A synopsis of current care of thalassaemia major patients in Hong Kong

Hong Kong Medical Journal

Volumn 17, Issue 4, 2011, Pages 261-266

  Au, W Y ^a   Lee, Vincent ^b   Lau, C W ^c   Yau, Jeffrey ^d   Chan, Desmond ^e   Chan, Eric Y T ^f   Cheung, Winnie W W ^a   Ha, S Y ^a   Kho, Bonnie ^g   Lee, C Y ^h   Li, Rever C H ^c   Li, C K ^b   Lin, S Y ^e   Ling, Alvin S C ⁱ   Mak, Vivien ⁱ   Sun, Lina ^g   Wong, Kris H F ⁱ   Wong, Raymond ^b   Yuen, H L ^d  

^a QUEEN MARY HOSPITAL   (Hong Kong)

^b CHINESE UNIVERSITY OF HONG KONG   (Hong Kong)

^c TUEN MUN HOSPITAL   (Hong Kong)

^d QUEEN ELIZABETH HOSPITAL   (Hong Kong)

^e UNITED CHRISTIAN HOSPITAL   (Hong Kong)

^f KWONG WAH HOSPITAL   (Hong Kong)

^g PAMELA YOUDE NETHERSOLE EASTERN HOSPITAL   (Hong Kong)

^h Caritas Medical Centre Hong Kong   (Hong Kong)

ⁱ PRINCESS MARGARET HOSPITAL   (Hong Kong)

Author keywords

Beta Thalassemia; Blood transfusion; Chelation therapy; Hemosiderosis; Iron chelating agents

Indexed keywords

BISPHOSPHONIC ACID DERIVATIVE; CALCIUM; DEFERASIROX; DEFERIPRONE; DEFEROXAMINE; FERRITIN; PEGINTERFERON; RIBAVIRIN; VITAMIN D;

AGE DISTRIBUTION; AGRANULOCYTOSIS; ARTICLE; CARDIOVASCULAR MAGNETIC RESONANCE; ERYTHROCYTE TRANSFUSION; FERRITIN BLOOD LEVEL; HEART FAILURE; HEART HEMOSIDEROSIS; HEMATOPOIETIC STEM CELL TRANSPLANTATION; HEPATITIS B; HEPATITIS C; HONG KONG; HUMAN; HYPOGONADISM; IMMIGRANT; IRON CHELATION; IRON OVERLOAD; IRON STORAGE; LIFE EXPECTANCY; MORTALITY; OSTEOPOROSIS; PRENATAL DIAGNOSIS; PREVALENCE; RETROSPECTIVE STUDY; SEX RATIO; SPLENECTOMY; THALASSEMIA MAJOR;

ADOLESCENT; ADULT; CHILD; CHILD, PRESCHOOL; ERYTHROCYTE TRANSFUSION; HONG KONG; HUMANS; INFANT; IRON OVERLOAD; MIDDLE AGED; OSTEOPOROSIS; RETROSPECTIVE STUDIES; THALASSEMIA;

EID: 80052925400     PISSN: 10242708     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (33)

