title: Successful treatment of iron overload with phlebotomies in two siblings with congenital dyserythropoietic anemia--type II (CDA-II).
abstract: Successful treatment of iron overload by phlebotomies has been reported in two splenectomized siblings with congenital dyserythropoietic anemia--type II (CDA-II). In both patients 400 ml of blood were withdrawn every month. During three years 12 200 ml of blood were removed. The serum ferritin levels decreased from 1450,4 microg/L and 1131,7 microg/L to 447 microg/L and 457 microg/l, respectively. The transferrin saturation dropped from 0,99 at the start of the therapy to 0,64 and 0,86, respectively. The values of Hb, Hct, erythrocyte counts and MCV did not change as well as did not change reticulocyte counts, reticulocyte index, and RDW. Both patients tolerated repeated phlebotomies well. The decrease of bilirubin and normal values of haptoglobin might be the concequence of diminished destruction of erythrocytes and their precursors. Our observation confirms that phlebotomies can be used with success in CDA patients with mild anemia as treatment modality of iron overload.
authors.affiliation: 2nd Department of Medicine, Division of Clinical Hematology, Charles University in Prague, Faculty of Medicine and University Hospital in Hradec Králové, Czech Republic. ladislavchrobak@seznam.cz
authors.forename: Ladislav
authors.initials: L
authors.lastname: Chrobák
mesh_terms: D000293:Adolescent; D000328:Adult; D000742:Anemia, Dyserythropoietic, Congenital / Q000097:blood / Q000235:genetics / Q000628:therapy*; D006801:Humans; D019190:Iron Overload / Q000628:therapy*; D008297:Male; D018962:Phlebotomy; D013156:Splenectomy