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目的研究总结维吾尔族Ph染色体阳性急性淋巴细胞白血病(Ph+-ALL)的临床特征、治疗转归。方法对36例维吾尔族Ph+-ALL患者进行回顾性分析。结果 36例患者外周血白细胞(1.9~452.0)×109·L-1,中位数105.8×109·L-1,其中<30×109·L-111例,≥30×109·L-125例;免疫分型B-ALL 35例,TALL 1例,在B-ALL中,Pro-B 1例,Common-B 27例,Pre-B 7例。外周血白细胞<30×109·L-1和≥30×109·L-1患者的治疗完全缓解(CR)率分别是63.6%、28.0%(P=0.043)。正常核型、单纯t(9;22)、伴附加染色体异常的治疗CR率分别是66.7%、53.8%和15.4%(P=0.048),2年总生存率(OS)分别是33.3%、35.9%和10.3%(P=0.011)。联合伊马替尼与非联合伊马替尼治疗的CR率分别为88.9%、22.2%(P=0.001),2年OS分别是59.3%、10.9%(P=0.007)。治疗随访期间是否合并败血症的2年OS分别是0.0、38.8%(P=0.035)。结论维吾尔族Ph+-ALL的主要临床特征以外周血白细胞高、Common-B分型多见;初诊时伴外周血白细胞≥30×109·L-1或附加染色体异常的患者对治疗效果有一定的负性影响,伴附加染色体异常或治疗期间合并败血症的患者预后差,联合伊马替尼治疗可明显提高治疗CR率及延长患者生存时间。
Objective To study and summarize the clinical features of Ph + -ALL in Uygur Ph chromosomes and to evaluate the outcome. Methods 36 cases of Uygur Ph + -ALL patients were retrospectively analyzed. Results Thirty-six patients had peripheral blood leukocytes (1.9-452.0) × 109 · L-1 and a median of 105.8 × 109 · L-1, of which <30 × 109 · L-111 cases and ≥30 × 109 · L-125 cases There were 35 cases of B-ALL and 1 case of TALL in immunophenotyping, 1 case of Pro-B, 27 cases of Common-B and 7 cases of Pre-B in B-ALL. The complete remission (CR) rates of peripheral white blood cells <30 × 109 · L-1 and ≥30 × 109 · L-1 were 63.6% and 28.0%, respectively (P = 0.043). The CR rates of patients with normal karyotype, simple t (9; 22) and additional chromosomal abnormalities were 66.7%, 53.8% and 15.4% (P = 0.048) respectively. The overall 2-year survival rates were 33.3% and 35.9 % And 10.3% (P = 0.011). The CR rates of combined imatinib and non-combined imatinib treatment were 88.9% and 22.2%, respectively (P = 0.001). The 2-year OS rates were 59.3% and 10.9%, respectively (P = 0.007). The 2-year OS with or without sepsis during follow-up was 0.0, 38.8%, respectively (P = 0.035). Conclusions The main clinical features of Ph + -ALL in Uighur are high peripheral blood leukocyte count and Common-B typing. The patients with newly diagnosed peripheral blood leukocytes ≥30 × 109 · L-1 or additional chromosomal abnormalities have certain therapeutic effect Negative effects, with additional chromosomal abnormalities or treatment of patients with sepsis during the poor prognosis, combined with imatinib treatment can significantly improve the treatment of CR rate and prolong survival time.