急性早幼粒细胞白血病两种PML—RARα融合基因亚型的临床研究  

Clinical study on two PML-RARα fusion gene isoforms in patients with acute promyelocytic leukemia

作　　者：袁燕慧[1] 吴德沛[2] 刘跃钧[2] 欧阳建[1] 

机构地区：[1]南京大学医学院附属鼓楼医院血液科,210008 [2]苏州大学附属第一医院血液科

出　　处：《白血病．淋巴瘤》2009年第5期274-276,共3页Journal of Leukemia & Lymphoma

摘　　要：目的探讨急性早幼粒细胞白血病（APL）PML-RARα融合基因亚型及其临床关系。方法采用巢式反转录聚合酶链反应（RT—PCR）检测92例初诊APL患者PML—RARα融合基因不同转录本，根据结果分为长型（L型）和短型（S型）两组，比较两组间的临床特征、治疗反应及预后。结果92例APL患者PML—RARα融合基因均为阳性，L型52例，占56．5％，S型40例，占43．5％；两组相比，患者性别、年龄、治疗前的白细胞计数、骨髓原始早幼粒细胞比例及染色体无明显差异；诱导治疗的完全缓解（CR）率、达CR的时间、维甲酸综合征（RAS）、弥漫性血管内凝血（DIC）、颅内出血的发生差异无统计学意义；两组缓解后总体生存率（OS）及无复发生存率（RFS）亦差异无统计学意义。结论APL患者PML—RARα融合基因亚型与临床疗效、预后无关。Objective To illustrate the clinical relevance of distinct PML-RARα fusion gene isoforms in acute promyelocytic leukemia （APL）. Methods The nested reverse transeriptase polymerase chain reaction （RT-PCR） was used to detect the long （L） or short （S） PML-RARα fusion gene isoforms in 92 newly diagnosed APL so as to evaluate the clinical feature, therapeutic reaction and prognosis of the two fusion gene isoforms. Results PML-RARα fusion gene was positive in all 92 APL patients, of which 52（56.5 %） was L type and 40 （43.5 %） was S type. There were no significant differences between L type and S type in the aspect of sex, age, white blood cell count,the percentage of bone marrow blasts plus promyelocytes and chromosome before treatment. And there were no significant differences between the two isoforms in complete remission （CR） rate, the time of getting CR as well as the occurrence of retinoic acid syndrome （RAS）, disseminated intravaseular coagulation （DIC）, intraeranial hemorrhage. Also, there were no significant differences in overall survival rate （OS） and relapse-free survival rate （RFS） between the two isoforms. Conclusion PML-RARα fusion gene isoforms in APL were not correlated with clinical therapeutic effect or prognosis.

关 键 词：白血病 早幼粒细胞 急性 PML—RARα融合基因 受体 维甲酸 

分 类 号：R733.71[医药卫生—肿瘤] R733.7[医药卫生—临床医学]