Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
J Korean Med Sci. 2013 Nov;28(11):1563-72. doi: 10.3346/jkms.2013.28.11.1563. Epub 2013 Oct 31.
Many Korean patients with transfusion-induced iron overload experience serious clinical sequelae, including organ damage, and require lifelong chelation therapy. However, due to a lack of compliance and/or unavailability of an appropriate chelator, most patients have not been treated effectively. Deferasirox (DFX), a once-daily oral iron chelator for both adult and pediatric patients with transfusion-induced iron overload, is now available in Korea. The effectiveness of deferasirox in reducing or maintaining body iron has been demonstrated in many studies of patients with a variety of transfusion-induced anemias such as myelodysplastic syndromes, aplastic anemia, and other chronic anemias. The recommended initial daily dose of DFX is 20 mg/kg body weight, taken on an empty stomach at least 30 min before food and serum ferritin levels should be maintained below 1000 ng/mL. To optimize the management of transfusion-induced iron overload, the Korean Society of Hematology Aplastic Anemia Working Party (KSHAAWP) reviewed the general consensus on iron overload and the Korean data on the clinical benefits of iron chelation therapy, and developed a Korean guideline for the treatment of iron overload.
许多韩国输血相关性铁过载患者会出现严重的临床后果,包括器官损伤,需要终身螯合治疗。然而,由于缺乏依从性和/或缺乏合适的螯合剂,大多数患者未得到有效治疗。地拉罗司(DFX)是一种每日一次的口服铁螯合剂,适用于输血相关性铁过载的成人和儿科患者,现已在韩国上市。DFX 可降低或维持体内铁含量,在多项针对骨髓增生异常综合征、再生障碍性贫血和其他慢性贫血等各种输血相关性贫血患者的研究中得到了证实。DFX 的推荐起始日剂量为 20 mg/kg 体重,空腹至少 30 分钟前服用,血清铁蛋白水平应保持在 1000ng/mL 以下。为了优化输血相关性铁过载的管理,韩国血液学会再生障碍性贫血工作组(KSHAAWP)回顾了铁过载的一般共识和韩国关于铁螯合治疗临床获益的数据,并制定了韩国铁过载治疗指南。