附加8号染色体克隆演变在慢性髓系白血病病程进展中的临床意义  

Clinical Significance of Additional Chromosomal 8 Clonal Evolution in CML Progression

作　　者：季鸥 朱光荣[1] 林琳 吴萸 季建敏 于慧 沈群 JI Ou;ZHU Guang-Rong;LIN Lin;WU Yu;JI Jian-Min;YU Hui;SHEN Qun(Department of Hematology,Affiliated Hospital,Nanjing University of Traditional Chinese Medicine,Nanjing 210029,Jiangsu Pro -vince,China;First Clinical Medical College,Nanjing University of Traditional Chinese Medicine,Nanjing 210046,Jiangsu Province,China)

机构地区：[1]南京中医药大学附属医院血液科,江苏南京210029 [2]南京中医药大学第一临床医学院,江苏南京210046

出　　处：《中国实验血液学杂志》2018年第6期1598-1603,共6页Journal of Experimental Hematology

基　　金：国家自然科学基金面上项目(81274139);国家自然科学基金青年基金(81703888)

摘　　要：目的:探讨附加8号染色体克隆演变在慢性髓系白血病(chronic myeloid leukemia,CML)病程进展中的意义。方法:报道1例CML患者在两次急粒变时出现8号染色体三体或四体的演变。结果:本例患者经历两次慢性髓系白血病急粒变(CML-AML),均发生了附加8号染色体。第一次急变检测到8号染色体三体(trisomy 8),第二次急变检测到8号染色体四体(tetrasomy 8),且有Myc基因异常表达,伴发中枢神经系统白血病,大剂量阿糖胞苷联合甲氨蝶呤获得短暂缓解。结论:附加8号染色体克隆演变是CML快速进展、预后不良的重要标志。Objectives: To explore the clinical significance of clonal evolution of additional chromosomal 8 in CML progression.Methods: An unusual case with the clonal evolution from trisomy 8 to tetrasomy 8 accompanied by 2 time of CML blast crisis ( BC) was reported.Results: This patient suffered from 2 time of CML blast crisis and the additional chromosome 8 aberrations were accompanied.Trisomy 8 and tetrasomy 8 were detected at first CML blast crisis and second CML blast crisis, respectively.After tetrasomy 8 was developed,the c-Myc was over-expressed and the central nervous system leukemia happened in this case.Only high dose Ara-C and MTX regimen could induce remission for a short period.Conclusion: These findings suggested that additional chromosome 8 aberrations are important marker for poor prognosis of CML patients and contribute to a poor prognosis.

关 键 词：8号染色体三体 8号染色体四体 克隆演变 慢性髓系白血病急粒变 

分 类 号：R733.72[医药卫生—肿瘤]