Non-transferrin-bound iron in long-term transfusion in children with congenital anemias.
Non-transferrin-bound iron (NTBI), a potentially toxic compound, is increased in the serum of patients with iron overload and fully saturated transferrin. We found markedly elevated NTBI levels in 16 children (including nine with sickle cell disease and five with beta-thalassemia) with iron overload secondary to prolonged transfusion therapy. During iron chelation with subcutaneous desferrioxamine infusion, NTBI levels decreased to normal, but became elevated within 2 to 4 hours after discontinuation of desferrioxamine. NTBI causes hepatic and cardiac toxicity in experimental systems, but our patients lacked sufficient organ dysfunction for this association to be made. The use of continuous 24-hour chelation to maintain NTBI at low levels may prevent progressive iron toxicity in patients who first received chelation therapy at an older age or who already have evidence of cardiac damage.
Wang
W C
WC
Ahmed
N
N
Hanna
M
M
D000293:Adolescent; D000328:Adult; D000745:Anemia, Hemolytic, Congenital / Q000628:therapy*; D000755:Anemia, Sickle Cell / Q000097:blood / Q000628:therapy; D000756:Anemia, Sideroblastic / Q000097:blood / Q000628:therapy; D001667:Binding, Competitive; D002648:Child; D003676:Deferoxamine / Q000008:administration & dosage; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D007263:Infusions, Parenteral; D007501:Iron / Q000097:blood* / Q000652:urine; D008099:Liver / Q000000981:diagnostic imaging; D008134:Long-Term Care; D008297:Male; D011379:Prognosis; D013789:Thalassemia / Q000097:blood / Q000628:therapy; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D014168:Transferrin / Q000097:blood; D065227:Transfusion Reaction
D014168:Transferrin; D005293:Ferritins; D007501:Iron; D003676:Deferoxamine