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急性早幼粒细胞白血病PML-RARα融合基因的临床研究

CLINICAL SIGNIFICANCE OF DETECTION OF PML RARα FUSION GENE OF APL
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摘要 目的 研究急性早幼粒细胞白血病PML-RARα融合基因的亚型、变化及其临床意义。方法 采用筑巢式逆转录酶聚合酶链反应(RT-PCR)技术,检测初治APL患者PML-RARα融合基因,监测完全缓解者微小残留病灶。结果 32例初治APL患者PML-RARα融合基因均为阳性(100%),其中S型为25%,L型为75%。L型的复发率及早期病死率分别为12.5%、12.5%,显著低于S型的复发率及早期病死率(25%和37.5%)。L型患者的预后比S型患者好。采用全反式维甲酸(ATRA)及小剂量高三尖杉酯碱诱导治疗,缓解后分别用化疗与维甲酸交替进行强化治疗,使PML-RARα阳性率随缓解期的延长而逐渐降低。同时监测16例APL缓解患者,PML-RARα融合基因阴性患者可长期生存。结论 PML-RARα融合基因的检测对APL的确诊、预测预后具有重要的临床意义。 Objective: To characterize of the expression patterns of PML-RARa fusion gene during differentiation therapy with ATRA. Methods: A 'nested' RT-PCR was used to study on 32 Acute Promyelocytic Leukemia (APL). Results: 2 major isoforms (type S and L) were detected in leukemia cells from 32 novel patients with APL. 8 of 32 (25.0%) patients with type S isoforms were accompanied with poor prognosis. 16 cases were detected sequentially by the RT-PCR method for dynamic observation of MSD during post remission therapy with ATRA and chemotherapy. The positive rate curve tended to downwards gradually. Other 16 cases of APL, long-term survivals and received the same post remission program, were detected by the RT-PCR method. The fusion gene was detectable and whoever without any abnormal evidence in bone marrow 10 cases was negative and had along-term survival CCR. Conclusion: The detection of PML-RARa fusion gene plays an important role in clinical diagnosis, evaluation of prognosis and prediction of relapse in APL.
出处 《实用临床医药杂志》 CAS 2003年第4期331-334,共4页 Journal of Clinical Medicine in Practice
基金 江苏省基础应用研究项目(BJ95122)
关键词 急性早幼粒细胞白血病 PML-RARα基因 ATRA PML-RARa fusion gene
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