Jabbour Elias, Kantarjian Hagop M, Koller Charles, Taher Ali
Department of Leukemia, The University of Texas M D Anderson Cancer Center, Houston, TX 77030, USA.
Cancer. 2008 Mar 1;112(5):1089-95. doi: 10.1002/cncr.23280.
Approximately 15,000 new cases of myelodysplastic syndromes (MDS) are expected in the United States each year.
The mainstay for the management of myelodysplastic syndromes (MDS) is supportive therapy with red blood cell (RBC) transfusions to improve the patient's quality of life. RBC transfusions enable adequate tissue oxygenation and increase hemoglobin levels, improve fatigue, and improve the physical and intellectual activity of patients. Up to 90% of patients with MDS will receive RBC transfusions during the course of their disease, and many will become chronically dependent on transfusions to manage their anemia. These transfusions lead to an accumulation of excess iron that, in turn, can develop into a condition known as iron overload, causing clinical consequences like hypertransaminasemia and cirrhosis, dilated cardiomyopathy, and progressive dysfunction of the endocrine glands.
Studies in patients with MDS have indicated that iron overload because of RBC transfusions was an independent, adverse prognostic factor for overall survival (OS) and leukemia-free survival (LFS): OS and LFS were significantly shorter in transfusion-dependent patients with MDS than in those who were not transfusion dependent.
Although the National Comprehensive Cancer Network guidelines for the treatment of patients with MDS recommend the use of RBC transfusions as supportive care, they further recommend that the iron burden of transfused patients be monitored regularly and that iron chelation therapy be considered to maintain serum ferritin levels of <1000 ng/mL.
在美国，预计每年有大约15000例新的骨髓增生异常综合征（MDS）病例。
骨髓增生异常综合征（MDS）治疗的主要手段是通过输注红细胞（RBC）进行支持性治疗，以提高患者的生活质量。输注红细胞可实现充足的组织氧合，提高血红蛋白水平，改善疲劳症状，并改善患者的身体和智力活动。高达90%的MDS患者在疾病过程中会接受红细胞输注，许多患者会长期依赖输血来治疗贫血。这些输血会导致过量铁的积累，进而可能发展为一种称为铁过载的病症，引发诸如高转氨酶血症和肝硬化、扩张型心肌病以及内分泌腺进行性功能障碍等临床后果。
对MDS患者的研究表明，因红细胞输注导致的铁过载是总生存期（OS）和无白血病生存期（LFS）的独立不良预后因素：依赖输血的MDS患者的OS和LFS明显短于非依赖输血的患者。
尽管美国国立综合癌症网络（National Comprehensive Cancer Network）针对MDS患者的治疗指南推荐使用红细胞输注作为支持性治疗，但进一步建议定期监测输血患者的铁负荷，并考虑采用铁螯合疗法以使血清铁蛋白水平维持在<1000 ng/mL。
