不典型慢性粒细胞白血病诊断及治疗进展  

Progress of diagnosis and treatment of atypical chronic myeloid leukemia

作　　者：杨丹[1] 徐燕丽[1] Yang Dan;Xu Yanli(Department of Hematology,Nanjing First Hospital,Nanjing Hospital Affiliated to Nanjing Medical University,Nanjing 210006,China)

机构地区：[1]南京医科大学附属南京医院(南京市第一医院)血液科,210006

出　　处：《白血病．淋巴瘤》2018年第6期368-372,共5页Journal of Leukemia & Lymphoma

摘　　要：不典型慢性粒细胞白血病（aCML）是费城染色体（Ph）及bcr-abl融合基因均阴性的罕见的血液系统恶性肿瘤,该病和慢性粒细胞白血病（CML）完全不同.根据其临床特点和主要血液学特征归类于骨髓增生异常综合征/骨髓增殖性肿瘤.由于该疾病临床发病率低及对其认识有限,导致临床上易发生漏诊或误诊,且存在患者生存期较短等问题.为了进一步提高aCML患者的诊断率及生存期,文章就其诊断标准、鉴别诊断、相关基因突变、预后评估及临床治疗进展进行综述.Atypical chronic myeloid leukemia （aCML） is a rare hematologic malignancy with both bcr-abl-negative and the absence of the Philadelphia chromosome, which is completely different from chronic myeloid leukemia （CML）. According to the clinical characteristics and the main hematological characteristics, it is classified as myelodysplastic/myeloproliferative neoplasms. Due to the low incidence and limited understanding of aCML, it can result in the clinical missed diagnosis or misdiagnosis, and the short survival time of patients. In order to further improve the diagnostic rate and survival time, this paper reviews the current diagnostic criteria, differential diagnosis, related gene mutations, prognosis evaluation and clinical treatments of aCML.

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 基因突变 异基因造血干细胞移植 

分 类 号：R733.72[医药卫生—肿瘤]