Anie K A, Massaglia P
Brent Sickle Cell and Thalassaemia Centre, Central Middlesex Hospital, Acton Lane, London, UK, NW10 7NS.
Cochrane Database Syst Rev. 2001(3):CD002890. doi: 10.1002/14651858.CD002890.
Thalassaemia is a group of genetic blood disorders characterised by the absence or reduction in the production of haemoglobin. Severity is variable from less severe anaemia, through thalassaemia intermedia, to profound severe anaemia (thalassaemia major). In thalassaemia major other complications include growth retardation, bone deformation, and enlarged spleen. Blood transfusion is required to treat severe forms of thalassaemia, but this results in excessive accumulation of iron in the body (iron overload), removed mostly by a drug called desferrioxamine through 'chelation therapy'. Non-routine treatments are bone marrow transplantation (which is age restricted), and possibly hydroxyurea, designed to raise foetal haemoglobin level, thus reducing anaemia. In addition, psychological therapies seem appropriate to improving outcome and adherence to medical treatment.
To examine the evidence that in patients with thalassaemia, psychological treatments improve the ability to cope with the condition, and improve both medical and psychosocial outcome.
The Cochrane Cystic Fibrosis and Genetic Disorders Group specialist trials register which comprises references from comprehensive electronic database searches, handsearching relevant journals and handsearching abstract books of conference proceedings. Also, searches on the Internet were performed.
All randomised or quasi-randomised controlled trials comparing the use of psychological intervention to no (psychological) intervention in patients with thalassaemia.
No trials of psychological therapies were found in the literature for inclusion at the present time.
There are no results to be reported at present.
REVIEWER'S CONCLUSIONS: As a chronic disease with a considerable role for self-management, psychological support seems appropriate for managing thalassaemia. However, no conclusions can be made about the use of specific psychological therapies in thalassaemia from the information currently available. This systematic review has clearly identified the need for well designed, adequately-powered, multicentre, randomised controlled trials assessing the effectiveness of specific psychological interventions for thalassaemia.
地中海贫血是一组遗传性血液疾病，其特征是血红蛋白生成缺乏或减少。严重程度各不相同，从较轻的贫血到中间型地中海贫血，再到严重的重度贫血（重型地中海贫血）。重型地中海贫血的其他并发症包括生长发育迟缓、骨骼变形和脾脏肿大。治疗严重型地中海贫血需要输血，但这会导致体内铁过量积累（铁过载），主要通过一种名为去铁胺的药物进行“螯合疗法”来清除。非常规治疗方法是骨髓移植（有年龄限制），还有可能使用羟基脲，其目的是提高胎儿血红蛋白水平，从而减轻贫血。此外，心理治疗似乎有助于改善治疗效果和提高对医疗治疗的依从性。
研究心理治疗对地中海贫血患者应对疾病的能力以及改善医疗和心理社会结局方面的证据。
Cochrane囊性纤维化和遗传疾病小组专业试验注册库，其中包括来自全面电子数据库检索、手工检索相关期刊以及手工检索会议论文摘要集的参考文献。此外，还在互联网上进行了检索。
所有比较心理干预与无（心理）干预在治疗地中海贫血患者中的随机或半随机对照试验。
目前在文献中未发现可纳入的心理治疗试验。
目前尚无结果可报告。
作为一种对自我管理有重要作用的慢性病，心理支持似乎适合用于管理地中海贫血。然而，根据目前可得的信息，无法就地中海贫血中特定心理治疗的使用得出结论。本系统综述明确指出需要设计良好、样本量充足、多中心的随机对照试验来评估特定心理干预对地中海贫血的有效性。
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