长期生存的急性早幼粒细胞白血病PML/RARα融合基因的变化及意义

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目的:观察长期生存的急性早幼粒细胞白血病(APL)PML/RARα融合基因的变化,探讨其临床意义。方法:运用筑巢式反转录聚合酶链(RT蛳PCR)方法,观察生存5 a以上的28例APL 的PML/RARα融合基因变化。结果:(1)初诊时10例PML/RARα融合基因测定9例阳性,阳性率90.0%。(2) 完全缓解后在不同时期阳性率测定结果:6个月时64.3 %,12 个月时46.7 %,24个月时31.6 %,36个月时25.0 %,48 个月时17.8 %,60个月时17.8 %。>60个月21.4 %。(3)3例60 个月时血象、骨髓为完全缓解状态,但PML/RARα融合基因阳性,其中2例62 个月、78 个月时复发。1例坚持化疗至96 个月时方转阴性,现持续缓解116 个月。3例PML/RARα融合基因由阴性转为阳性后复发。其余病例PML/RARα融合基因持续阴性,血象、骨髓完全缓解。(4)缓解后中止治疗的3例中,1例PML/RARα融合基因持续阳性在62 个月时复发死亡。2例由阴性转为阳性病例中1例在119 个月时复发死亡。1例CR 99个月复发,经白血康治疗后CR2。CR2 6个月时仍阳性。现生存112 个月。结论:长期生存的APL患者PML/RARα融合基因持续阳性或由阴性转为阳性常与复发有关。持续阴性常伴随长期无病生存。完全缓解后的维持治疗尚为一艰巨而长期的过程。对持续阳性病例,坚持长期、足够强烈的继续治疗,维持持续的首 Objective: To observe the changes of PML / RARα fusion gene in long-term survival of acute promyelocytic leukemia (APL), and to explore its clinical significance. Methods: The changes of PML / RARα fusion gene expression in 28 APL patients who survived for more than 5 years were observed by nested reverse transcription polymerase chain reaction (RT 蛳 PCR). Results: (1) In the first visit, 9 cases of PML / RARα fusion gene were positive, the positive rate was 90.0%. (2) The results of positive rate in different periods after complete remission were 64.3% at 6 months, 46.7% at 12 months, 31.6% at 24 months, 25.0% at 36 months, 17.8% at 48 months, 60 17.8% of months. > 60 months and 21.4%. (3) The hemogram of 3 cases at 60 months had a complete remission of bone marrow, but the PML / RARα fusion gene was positive, of which 2 cases relapsed at 62 months and 78 months. One case of adherence to chemotherapy to 96 months when the negative side, the ongoing remission of 116 months. Three cases of PML / RARα fusion gene from negative to positive recurrence. Other cases of persistent PML / RARα fusion gene, blood, bone marrow completely relieved. (4) Of the 3 patients who stopped treatment after remission, one case of PML / RARα fusion gene persisted positive and died at 62 months. One of the two cases, negative to positive, died of recurrence at 119 months. One patient had recurrent CR 99 months after treatment with Bai Xue Kang CR2. CR2 is still positive at 6 months. Now lives for 112 months. Conclusion: Long-term survival of APL patients with PML / RARα fusion gene from positive or negative to positive often associated with recurrence. Persistent negative often accompanied by long-term disease-free survival. Maintenance therapy after complete remission is still an arduous and long-term process. Persistent positive cases, adhere to the long-term, strong enough to continue treatment, to maintain sustained first
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