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目的研究分析BCR/ABL阳性急性淋巴细胞白血病(BCR/ABL+-ALL)临床特征及治疗转归,筛选可能影响临床疗效的相关预后因素。方法我院2001年1月至2008年5月荧光原位杂交检查确诊为原发BCR/ABL+-ALL59例,经VDLP±Ara-C方案诱导化疗,化疗不缓解者或准备移植者给予伊马替尼治疗,其中接受伊马替尼联合治疗17例,行异基因造血干细胞移植(allo-HSCT)16例。结果59例BCR/ABL+-ALL患者中位年龄32(3～69)岁,经第1疗程诱导治疗后获CR共32例(54.2%)。外周血白细胞计数<30×109/L、30～99.9×109/L和≥100×109/L的CR率分别是75.0%(18/24例)、56.3%(9/15例)和26.3%(5/19例)(P=0.006),2年总生存率(OS)分别是24.7%、22.5%和21.1%(P=0.180)。FAB分型:L1、L2、急性混合性白血病的CR率分别是66.7%(6/9例)、63.2%(24/38例)和22.2%(2/9例)(P=0.029),2年OS分别是22.2%、27.0%和22.0%(P=0.623)。免疫分型:单纯ALL、伴髓系抗原表达ALL、急性混合性白血病的CR率分别是61.1%(11/18例)、60.6%(20/33例)和12.5%(1/8例)(P=0.039),2年OS分别是22.7%、21.0%和18.8%(P=0.643)。染色体核型:正常核型、单纯t(9;22)、Ph+伴附加染色体异常的CR率分别是71.4%(5/7例)、70.8%(17/24例)和37.5%(9/24例)(P=0.046),2年OS分别是42.9%、34.0%和7.3%(P=0.000)。是否合并败血症的2年OS分别是5.0%和36.0%(P=0.005)。含伊马替尼治疗组与不含伊马替尼治疗组、接受allo-HSCT治疗组和仅化疗组的2年OS分别是48.0%和11.2%(P=0.001)、54.2%和8.5%(P=0.000)。结论外周血白细胞计数≥100×109/L、形态学或免疫学类型为急性混合性白血病、Ph+伴附加染色体异常对BCR/ABL+-ALL的疗效有一定的负性影响,Ph+伴附加染色体异常、合并败血症者生存期短,伊马替尼联合治疗和allo-HSCT均可延长BCR/ABL+-ALL的生存期。
Objective To study the clinical characteristics and prognosis of BCR / ABL-positive acute lymphoblastic leukemia (BCR / ABL + -ALL) and to screen the related prognostic factors that may influence the clinical curative effect. Methods A total of 59 cases of primary BCR / ABL + -ALL were diagnosed in our hospital from January 2001 to May 2008 by fluorescence in situ hybridization. Indomethacin Nepalese treatment, of which 17 cases received imatinib combined treatment, allogeneic hematopoietic stem cell transplantation (allo-HSCT) in 16 cases. Results The median age of 59 patients with BCR / ABL + -ALL was 32 (3-69) years. After the first course of treatment, 32 patients (54.2%) had CR. The CR rates of peripheral blood leukocyte count <30 × 109 / L, 30 ~ 99.9 × 109 / L and ≥100 × 109 / L were 75.0% (18/24 cases), 56.3% (9/15 cases) and 26.3% (5/19 cases) (P = 0.006). The 2-year OS was 24.7%, 22.5% and 21.1% respectively (P = 0.180). FAB typing: The CR rates of L1, L2 and acute mixed leukemia were 66.7% (6/9), 63.2% (24/38) and 22.2% (2/9) respectively (P = 0.029) The annual OS was 22.2%, 27.0% and 22.0%, respectively (P = 0.623). Immunophenotyping: ALL, ALL with myeloid antigen expression, and CR rates of acute mixed leukemia were 61.1% (11/18), 60.6% (20/33) and 12.5% (1/8) P = 0.039). The 2-year OS was 22.7%, 21.0% and 18.8%, respectively (P = 0.643). Chromosomal karyotype: The CR rates of normal karyotype, simple t (9; 22), and Ph + with additional chromosomal abnormalities were 71.4% (5/7), 70.8% (17/24) and 37.5% (P = 0.046) and 2 years OS were 42.9%, 34.0% and 7.3%, respectively (P = 0.000). The 2-year OS with or without sepsis was 5.0% and 36.0%, respectively (P = 0.005). The 2-year OS was 48.0% and 11.2% (P = 0.001), 54.2% and 8.5%, respectively, in the imatinib-treated and non-imatinib-treated groups P = 0.000). Conclusions The peripheral blood leukocyte count≥100 × 109 / L, the morphology or immunology type is acute mixed leukemia, Ph + associated with additional chromosomal abnormalities have a certain negative effect on the efficacy of BCR / ABL + -ALL, Ph + with additional chromosomal abnormalities, Short-term survival of patients with combined sepsis, imatinib combined treatment and allo-HSCT can extend the survival of BCR / ABL + -ALL.