地西他滨联合芦可替尼治疗老年不典型慢性粒细胞白血病:1例报道并文献复习  

Decitabine combined with ruxolitinib in treatment of elderly patient with atypical chronic myeloid leukemia: a case report and literature review

作　　者：娄典 刘利 秦炜炜 Lou Dian;Liu Li;Qin Wei-Wei(Department of Hematology,the Second Affiliated Hospital of Air Force Military Medical University,Xi’an,Shaanxi 710038,China)

机构地区：[1]空军军医大学第二附属医院血液内科,陕西西安710038

出　　处：《解放军医学杂志》2023年第2期211-217,共7页Medical Journal of Chinese People's Liberation Army

基　　金：陕西省重点研发计划(2019SF-080)。

摘　　要：目的 报道1例经地西他滨联合芦可替尼方案治疗的老年不典型慢性粒细胞白血病(aCML)的诊治经过并复习文献,以提高对aCML的认识。方法 报告1例伴CSF3R T618I突变的老年aCML患者的病例资料及应用地西他滨联合芦可替尼方案治疗的临床过程。检索中国知网、万方数据知识服务平台、PubMed数据库(截至2021年12月),结合文献报道总结aCML的临床特点及应用地西他滨方案联合芦可替尼治疗的有效性和安全性。结果 本病例系72岁男性,因“乏力、头晕1个月余”入院。血常规提示白细胞计数明显增高,骨髓细胞形态学提示粒系明显增生伴粒系发育异常,原始细胞<20%,二代测序显示CSF3R T618I突变,诊断为aCML。给予地西他滨治疗4个周期,并联合芦可替尼靶向治疗后,患者达到形态学完全缓解(CR),且分子应答良好,长期口服芦可替尼维持治疗病情稳定。国内外报道采用去甲基化药物地西他滨或阿扎胞苷治疗aCML的患者共11例,其中8例采用单药地西他滨治疗[总体CR率为87.5%(7/8)],2例采用单药阿扎胞苷治疗[有效率为50.0%(1/2)],1例采用地西他滨联合CAG方案(重组人粒细胞集落刺激因子+阿糖胞苷+阿克拉霉素)化疗,患者达到CR;采用芦可替尼治疗伴CSF3R T618I突变的aCML患者共4例,治疗的总体有效率为50.0%(2/4)。目前国内外尚无地西他滨联合芦可替尼方案治疗老年aCML的病例报道。结论 aCML是一种罕见病,其诊断依赖形态学检测,本例采用地西他滨联合芦可替尼方案治疗安全有效。Objective By literature review to retrospectively study the diagnosis and treatment process of decitabine combined with ruxolitinib in treatment of elderly patient with atypical chronic myeloid leukemia(aCML) for improving the understanding of aCML. Methods To report a case data of elderly aCML patients with CSF3R T618I mutation and the clinical treatment process using decitabine combined with ruxolitinib. Search CNKI, Wanfang data knowledge service platform, PubMed database(as of December 2021). Combined with literature reports to summarize the clinical characteristics of aCML and the effectiveness and safety in treatment applying decitabine combined with ruxolitinib. Results A case of 72-year-old man, admitted to our hospital since fatigue and dizziness for more than 1 month, and diagnosed as aCML with obviously increased white blood cells, granulocytic proliferation and granulocytic dysplasia with primitive cells <20%, and CSF3R T618I mutation detected by next-generation sequencing(NGS) analysis. After targeted therapy with ruxolitinib and 4 courses of treatment with decitabine, the patient achieved complete remission(CR) on morphology and had a good response on molecular. The patient?s primary disease remained stable by maintenance therapy with ruxolitinib. A total of 11 cases treated with hypomethylating agents such as decitabine and azacitidine in aCML were found in literature, including 8 cases treated with decitabine alone [the CR rate was 87.5%(7/8)],2 cases treated with azacitidine alone [the effective rate was 50.0%(1/2)], and 1 case treated with decitabine combined with CAG(recombinant human granulocyte colony-stimulating factor + cytarabine + aclacinomycin) chemotherapy and achieved CR. A total of 4 cases treated with ruxolitinib alone in CSF3R T618I mutant aCML were found in literature and the overall efficiency was 50.0%(2/4). No case treated with drug combination in treatment of decitabine and ruxolitinib were found by searching literature.Conclusion aCML is a kind of rare disease, the diagnosis i

关 键 词：不典型慢性粒细胞白血病 骨髓增生异常综合征/骨髓增殖性肿瘤 地西他滨 芦可替尼 靶向治疗 

分 类 号：R733.3[医药卫生—肿瘤]