Hagar R W, Morris C R, Vichinsky E P
Adult Sickle Cell Center, Children's Hospital and Research Center at Oakland, Oakland, CA 94618, USA.
Br J Haematol. 2006 May;133(4):433-5. doi: 10.1111/j.1365-2141.2006.06053.x.
Pulmonary hypertension is common in adults with thalassaemia and other haemolytic anaemias. It was hypothesised that regular transfusions in thalassaemia major should both decrease the chronic haemolytic rate and be protective from pulmonary hypertension (PHT). To reduce the contribution of existing cardiac disease to PHT, the subjects were limited to patients with normal left ventricular shortening fractions. Associations with multiple laboratory markers of haemolysis, serum ferritin levels, chest X-rays findings and splenectomy status were also considered. We found no biochemical, transfusional, or clinical (except gender) differences in transfused thalassaemia patients with or without pulmonary hyper tension.
肺动脉高压在患有地中海贫血和其他溶血性贫血的成年人中很常见。据推测，重度地中海贫血患者定期输血既能降低慢性溶血率，又能预防肺动脉高压（PHT）。为减少现有心脏病对PHT的影响，研究对象仅限于左心室缩短分数正常的患者。还考虑了溶血的多种实验室指标、血清铁蛋白水平、胸部X线检查结果和脾切除状态之间的关联。我们发现，无论有无肺动脉高压，接受输血的地中海贫血患者在生化、输血或临床方面（性别除外）均无差异。
