Tang Yuting, Wang Ying, Hu Liang, Meng Fankai, Xu Danmei, Wan Kai, Huang Lifang, Li Chunrui, Zhou Jianfeng
Department of Hematology, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology 1095 Jie-Fang Avenue, Wuhan 430030, Hubei, P. R. China.
Department of Oncology, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology 1095 Jie-Fang Avenue, Wuhan 430030, Hubei, P. R. China.
Int J Clin Exp Pathol. 2015 Nov 1;8(11):15294-300. eCollection 2015.
Acute Promyelocytic Leukemia (APL) is one of the most curable leukemia which shows great sensitivity to all-trans retinoic acid (ATRA) although a small number of the patients present poor prognosis and short survival. Isochromosome 17 in APL which usually bears an additional copy of RARA/PML fusion gene is considered to be a negative factor on its prognosis. Cryptic t(15;17) on i(17q) leads to an extra copy of PML/RARA rather than RARA/PML which may confer a worse prognosis. We describe here a rare APL case with complex chromosomal abnormality including isochromosome 17 bearing cryptic t(15;17) showing poor outcome. The patient lacks a classic t(15;17) and fluorescence in situ hybridization (FISH) presents 2 PML/RARA fusion signals on both long arms of the isochromosome. The patient also acquired a secondary mutation at relapse when the initial karyotype was already a complex karyotype involving chromosome 13, 17 and 22 at the same time. The poor response of this patient to traditional chemotherapy like ATRA and novel therapy like arsenic trioxide (ATO) suggests that early auto-hematological stem cell transplantation may be the choice of APL with isochromosome 17 especially with cryptic t(15;17) on i(17q). We are the first to show a clear history and evidence of FISH of these kind of cases. A small summary of cases with cryptic t(15;17) on isochromosome 17 is also made.
急性早幼粒细胞白血病(APL)是最可治愈的白血病之一,对全反式维甲酸(ATRA)表现出高度敏感性,尽管少数患者预后较差且生存期短。APL中的17号等臂染色体通常携带额外拷贝的RARA/PML融合基因,被认为是其预后的负面因素。17号等臂染色体上隐匿性t(15;17)导致额外拷贝的PML/RARA而非RARA/PML,这可能预示更差的预后。我们在此描述一例罕见的伴有复杂染色体异常的APL病例,包括携带隐匿性t(15;17)的17号等臂染色体,其预后不良。该患者缺乏典型的t(15;17),荧光原位杂交(FISH)显示在等臂染色体的两条长臂上均有2个PML/RARA融合信号。该患者在复发时还出现了二次突变,而初始核型已是同时涉及13号、17号和22号染色体的复杂核型。该患者对ATRA等传统化疗以及三氧化二砷(ATO)等新型疗法反应不佳,提示早期自体造血干细胞移植可能是伴有17号等臂染色体尤其是17号等臂染色体上有隐匿性t(15;17)的APL的治疗选择。我们首次展示了这类病例清晰的病史和FISH证据。同时还对17号等臂染色体上有隐匿性t(15;17)的病例进行了简要总结。