文章摘要
孙振容,赖琴城,刘昊东,等.塞利尼索治疗骨髓增生异常综合征 /骨髓增殖性肿瘤伴中性粒细胞增多症 1例及文献复习[J].安徽医药,2024,28(11):2311-2315.
塞利尼索治疗骨髓增生异常综合征 /骨髓增殖性肿瘤伴中性粒细胞增多症 1例及文献复习
Efficacy of the patient with MDS/MPN with neutrophilia containing multiple treated with selinexor: one case report and literature review
  
DOI:10.3969/j.issn.1009-6469.2024.11.043
中文关键词: 塞利尼索  骨髓增生异常综合征  骨髓增殖性肿瘤  中性粒细胞增多症  基因突变  治疗
英文关键词: Selinexor  Myelodysplastic syndromes  Myeloproliferative neoplasm  Neutrophilia  Gene mutation  Treatment
基金项目:
作者单位E-mail
孙振容 潍坊市人民医院血液内科山东潍坊 261000  
赖琴城 潍坊市人民医院血液内科山东潍坊 261000  
刘昊东 潍坊市人民医院血液内科山东潍坊 261000  
綦畅鹏 潍坊市人民医院血液内科山东潍坊 261000  
冉学红 潍坊市人民医院血液内科山东潍坊 261000 ranxuehong1967@sina.com 
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中文摘要:
      目的探讨塞利尼索治疗含有多个不良预后因素的骨髓增生异常综合征 /骨髓增殖性肿瘤( MDS/MPN)伴中性粒细胞增多症的疗效。方法回顾性分析潍坊市人民医院 2021年 5月收治的 1例 MDS/MPN伴中性粒细胞增多症的病人的临床资料,并复习相关文献。结果 70岁男性,血常规示白细胞计数明显增高,主要为中性粒细胞及前体细胞,骨髓形态示粒系增生活跃伴有发育异常,二代测序示 SETBP1、ASXL1、NRAS、KRAS、U2AF1基因突变,诊为 MDS/MPN伴中性粒细胞增多症。应用去甲基化药物联合塞利尼索治疗有效,疾病病程由进展恶化转为稳定,生存质量提高,血液学指标改善。结论 MDS/MPN伴中性粒细胞增多症是罕见的血液肿瘤,目前无标准治疗方法;应用去甲基化药物联合塞利尼索治疗安全有效,为不适合异基因造血干细胞移植的病人带来了新的治疗选择,并可能为病人带来临床获益。
英文摘要:
      Objective To explore of the efficacy of selinisol in the treatment of myelodysplastic syndrome/myeloproliferative tumor(MDS/MPN) with multiple adverse prognostic factors and neutrophilia.Methods The clinical data of a patient with MDS/MPN withneutrophilia admitted to Weifang People's Hospital in May 2021 were retrospectively analyzed, and the relevant literature was reviewed.Results The patient was a 70-year-old male, with a significantly higher white blood cell count, mainly neutrophils and myeloid precursors,a bone marrow morphology showing active granulocytic proliferation with abnormal development, and NGS analysisshowing mutations in SETBP1,ASXL1,NRAS,KRAS,and U2AF1 genes,diagnosed as MDS/MPN with neutrophilia. Demethylated drugcombined with celinisol was effective,the disease course changed from progressive deterioration to stability, quality of life was improved,and hematological indexes were improved.Conclusions MDS/MPN with neutrophilia is a rare hematologic tumor for which there is nostandard treatment.The treatment of demethylated drugs combined with selinisol is safe and effective, which brings a new treatment option for patients who are not suitable for allogeneic hematopoietic stem cell transplantation and may bring clinical benefits to patients.
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