[Congenital dyserythropoietic anemia type III. A case report].
Congenital dyserythropoietic anaemia type III is a rare disorder characterized by mild to moderate anaemia, ineffective erythropoiesis, and morphologic abnormalities of mature red blood cells and their precursors. The most extraordinary feature of this condition is the large number of multinuclear erythroblasts found in the bone marrow, some containing up to 12 nuclei. This type of anaemia is an autosomal dominant inherited disorder, though sporadic cases have been described. Little conclusive is known about the pathogenesis of congenital dyserthropoietic anaemia type III. At present the management of patients consists of observation and supportive care. We describe a 20 year old man who was admitted to a county hospital, showing the typical features of this rare illness. He had a Hb value of 10.2 g/100 ml.
Holm
T
T
Sykestua, Asegarden militaerleir.
Kildahl-Andersen
O
O
Sjo
M
M
D000328:Adult; D000742:Anemia, Dyserythropoietic, Congenital / Q000097:blood / Q000473:pathology / Q000628:therapy; D006801:Humans; D008297:Male