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目的探讨依赖输血的慢性血液病铁过载临床结果。方法回顾性分析依赖输血(1年以上,≥50单位/例)的骨髓增生异常综合征(MDS)和再生障碍性贫血(AA)患者10例的临床表现、重要脏器功能、影像学变化、内分泌改变和血清铁蛋白(SF)浓度,了解体内铁负荷程度,去铁治疗及临床转归。结果 SF均明显增高(1830～4586ng/ml);8例患者肝功能异常,7例肝脏CT值增高。SF>3500ng/ml患者合并皮肤色素沉着,肝脾肿大和内分泌异常,其中的6例已经死亡。7例患者接受了15～60d去铁治疗,持续依赖输血者SF无下降。结论低危MDS和AA长期输血会导致输血性铁过载,严重者合并重要脏器功能及影像的异常,甚至死亡。去铁治疗宜在适时(SF>1000ng/ml)开始,并持续给予直到SF<1000ng/ml。
Objective To investigate the clinical outcome of iron overload in chronic hematological diseases. Methods The clinical manifestations, major organ function and imaging changes in 10 patients with myelodysplastic syndrome (MDS) and aplastic anemia (AA) dependent on transfusion (more than 1 year and ≥50 units / case) were retrospectively analyzed. Endocrine changes and serum ferritin (SF) concentrations, understand the extent of iron load in vivo, to iron treatment and clinical outcome. Results SF were significantly higher (1830 ~ 4586ng / ml); 8 patients with abnormal liver function, hepatic CT value increased in 7 cases. SF> 3500ng / ml patients with skin pigmentation, hepatosplenomegaly and endocrine abnormalities, of which 6 cases have died. Seven patients underwent 15 to 60 days of deferox treatment, with no dependence on SF in transfusion recipients. Conclusions Long-term transfusion of low-risk MDS and AA can lead to overloading of transfusion iron. In severe cases, the function and imaging abnormalities of important organs and even death may be combined. Iron-free therapy should be started on time (SF> 1000 ng / ml) and continued until SF <1000 ng / ml.