Studies of ineffective erythropoiesis and peripheral haemolysis in congenital dyserythropoietic anaemia type II.
Erythrokinetic parameters were estimated in six patients suffering from congenital dyserythropoietic anaemia type II (CDA II) by means of a mathematical model of iron kinetics. A wide variation in effectiveness of erythroid activity was observed, and a significant negative correlation was found between ineffective erythropoiesis and peripheral haemolysis. In four patients with prominent peripheral haemolysis, splenectomy was carried out. Marked improvement in their clinical condition and in haemoglobin level resulted.
D000293:Adolescent; D000328:Adult; D000742:Anemia, Dyserythropoietic, Congenital / Q000097:blood* / Q000378:metabolism / Q000628:therapy; D000745:Anemia, Hemolytic, Congenital / Q000097:blood*; D001853:Bone Marrow / Q000378:metabolism; D002648:Child; D004905:Erythrocyte Aging; D004920:Erythropoiesis; D005260:Female; D006454:Hemoglobins / Q000096:biosynthesis; D006461:Hemolysis; D006801:Humans; D007501:Iron / Q000378:metabolism; D008297:Male; D013156:Splenectomy
D006454:Hemoglobins; D007501:Iron