title: Prevalence and distribution of iron overload in patients with transfusion-dependent anemias differs across geographic regions: results from the CORDELIA study.
abstract: OBJECTIVE
  The randomized comparison of deferasirox to deferoxamine for myocardial iron removal in patients with transfusion-dependent anemias (CORDELIA) gave the opportunity to assess relative prevalence and body distribution of iron overload in screened patients.
  
  
  METHODS
  Patients aged ≥ 10 yr with transfusion-dependent anemias from 11 countries were screened. Data were summarized descriptively, overall and across regions.
  
  
  RESULTS
  Among 925 patients (99.1% with β-thalassemia major; 98.5% receiving prior chelation; mean age 19.2 yr), 36.7% had myocardial iron overload (myocardial T2* ≤ 20 ms), 12.1% had low left ventricular ejection fraction. Liver iron concentration (LIC) (mean 25.8 mg Fe/g dw) and serum ferritin (median 3702 ng/mL) were high. Fewer patients in the Middle East (ME; 28.5%) had myocardial T2* ≤ 20 ms vs. patients in the West (45.9%) and Far East (FE, 40.9%). Patients in the West had highest myocardial iron burden, but lowest LIC (26.9% with LIC < 7 mg Fe/g dw) and serum ferritin. Among patients with normal myocardial iron, a higher proportion of patients from the ME and FE had LIC ≥ 15 than < 7 mg Fe/g dw (ME, 56.7% vs. 17.2%; FE, 78.6% vs. 7.8%, respectively), a trend which was less evident in the West (44.6% vs. 33.9%, respectively). Transfusion and chelation practices differed between regions.
  
  
  CONCLUSIONS
  Evidence of substantial myocardial and liver iron burden across regions revealed a need for optimization of effective, convenient iron chelation regimens. Significant regional variation exists in myocardial and liver iron loading that are not well explained; improved understanding of factors contributing to differences in body iron distribution may be of clinical benefit.
authors.affiliation: Ege University Hospital, Izmir, Turkey.
authors.forename: Yesim
authors.initials: Y
authors.lastname: Aydinok
authors.affiliation: University College London, London, UK.
authors.forename: John B
authors.initials: JB
authors.lastname: Porter
authors.affiliation: University of Turin, Turin, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Piga
authors.affiliation: Ain Shams University, Cairo, Egypt.
authors.forename: Mohsen
authors.initials: M
authors.lastname: Elalfy
authors.affiliation: Cairo University, Cairo, Egypt.
authors.forename: Amal
authors.initials: A
authors.lastname: El-Beshlawy
authors.affiliation: Cukurova University Medical Faculty, Adana, Turkey.
authors.forename: Yurdanur
authors.initials: Y
authors.lastname: Kilinç
authors.affiliation: Siriraj Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Vip
authors.initials: V
authors.lastname: Viprakasit
authors.affiliation: Akdeniz University, Antalya, Turkey.
authors.forename: Akif
authors.initials: A
authors.lastname: Yesilipek
authors.affiliation: Novartis Pharmaceuticals, East Hanover, NJ, USA.
authors.forename: Dany
authors.initials: D
authors.lastname: Habr
authors.affiliation: Novartis Pharma AG, Basel, Switzerland.
authors.forename: Erhard
authors.initials: E
authors.lastname: Quebe-Fehling
authors.affiliation: NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital, London, UK.
authors.forename: Dudley J
authors.initials: DJ
authors.lastname: Pennell
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000740:Anemia / Q000150:complications* / Q000628:therapy; D002648:Child; D003676:Deferoxamine / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007501:Iron / Q000378:metabolism; D007502:Iron Chelating Agents / Q000627:therapeutic use; D019190:Iron Overload / Q000188:drug therapy / Q000453:epidemiology* / Q000209:etiology* / Q000378:metabolism; D008099:Liver / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000378:metabolism / Q000473:pathology; D015995:Prevalence; D014018:Tissue Distribution; D065227:Transfusion Reaction; D055815:Young Adult
chemical_list: D007502:Iron Chelating Agents; D007501:Iron; D003676:Deferoxamine
keywords: distribution; heart; iron; liver; prevalence; thalassemia