摘要目的 观察髓系肉瘤异体移植后地西他滨及达沙替尼维持治疗的效果.方法 1例29岁髓系肉瘤合并急性髓系白血病患者,c-kit基因突变阳性,染色体核型分析:t(8;21).诱导治疗缓解后行同胞全相合异基因造血干细胞移植,预处理方案为地西他滨+ FLAG+改良的Bu/Cy方案,采用环孢素A+吗替麦考酚酯+短程氨甲蝶呤预防移植物抗宿主病,回输供者细胞数:17.59×106/kg的CD34+,11.73×108/kg的MNC,中性粒细胞及血小板植入时间均为移植后13 d.移植后50d开始应用地西他滨,每个月1疗程,共5疗程;同时在移植后100 d开始给予达沙替尼口服.结果 该病例随访至移植后16个月,期间复查骨髓细胞学、AML/ETO融合基因定量、脑脊液细胞形态学和腹腔增强CT均未见明显异常.结论 异基因造血干细胞移植是治疗髓系肉瘤的有效手段.地西他滨对于髓系肉瘤可能有一定效果,可用于移植后维持治疗;酪氨酸激酶抑制剂对于c-kit基因突变阳性的髓系肉瘤患者可能是一种减少复发的手段.本病例在异基因造血干细胞移植后应用地西他滨及达沙替尼移维持治疗,在国内外文献报道中尚属首例,并取得良好效果.

abstractsObjective To observe the efficacy of maintenance treatment with decitabine and dasatinib after allogenic hematopoietic stem cell transplantation for myeloid sarcoma.Methods A 29-year-old male patient was diagnosed with abdominal myeloid sarcoma and acute myeloid leukemia with c-kit mutation and t(8;21).Allogeneic hematopoietic stem cell transplantation was performed after inducted remission.The conditioning regimen was decitabine + FLAG + modified Bu/Cy.Prophylaxis of GVHD was performed with cyclosporine mycophenolate mofetil and short-term methotrexate.The patient received 11.73 × 108 mononucleated cells/kg and 17.59 × 106CD34+ cells/kg from donor.At Day 13 post-transplantation,neutrophils reached 0.5 × 109/L and platelet count was 20 × 109/L.Decitabine was prescribed since Day 50 post-transplantation monthly for 5 courses.And dasatinib was offered orally since Day 100 for 4 months.Results It was followed up to 16 months post-transplantation.There were no obvious abnormalities of bone marrow cytology,AML/ETO fusion gene quantification,cerebrospinal fluid or abdominal enhanced computed tomography (CT).Conclusions Hematopoietic stem cell transplantation is an effective treatment for myeloid sarcoma.Decitabine has some efficacy for myeloid sarcoma and it may be used for maintenance treatment after transplantation.Tyrosine kinase inhibitors reduce recurrence in myeloid sarcoma with c-kit mutation.The treatment of decitabine and dasatinib after allogeneic hematopoietic stem cell transplantation yield excellent outcomes.This is the first report in domestic and foreign literatures.