title: High nontransferrin bound iron levels and heart disease in thalassemia major.
abstract: Although the presence of nontransferrin bound plasma iron (NTBI) in transfusional iron overload is well documented, knowledge about its clinical significance is limited. We assessed NTBI levels in a large and homogeneous series of thalassemia patients on regular transfusion and chelation and explored the hypothesis that NTBI levels may be associated with relevant clinical outcomes: in particular, heart disease. Among 174 patients with thalassemia major and intermedia, we showed the presence NTBI in 145 of 174 or 83.3% of cases. NTBI levels correlated with transferrin saturation, age, and ALT, and not with serum ferritin or liver iron concentrations. At a multiple regression analysis, transferrin saturation and heart disease but not age was independent predictors of NTBI. Patients with heart disease had NTBI levels significantly higher than those without. All patients with heart disease had transferrin saturation above 70%, and all were NTBI positive. Conversely, none of the patients without NTBI and/or with transferrin saturation less than 70% had preclinical or clinical heart disease. To our knowledge, this is the first documentation of a link between the presence of NTBI in thalassemic patients with transfusional iron overload and heart disease. Further investigation from these preliminary findings may clarify whether NTBI assessment may have a role in evaluating the risks and optimizing treatment for transfusion-dependent patients.
authors.affiliation: Department of Clinical and Biological Sciences, University of Torino, Turin, Italy. antonio.piga@unito.it
authors.forename: Antonio
authors.initials: A
authors.lastname: Piga
authors.forename: Filomena
authors.initials: F
authors.lastname: Longo
authors.forename: Lorena
authors.initials: L
authors.lastname: Duca
authors.forename: Simona
authors.initials: S
authors.lastname: Roggero
authors.forename: Tiziana
authors.initials: T
authors.lastname: Vinciguerra
authors.forename: Roberto
authors.initials: R
authors.lastname: Calabrese
authors.forename: Chaim
authors.initials: C
authors.lastname: Hershko
authors.forename: Maria Domenica
authors.initials: MD
authors.lastname: Cappellini
mesh_terms: D000293:Adolescent; D000328:Adult; D001145:Arrhythmias, Cardiac / Q000097:blood / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000209:etiology*; D007501:Iron / Q000097:blood*; D019190:Iron Overload / Q000150:complications* / Q000175:diagnosis / Q000209:etiology; D008297:Male; D008875:Middle Aged; D012044:Regression Analysis; D013789:Thalassemia / Q000628:therapy*; D065227:Transfusion Reaction; D018487:Ventricular Dysfunction, Left / Q000097:blood / Q000209:etiology*; D055815:Young Adult
chemical_list: D005293:Ferritins; D007501:Iron