Iron overload and iron-chelating therapy in hemoglobin e-β thalassemia

American Journal of Pediatric Hematology/Oncology

Volumn 22, Issue 6, 2000, Pages 593-597

  Olivieri, Nancy F ^a   De Silva, Shanthimala ^a   Premawardena, Anuja ^a   Sharma, Supriya ^a   Viens, Adrian M ^a   Taylor, Chelsea M ^a   Brittenham, Gary M ^a   Weatherall, David J ^a  

^a UNIVERSITY OF TORONTO   (Canada)

Author keywords

Hemoglobin E; Iron loading; Iron quantitation; Iron chelating therapy; Key Words; Liver biopsy; thalassemia

Indexed keywords

HEMOGLOBIN E; IRON; IRON CHELATING AGENT;

ADULT; ARTICLE; BETA THALASSEMIA; BLOOD; BLOOD TRANSFUSION; CASE REPORT; HUMAN; IRON OVERLOAD; LIVER; MALE; METABOLISM; PATHOLOGY; PRACTICE GUIDELINE;

ADULT; BETA-THALASSEMIA; BLOOD TRANSFUSION; HEMOGLOBIN E; HUMANS; IRON; IRON CHELATING AGENTS; IRON OVERLOAD; LIVER; MALE; PRACTICE GUIDELINES;

EID: 0034323388     PISSN: 01928562     EISSN: None     Source Type: Journal    
DOI: 10.1097/00043426-200011000-00029     Document Type: Article

References (24)

1. Weatherall DJ. Hemoglobin E β-thalassemia: an increasingly common disease with some diagnostic pitfalls. J Pediatr 1998; 132:765-7.
2. Olivieri NF, Brittenham GM. Iron-chelating therapy and the treatment of thalassemia. Blood 1997; 89:739-61.
3. Olivieri NF, De Silva S, Fischer C, et al. Natural history study of Hemoglobin E/β thalassemia. Blood 1998; 92(S1):532a.
4. Pippard MJ, Warner GT, Callender ST, et al. Iron absorption and loading in beta-thalassaemia intermedia. Lancet 1979; 2:819-21.
5. Pootrakul P, Kitcharoen K, Yansukon P, et al. The effect of erythroid hyperplasia on iron balance. Blood 1988; 71:1124-9.
6. Olivieri NF, Sharma S, DeSilva S, et al. Impact of a low transfusion regimen on growth and iron loading in hemoglobins E/β thalassemia. Blood 1999; 94(S1):422a.
7. Brittenham GM, Cohen AR, McLaren CE, et al. Hepatic iron stores and plasma ferritin concentration in patients with sickle cell anemia and thalassemia major. Am J Hematol 1993; 42:81-5.
8. Brittenham GM, Farrell DE, Harris JW, et al. Magnetic-susceptibility measurement of human iron stores. N Engl J Med 1982; 307:1671-5.
9. Angelucci E, Giovagnoni A, Valeri G, et al. Limitations of magnetic resonance imaging in measurement of hepatic iron. Blood 1997; 90:4736-42.
10. Cartwright GE, Edwards CQ, Kravitz K, et al. Hereditary hemochromatosis: phenotypic expression of the disease. N Engl J Med 1979; 301:175-9.
11. Brittenham GM, Griffith PM, Nienhuis AW, et al. Efficacy of deferoxamine in preventing complications of iron overload in patients with thalassemia major. N Engl J Med 1994; 331:567-73.
12. Olivieri NF. Thalassaemia: Clinical Management. In: Rodgers GP, ed. Bailliere's Clinical Hematology. London: Bailliere Tindall, 1998;11:147-62.
13. Porter JB. A risk-benefit assessment of iron-chelation therapy. Drug Saf 1997; 17:407-21.
14. Barry M, Flynn D, Letsky E, et al. Long term chelation therapy in thalassemia major: effect on liver iron concentration, liver histology and clinical progress. Br Med J 1974; 2:16-20.
15. Niederau C, Fischer R, Purschel A, et al. Long-term survival in patients with hereditary hemochromatosis. Gastroenterology 1996; 110:1107-79.
16. Olivieri NF. The β thalassemias. N Engl J Med 1999; 341:99-109.
17. Bronspeigel-Weintrob N, Olivieri NF, Tyler BJ, et al. Effect of age at the start of iron chelation therapy on gonadal function in β-thalassemia major. N Engl J Med 1990; 323:713-9.
18. Italian Working Group on Endocrine Complications in Non-endocrine Diseases. Multicenter study on prevalence of endocrine complications in thalassaemia major. Clin Endocrinol 1995;42:581-6.
19. Olivieri NF, Nathan DG, MacMillan JH, et al. Survival of medically treated patients with homozygous β thalassemia. N Engl J Med 1994; 331:574-8.
20. Gabutti V, Piga A. Results of long-term iron-chelating therapy. Acta Haematol 1996; 95:26-36.
21. Giardini C. Treatment of β-thalassemia. Curr Opin Hematol 1997; 4:79-87.
22. Hershko C. Iron chelators for thalassaemia. Br J Haematol 1998; 101:399-406.
23. Pippard MJ, Callender ST, Warner GT, et al. Iron absorption and loading in beta-thalassaemia intermedia. Lancet 1979;ii:819-21.
24. Warner GT, Oliver R. A whole-body counter for clinical measurements utilizing the “shadow shield” technique. Phys Med Biol 1966; 11:83-93.