造血干细胞移植后维持治疗腹腔髓系肉瘤1例报告并文献复习

Zhenkun Dong, Xinsheng Xie, Zhongxing Jiang, D. Wan, Jia Liu, Wenwen Ding, Haiqiong Wang, Fei He
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摘要

目的观察异基因造血干细胞移植后地西他滨联合达沙替尼维持治疗髓系肉瘤的疗效。方法1例29岁男性腹腔髓系肉瘤合并急性髓系白血病合并c-kit突变和t(8;21)。诱导缓解后进行同种异体造血干细胞移植。调理方案为地西他滨+ FLAG +改性Bu/Cy。预防GVHD采用环孢素霉酚酸酯和短期甲氨蝶呤。患者分别接受供体提供的11.73×108单核细胞/kg和17.59×106CD34+细胞/kg。移植后第13天,中性粒细胞达到0.5×109/L,血小板计数20×109/L。从移植后第50天开始每月开地西他滨5个疗程。从第100天起口服达沙替尼,持续4个月。结果随访至移植后16个月。骨髓细胞学、AML/ETO融合基因定量、脑脊液及腹部增强CT检查均未见明显异常。结论造血干细胞移植是治疗髓系肉瘤的有效方法。地西他滨对髓系肉瘤有一定疗效,可用于移植后的维持治疗。酪氨酸激酶抑制剂可减少c-kit突变髓系肉瘤的复发。异基因造血干细胞移植后地西他滨和达沙替尼治疗效果良好。这在国内外文献中尚属首次报道。关键词:造血干细胞移植;髓系肉瘤;隐性基因
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Maintenance therapy after hematopoietic stem cell transplantation for abdominal myeloid sarcoma: a case report and literature review
Objective 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. Key words: Hematopoietic stem cell transplantation; Myeloid sarcoma; Allogene
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