【摘要】 目的:研究变异性Ph+白血病患者的临床特点及其预后。方法:对4例有完整资料可供分析的变异性Ph+成人白血病患者进行形态学、细胞遗传学、免疫学和分子生物学检查,并对其治疗预后进行观察。结果:在总数4例患者中,3例为慢性髓系白血病患者(其中1例处于慢性期,2例处于加速期),1例为成人急性B淋巴细胞白血病患者。患者细胞遗传学检查表明:4例中2例CML为t(9;22;14)异常,1例t(5;9;22)异常,而BCR/ABL融合基因3例均为e14a2型;1例成人B-ALL t(9;22;17)异常,其BCR/ABL融合基因为e13a3型。4例全部接受含甲磺酸伊马替尼方案治疗。结果表明,1例e13a3型BCR/ABL融合基因阳性成人B-ALL分子生物学持续缓解4个月后复发,并于第10个月死亡;而3例CML患者目前仍处于分子生物学缓解阶段,无病生存期分别达10、19、37个月。结论:变异性Ph染色体阳性白血病患者对第一代酪氨酸激酶抑制剂治疗反应良好,但BCR/ABL融合基因为e13a3型患者的预后有待于进一步研究证实。更多还原

【Abstract】 Objective: To study the clinical characteristics and prognosis of patients with variant Ph chromosomepositive leukemia. Methods: The defection of morphology, cytogenetics, immunology and molecular biology was performed in 4 cares of variant Ph chromosome-positive leukemia, and the therepeuitics outcome of 4 patients was evaluated. Results: Among 4 cases of variant Ph+ leukemia, 3 cases were patients with CML, including 1 case in chronic phase and 2 cases in accelerated phase; and 1 cases was patient with adult B acute lymphoblasric leukemia(B-ALL).The defecfion of cytogenetics in 4 cases showed that 2 cases of CML displayed t(9; 22; 14) abnormality, 1 case of CML displayed t(5; 9; 22) abnormality, moreover, the BCR/ABL fution gane in 3 cases of CML all was e14 a2 type, 1 cases of adult B-ALL disylayed t(9; 22; 17) abnormatlity, BCR/ABL fution gene of this case was e13 a3 type, 4 patients all received treatment wire chemotherapeptic regimen contaiming methanesulfanate imatinib. As a result, 1 cases of adult B-ALL with e13 a3 type BCR/ABL fusion gene positive relapsed after molecular biology remission for 4 months and died in the 10 th month; and yet 3 cases of CML are still in molecular biology remission, the disease-free survival time of these3 cases was 10, 19 and 27 months respectively. Conclusion: The patients with variant Ph chromosome-positive leukemia will response to the first generation tyrosine kinase inhibitors, but the prognosis of patients with e13 a3 type of BCR/ABL fusion gene remains to be further explored.更多还原