许多韩国输血引起的铁超负荷患者经历了严重的临床后遗症,包括器官损伤,需要终身螯合治疗.然而,由于缺乏依从性和/或缺乏适当的螯合剂,大多数患者没有得到有效治疗。Deferasirox(DFX),每日一次口服铁螯合剂,适用于输血诱导的铁超负荷的成人和儿科患者,现在可以在韩国使用。地拉罗司在减少或维持体内铁的有效性已在许多研究中得到证实,这些研究涉及多种输血引起的贫血,如骨髓增生异常综合征,再生障碍性贫血,和其他慢性贫血。DFX的推荐初始日剂量为20mg/kg体重,在食物前至少30分钟空腹服用,血清铁蛋白水平应保持在1000ng/mL以下。优化输血引起的铁过载的管理,韩国血液学协会再生障碍性贫血工作组(KSHAAWP)回顾了关于铁超负荷的普遍共识和关于铁螯合疗法临床益处的韩国数据,并制定了韩国铁过载治疗指南。
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.