erythroblasts: multinuclearity, abnormal nuclear fragments, and intrachromatin bridges between cells, associated with ineffective erythropoiesis. Three major types of CDA (types I, II, and III) are defined (see imageTable 446-1 on the Nelson Textbook of Pediatrics website at www.expertconsult.com), although additional subgroups and variants have also been identified. These rare disorders are characterized by variable degrees of anemia, increased marrow erythroid activity (ineffective erythropoiesis), and secondary hemochromatosis.





Type I Congenital Dyserythropoietic Anemia




Jun 18, 2016 | Posted by in PEDIATRICS | Comments Off on Congenital Dyserythropoietic Anemias

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