CONGENITAL DYSERYTHROPOIETIC ANEMIAS

The congenital dyserythropoietic anemias (CDAs) are a group of relativ ely rare inherited anemias that share in common ineffective erythropoi esis and morphologic abnormalities of mature red blood cells and their precursors. Three major types of CDA and a number of variants have be en described, The diagnosis and categorization of these disorders are facilitated by microscopic examination of the blood and bone marrow an d by serologic testing, Management of patients currently consists of o bservation and supportive care. Because patients with CDAs may be at s ignificant risk for secondary hemochromatosis, they require monitoring for this condition, Splenectomy may be of benefit in certain cases in which the anemia is particularly severe, Over the past few years adva nces have been made in understanding the pathogenesis of these disorde rs, and it now appears that CDA II results from enzymatic defects in t he cellular glycosylation pathway. (C) 1996 Wiley-Liss, Inc.