Department of Medicine, Queen Mary Hospital, Hong Kong.
Hong Kong Med J. 2011 Aug;17(4):261-6.
To provide a synopsis of current thalassaemia major patient care in Hong Kong.
Retrospective study.
All haematology units of the Hospital Authority in Hong Kong.
All patients with thalassaemia major with regular transfusion.
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.
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.
概述香港重型地中海贫血患者的现有治疗情况。
回顾性研究。
香港医院管理局的所有血液科单位。
所有接受定期输血的重型地中海贫血患者。
截至目前，医院管理局共照顾 363 名重型地中海贫血患者。产前诊断有助于减少本地新发病例的数量，但近年来出现了移民病例。这些患者的平均年龄为 23 岁（范围为 1-52 岁），其中 78%为成年人。2009 年，他们共接受了 18782 单位的血液。这占香港红十字会全部血液消耗的 9.5%。过去，心脏铁过载是导致死亡的主要原因（65%），很少有患者能活到 45 岁以上。磁共振成像（T2 MRI）评估心脏铁含量并指导使用口服去铁酮螯合疗法，降低了心力衰竭和心脏铁沉积症的发生率，从而降低了死亡率并提高了预期寿命。
香港地中海贫血治疗的未来前景光明。通过更好的输血和螯合作用，年轻患者的生长和内分泌缺陷应该可以避免。
