非典型慢性髓性白血病：一种具有管理挑战的罕见疾病。
Atypical chronic myeloid leukemia: a rare entity with management challenges.
机构信息
Department of Medicine, Michigan State University, 788 Service Rd, East Lansing, MI 48824, USA.
Department of Internal Medicine, Division of Hematology & Oncology, University of Nebraska Medical Center, NE 68198, USA.
出版信息
Future Oncol. 2018 Jan;14(2):177-185. doi: 10.2217/fon-2017-0334. Epub 2017 Dec 11.
The aim of our study was to review the clinicopathologic features and management of atypical chronic myeloid leukemia (aCML). Relevant manuscripts published in English were searched using PubMed. aCML is diagnosed as per WHO 2016 classification in the presence of leukocytosis ≥13 × 109/l with circulating neutrophil precursors ≥10%, monocytes less than 10%, minimal basophils, hypercellular bone marrow with granulocytic proliferation and dysplasia, bone marrow blast less than 20% and absence of BCR/ABL fusion gene. Common cytogenetic features and mutations include trisomy 8, and mutations in SETBP1 and ETNK1. Median survival is 1-2 years. Hematopoietic stem cell transplant may be the only curative option. Ruxolitinib and dasatinib are emerging therapeutic options. Thus, aCML is a rare entity with poor survival. Novel therapies are needed.
我们研究的目的是回顾不典型慢性髓系白血病（aCML）的临床病理特征和治疗方法。使用 PubMed 搜索了以英文发表的相关文献。根据 2016 年 WHO 分类，当外周血白细胞计数≥13×109/L，循环中性粒细胞前体≥10%，单核细胞<10%，嗜碱性粒细胞<1%，骨髓增生明显伴粒系增生和发育异常，骨髓原始细胞<20%，无 BCR/ABL 融合基因时，诊断为 aCML。常见的细胞遗传学特征和突变包括三体 8、SETBP1 和 ETNK1 突变。中位生存期为 1-2 年。造血干细胞移植可能是唯一的治愈方法。鲁索利替尼和达沙替尼是新兴的治疗选择。因此，aCML 是一种生存预后差的罕见疾病，需要新的治疗方法。
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