No evidence for myocardial iron overload and free iron species in multitransfused patients with sickle/beta-thalassaemia.
Iron overload (IO) in the heart is a life-threatening complication in transfusion-dependent patients with thalassaemia major (TM) and to a lesser extent in sickle cell disease (SCD), while no data are available in patients with sickle/beta(0)-thalassaemia. Iron deposition in the heart, liver and pancreas was assessed using T2* MRI sequences, as well as free iron species assays - non-transferrin bound iron (NTBI) and labile plasma iron (LPI), in addition to serum ferritin, percentage transferrin saturation and serum hepcidin, in 10 multitransfused patients (>30 yr) with sickle/beta(0)-thalassaemia. None of the patients had iron deposition in the heart. Three patients had mild, one had moderate, and two had severe liver IO. Two patients had mild iron deposition in the pancreas. In all the patients, serum hepcidin levels were normal - NTBI and LPI were not detected. Possible explanations of these findings are discussed.
Ghoti
Hussam
H
Edith Wolfson Medical center, Holon, Israel. drghoti123@yahoo.com
Goitein
Orly
O
Koren
Ariel
A
Levin
Carina
C
Kushnir
Tamar
T
Rachmilewitz
Eliezer
E
Konen
Eli
E
D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000473:pathology / Q000628:therapy*; D023181:Antimicrobial Cationic Peptides / Q000097:blood; D005260:Female; D064451:Hepcidins; D006801:Humans; D007501:Iron / Q000032:analysis* / Q000097:blood; D019190:Iron Overload / Q000209:etiology* / Q000473:pathology; D008099:Liver / Q000737:chemistry / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000737:chemistry* / Q000473:pathology; D010179:Pancreas / Q000737:chemistry / Q000473:pathology; D065227:Transfusion Reaction; D055815:Young Adult; D017086:beta-Thalassemia / Q000097:blood / Q000473:pathology / Q000628:therapy*
D023181:Antimicrobial Cationic Peptides; C578702:HAMP protein, human; D064451:Hepcidins; D007501:Iron