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慢性粒单核细胞白血病的诊断与治疗

Diagnosis and treatment of chronic myelomonocytic leukemia.

作者信息

Kwon Jihyun

机构信息

Division of Hematology and Oncology, Department of Internal Medicine, Chungbuk National University College of Medicine, Chungbuk National University Hospital, Cheongju, Korea.

出版信息

Blood Res. 2021 Apr 30;56(S1):S5-S16. doi: 10.5045/br.2021.2020321.

Abstract

Chronic myelomonocytic leukemia (CMML) is a clonal disorder of hematopoietic cells and is a complex of heterogeneous conditions with both myeloproliferative and myelodysplastic features. The diagnosis of CMML is made using morphologic criteria including monocyte-dominant leukocytosis, dysplastic changes, and increased blasts in the bone marrow. Recently, the identification of monocyte subtypes in peripheral blood using multiparameter flow cytometry has been actively studied. Chromosomal abnormalities are the basis of CMML risk stratification, and mutations in several genes including ASXL1 are known to be important not only for the diagnosis and treatment of this disease but also for predicting its prognosis. The standard treatment principles for CMML have not yet been clearly defined; however, hypomethylating agents are mainly considered the frontline therapy in most cases. Although allogeneic hematopoietic stem cell transplantation has limited applications owing to its toxicity, it still plays an important role as the only curative treatment option. Researchers are continuing to develop new drugs for CMML treatment and to prove their clinical usefulness. This review summarizes what is known to date on the diagnosis, treatment, and prognostic factors of CMML and presents future directions by analyzing recent research trends.

摘要

慢性粒单核细胞白血病(CMML)是一种造血细胞的克隆性疾病,是一组具有骨髓增殖和骨髓发育异常特征的异质性病症。CMML的诊断依据形态学标准,包括以单核细胞为主的白细胞增多、发育异常改变以及骨髓中原始细胞增多。近来,利用多参数流式细胞术对外周血单核细胞亚型进行鉴定的研究十分活跃。染色体异常是CMML危险分层的基础,已知包括ASXL1在内的多个基因的突变不仅对该病的诊断和治疗很重要,而且对预测其预后也很重要。CMML的标准治疗原则尚未明确界定;然而,在大多数情况下,去甲基化药物主要被视为一线治疗方法。尽管异基因造血干细胞移植因其毒性而应用有限,但它作为唯一的治愈性治疗选择仍发挥着重要作用。研究人员正在继续研发用于CMML治疗的新药,并证明其临床实用性。本综述总结了目前已知的CMML的诊断、治疗和预后因素,并通过分析近期研究趋势提出了未来的方向。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/88da/8094002/1911e6401783/br-56-s1-5-f1.jpg

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