Molecular genetics and management of world health organization defined atypical chronic myeloid leukemia

被引:5
|
作者
Sun, Yingxin [1 ,2 ]
Wang, Qinrong [1 ,2 ]
Zhang, Xingxia [3 ,4 ]
Zhang, Zhiyu [1 ,2 ]
Wang, Qian [1 ,2 ]
Cen, Jiannong [1 ,2 ]
Zhu, Mingqing [1 ,2 ]
Pan, Jinlan [1 ,2 ]
Liu, Dandan [1 ,2 ]
Wu, Depei [1 ,2 ]
Cai, Yifeng [5 ]
Chen, Suning [1 ,2 ]
机构
[1] Soochow Univ, Affiliated Hosp 1, Jiangsu Inst Hematol, Natl Clin Res Ctr Hematol Dis,Dept Hematol, Suzhou, Peoples R China
[2] Minist Hlth, Dept Thrombosis & Hemostasis, Key Lab Thrombosis & Hemostasis, Suzhou, Peoples R China
[3] Huaian Hosp, Xuzhou Med Coll, Dept Hematol, Huaian, Peoples R China
[4] Huaian Second Peoples Hosp, Huaian, Peoples R China
[5] Nantong Univ, Affiliated Hosp, Dept Hematol, Nantong, Peoples R China
基金
中国国家自然科学基金; 国家重点研发计划;
关键词
Atypical chronic myeloid leukemia; Myelodyslastic; myeloproliferative neoplasms; Mutation; Treatment; Clinical outcomes; DIAGNOSIS; MDS/MPN; CML;
D O I
10.1007/s00277-023-05106-8
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Atypical chronic myeloid leukemia (CML) is a rare BCR::ABL1-negative hematopoietic stem cell disease characterized by granulocytic proliferation and granulocytic dysplasia. Due to both the challenging diagnosis and the rarity of atypical CML, comprehensive molecular annotation-based analyses of this disease population have been scarce, and it is currently difficult to identify the optimal treatment for atypical CML. To explore atypical CML genomic landscape and treatment options, we performed a systematic retrospective of the clinical data and outcomes of 31 atypical CML patients. We observed that the molecular landscape of atypical CML was highly heterogeneous, with multiple molecular events driving its pathogenesis. Patients with atypical CML had a low response to current therapies, with an overall response rate (ORR) of 33.3% to hypomethylating agent (HMA)-based therapy. The current treatment strategies, including hematopoietic stem cell transplantation (HSCT), did not improve overall survival (OS) in atypical CML patients, with a median survival of 20 months. Thus, the benefits from HSCT and candidates for HSCT remain to be further evaluated. Acute myeloid leukemia (AML)-like chemotherapy followed by bridging allogeneic HSCT may be an ideal regimen for suitable individuals. The large-scale and prospective clinical studies will help to address the dilemma.
引用
收藏
页码:777 / 785
页数:9
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