Noguer Mathilde, Berthon Phanélie, Makowski Caroline, Messonnier Laurent A
Laboratoire Interuniversitaire de Biologie de la Motricité, Univ Savoie Mont Blanc, Chambéry, France.
Service de Médecine Interne, Centre Hospitalier Grenoble Alpes, La Tronche, France.
Br J Haematol. 2025 Jan;206(1):86-93. doi: 10.1111/bjh.19847. Epub 2024 Nov 19.
Transfusion-dependent β-thalassaemia (TDβT) is a genetic disorder characterised by reduced or absent β-globin chain synthesis, resulting in chronic anaemia. Treatment consists of regular blood transfusions and chelation therapy to limit iron overload and its negative effects on organs (e.g. heart, lungs and liver). Despite improved life expectancy with transfusions and chelation therapy, TDβT patients experience poor quality of life and impaired exercise capacity (mainly due to muscle deconditioning and reduced cardiac inotropism). The aims of this review are (i) to discuss the role of organ dysfunction in the impairment of exercise capacity and reduced quality of life of TDβT patients; and (ii) to discuss physical activity as a potential therapeutic strategy to alleviate the multifactorial impact of the disease.
依赖输血的β地中海贫血（TDβT）是一种遗传性疾病，其特征是β珠蛋白链合成减少或缺乏，导致慢性贫血。治疗包括定期输血和螯合疗法，以限制铁过载及其对器官（如心脏、肺和肝脏）的负面影响。尽管输血和螯合疗法提高了预期寿命，但TDβT患者的生活质量较差，运动能力受损（主要由于肌肉失用和心脏收缩力降低）。本综述的目的是：（i）讨论器官功能障碍在TDβT患者运动能力受损和生活质量下降中的作用；（ii）讨论体育活动作为一种潜在的治疗策略，以减轻该疾病的多因素影响。
