地西他滨治疗老年难治性骨髓增生异常综合征-环形铁粒幼细胞性贫血一例并文献复习

目的 报道地西他滨治疗老年难治性骨髓增生异常综合征-环形铁粒幼细胞性贫血(MDS-RAS)患者1例,通过文献复习探讨其疗效.方法 采用地西他滨每天20 mg/m2×5 d为1个疗程、4个疗程的方案治疗.观察其症状、血象、骨髓像、T细胞极化状态、细胞免疫和染色体等变化,依据WHO(2008)疗效判定标准判定疗效.结果 患者第2疗程后乏力、食欲不振等全身症状消失.1个疗程后血象开始上升,4个疗程后Hb、Plt、WBC基本恢复正常.2个疗程后复查骨髓环形铁幼粒细胞由治疗前0.16降至0,T细胞极化状态基本恢复正常,染色体由复杂核型转为正常.结论 地西他滨是老年难治性MDS-RAS患者治疗的有效药物,有效率和长期疗效有待于增加病例进一步观察.

Treatment of a elderly patient of myelodysplastic syndrome-ring sideroblastic refratory anemia with decitabine and review of literatures

Objective To study the effect of decitabine treating elderly patient with myelodysplastic syndrome-ring sideroblastic refractory anemia (MDS-RAS) and review the literatures. Methods Decitabine treated a patient with MDS-RAS four courses, at the dose of 25 mg everyday for 5 days per course. Observed the change of symptoms, peripheral blood cell counts, myelogram, T cell polarization, cellular immunity,chromosome. Determined the curative effect combined with efficiency standard of WHO 2008. Results The clinical symptoms got better after two courses. Peripheral blood cell counts began to get better after one course. The number of leukocyte, hemoglobin and platelet got nearly normal after four courses. After two courses, T cell polarization state got normal, the number of iron ring promyelocyticin bone marrow declined from 16 % to 0 and chromosome changed from complex karyotype to normal. Conclusion Decitabine is an effective drug to the old patient with MDS-RAS. But it needs to increase the number of cases and follow up long-term to observe the effective rate and long-term efficacy.