1. Lau YL, Chan LC, Chan YY, et al. Prevalence and genotypes of alpha-and beta-thalassemia carriers in Hong Kong-implications for population screening. N Engl J Med 1997;336:1298-301.
2. Leung KY, Lee CP, Tang MH, et al. Cost-effectiveness of prenatal screening for thalassaemia in Hong Kong. Prenat Diagn 2004;24:899-907.
3. Lee AC, Wong KW, So KT, Cheng MY. Why are thalassaemia patients born when prenatal screening is available? Hong Kong Med J 1998;4:121-4.
4. Ho HK, Ha SY, Lam CK, et al. Alloimmunization in Hong Kong southern Chinese transfusion-dependent thalassemia patients. Blood 2001;97:3999-4000.
5. Li CK, Luk CW, Ling SC, et al. Morbidity and mortality patterns of thalassaemia major patients in Hong Kong: retrospective study. Hong Kong Med J 2002;8:255-60.
6. Cohen AR. New advances in iron chelation therapy. Hematology Am Soc Hematol Educ Program 2006:42-7.
7. Ha SY, Chik KW, Ling SC, et al. A randomized controlled study evaluating the safety and efficacy of deferiprone treatment in thalassemia major patients from Hong Kong. Hemoglobin 2006;30:263-74.
8. Anderson LJ, Holden S, Davis B, et al. Cardiovascular T2-star (T2*) magnetic resonance for the early diagnosis of myocardial iron overload. Eur Heart J 2001;22:2171-9.
9. Pennell DJ. T2* magnetic resonance and myocardial iron in thalassemia. Ann N Y Acad Sci 2005;1054:373-8.
10. He T, Kirk P, Firmin DN, et al. Multi-center transferability of a breath-hold T2 technique for myocardial iron assessment. J Cardiovasc Magn Reson 2008;10:11.
11. Au WY, Lam WW, Chu W, et al. A T2* magnetic resonance imaging study of pancreatic iron overload in thalassemia major. Haematologica 2008;93:116-9.
12. Au WY, Lam WW, Chu WW, et al. A cross-sectional magnetic resonance imaging assessment of organ specific hemosiderosis in 180 thalassemia major patients in Hong Kong. Haematologica 2008;93:784-6.
13. Lam WW, Au WY, Chu WC, Tam S, Ha SY, Pennell D. One-stop measurement of iron deposition in the anterior pituitary, liver and heart in thalassemia patients. J Magn Reson Imaging 2008;28:29-33.
14. Pennell DJ, Berdoukas V, Karagiorga M, et al. Randomized controlled trial of deferiprone or deferoxamine in betathalassemia major patients with asymptomatic myocardial siderosis. Blood 2006;107:3738-44.
15. Ha SY, Mok AS, Chu WC, et al. A practical chelation protocol based on stratification of thalassemic patients by serum ferritin and magnetic resonance imaging cardiac T2*. Hemoglobin 2009;33:323-31.
16. Borgna-Pignatti C, Cappellini MD, De Stefano P, et al. Cardiac morbidity and mortality in deferoxamine-or deferiprone-treated patients with thalassemia major. Blood 2006;107:3733-7.
17. Telfer P, Coen PG, Christou S, et al. Survival of medically treated thalassemia patients in Cyprus. Trends and risk factors over the period 1980-2004. Haematologica 2006;91:1187-92.
18. Modell B, Khan M, Darlison M, Westwood MA, Ingram D, Pennell DJ. Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance. J Cardiovasc Magn Reson 2008;10:42.
19. Cappellini MD, Cohen A, Piga A, et al. A phase 3 study of deferasirox (ICL670), a once-daily oral iron chelator, in patients with beta-thalassemia. Blood 2006;107:3455-62.
20. Cappellini MD, Porter J, El-Beshlawy A, et al. Tailoring iron chelation by iron intake and serum ferritin: the prospective EPIC study of deferasirox in 1744 patients with transfusiondependent anemias. Haematologica 2009;95:557-66.
21. Pennell DJ, Porter JB, Cappellini MD, et al. Efficacy of deferasirox in reducing and preventing cardiac iron overload in beta-thalassemia. Blood 2010;115:2364-71.
22. Kirk P, Roughton M, Porter JB, et al. Cardiac T2* magnetic resonance for prediction of cardiac complications in thalassemia major. Circulation 2009;120:1961-8.
23. Au WY, Kung AWC, Chan GC, Ha SY, Tam S, Liang R. High prevalence of osteoporosis / osteopenia and favorable oneyear treatment response to oral alendronate, calcium and vitamin D supplements: a prospective study of 122 Chinese patients with beta thalassemia and hemoglobin H disease [abstract]. Blood 2002;100:235a.
24. Voskaridou E, Terpos E. Pathogenesis and management of osteoporosis in thalassemia. Pediatr Endocrinol Rev 2008;6 Suppl 1:86S-93S.
25. Leung TF, Chu Y, Lee V, et al. Long-term effects of pamidronate in thalassemic patients with severe bone mineral density deficits. Hemoglobin 2009;33:361-9.
26. Sookoian S, Castaño G. Evaluation of a third generation anti-HCV assay in predicting viremia in patients with positive HCV antibodies. Ann Hepatol 2002;1:179-82.
27. Gamberini MR, Francesconi R, Fortini M, et al. HCV and HGV infection, iron overload and liver disease in multitransfused patients with thalassaemia and persistently normal or abnormal transaminase levels. Pediatr Endocrinol Rev 2004;2 Suppl 2:259S-266S.
28. Puoti C. HCV carriers with persistently normal ALT Levels: not too much healthy, not true patients. Rom J Gastroenterol 2004;13:329-32.
29. Li CK, Chan PK, Ling SC, Ha SY. Interferon and ribavirin as frontline treatment for chronic hepatitis C infection in thalassaemia major. Br J Haematol 2002;117:755-8.
30. HIV-From epidemiology to diagnosis. HKSAR Government website: http://www.info.gov.hk/aids/pdf/g104htm/1.1.htm. Accessed Dec 2010.
31. Chung BH, Ha SY, Chan GC, et al. Klebsiella infection in patients with thalassemia. Clin Infect Dis 2003;36:575-9.
32. Au WY, Cheung WC, Chan GC, Ha SY, Khong PL, Ma ES. Risk factors for hyperbilirubinemia and gallstones in Chinese patients with b thalassemia syndrome. Haematologica 2003;88:220-2.
33. Li CK, Lee V, Shing MM, Leung TF. Haematopoietic stem cell transplantation for thalassaemia in Chinese patients. Hong Kong Med J 2009;15(3 Suppl 3):39S-41S.