ABSTRACT
Hong Kong Med J 2011;17:261–6 | Number 4, August 2011
ORIGINAL ARTICLE
A synopsis of current care of thalassaemia major patients in Hong Kong
WY Au, Vincent Lee, CW Lau, Jeffrey Yau, Desmond Chan, Eric YT Chan, Winnie WW Cheung, SY Ha, Bonnie Kho, CY Lee, Rever CH Li, CK Li, SY Lin, Alvin SC Ling, Vivien Mak, Lina Sun, Kris HF Wong, Raymond Wong, HL Yuen
Department of Medicine, Queen Mary Hospital, Hong Kong
OBJECTIVE. To provide a synopsis of current thalassaemia major patient care in Hong Kong.
DESIGN. Retrospective study.
SETTING. All haematology units of the Hospital Authority in Hong Kong.
PATIENTS. All patients with thalassaemia major with regular transfusion.
RESULTS. To date, there were 363 thalassaemia major patients under the care of the Hospital Authority. Prenatal diagnosis has helped to reduce the number of indigenous new cases, but in recent years immigrant cases are appearing. The patients have a mean age of 23 (range, 1-52) years, and 78% of them are adults. In 2009, they received 18 782 units of blood. This accounted for 9.5% of all blood consumption from the Hong Kong Red Cross. In the past, cardiac iron overload was the major cause of death (65%) and few patients survived beyond the age of 45 years. The availability of cardiac iron assessment by magnetic resonance imaging (T2* MRI) to direct the use of oral deferiprone chelation has reduced the prevalence of heart failure and cardiac haemosiderosis, which should reduce mortality and improve life expectancy.
CONCLUSION. The future for thalassaemia care in Hong Kong is bright. With better transfusion and chelation, it should be possible to avoid growth and endocrine deficiencies in younger patients.
Key words: beta-Thalassemia; Blood transfusion; Chelation therapy; Hemosiderosis; Iron chelating agents
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