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59例BCR/ABL阳性急性淋巴细胞白血病患者的临床特征与预后

[Clinical characteristics and outcomes of 59 patients with acute lymphoblastic leukemia positive for BCR/ABL].

作者信息

Liu Zhi, Liu Xiao-Li, Du Qing-Feng, Xu Na, Zhong Min, Song Lan-Lin, Yi Zheng-Shan, Liu Qi-Fa, Meng Fan-Yi, Zhou Shu-Yun

机构信息

Department of Hematology, Nanfang Hospital, Southern Medical University, Guangzhou, China.

出版信息

Nan Fang Yi Ke Da Xue Xue Bao. 2009 Mar;29(3):512-5.

Abstract

OBJECTIVE

To study the clinical characteristics and outcomes of BCR/ABL-positive acute lymphoblastic leukemia (BCR/ABL360888725-ALL) and screen the prognostic factors for BCR/ABL360888725-ALL.

METHODS

From January 2001 to May 2008, 59 patients (median age of 32 years ranging from 3 to 69 years) with the diagnosis of BCR/ABL360888725-ALL by fluorescence in situ hybridization received induction chemotherapy with VDLP-/+Ara-C regimen. The patients who failed to respond to the chemotherapy received subsequent consolidation chemotherapy with imatinib (400-800 mg/day) (17 cases) or allogeneic hematopoietic stem cell transplantation (allo-HSCT) (16 cases).

RESULTS

Of the 59 patients, 32 (58.3%) achieved complete remission (CR) after the first induction cycle. In patients with peripheral white blood cell (WBC) count <30=10(9)/L, 30-99.9(9)/L and > or =100(9)/L, the CR rates were 75.0% (18/24), 56.3% (9/15) and 26.3% (5/19) (P=0.006), and the overall survival probability of 2 years ( OSs of 2-yrs) was 24.7%, 22.5% and 21.1%, respectively (P=0.180). According to the FAB classification, 56 cases were divided into L1, L2 and biphenotypic acute leukemia (BAL) subgroups, and their CR rates were 66.7% (6/9), 63.2% (24/38) and 22.2% (2/9) (P=0.029), with OSs of 2-yrs of 22.2%, 27.0% and 22.0%, respectively (P=0.623). In terms of immunophenotype grouping by EGIL, the patients with ALL, myeloid antigen-positive ALL and BAL had CR rates of 61.1% (11/18), 60.6% (20/33) and 12.5% (1/8) (P=0.039), and the OSs of 2-yrs of 22.7%, 21.0% and 18.8%, respectively (P=0.643). In 55 patients with known karyotype, the CR rates were 71.4%(5/7), 70.8% (17/24) and 37.5% (9/24) in normal, sole t(9;22) abnormality, t(9;22) with additional abnormalities groups (P=0.046), with the OSs of 2-yrs of 42.9%, 34.0% and 7.3%, respectively (P=0.000). The patients complicated by septicemia had significantly lower OSs of 2-yrs than those without septicemia (0% vs 38.8%, P=0.005). The OSs of 2-yrs were significantly higher in patients with consolidation chemotherapy with imatinib than those without (48.0% vs 11.2%, P=0.001), and allo-HSCT was associated with significantly higher OSs of 2-yrs than exclusive chemotherapy (54.2% and 8.5%, P=0.000).

CONCLUSION

BCR/ABL360888725-ALL with WBC> or =100 x 10(9)/L, presence of BAL diagnosed by FAB or FACM, t(9;22) with additional chromosome abnormalities all adversely affect the treatment results, and additional chromosome abnormalities and septicemia are associated with lower OSs of 2-yrs. Imatinib treatment and allo-HSCT can both improve the OSs of 2-yrs of the patients with BCR/ABL(+)-ALL.

摘要

目的

研究BCR/ABL阳性急性淋巴细胞白血病(BCR/ABL360888725-ALL)的临床特征及预后,筛选BCR/ABL360888725-ALL的预后因素。

方法

2001年1月至2008年5月,59例(年龄中位数32岁,范围3至69岁)经荧光原位杂交诊断为BCR/ABL360888725-ALL的患者接受VDLP-/+阿糖胞苷方案诱导化疗。化疗无效的患者随后接受伊马替尼(400-800mg/天)巩固化疗(17例)或异基因造血干细胞移植(allo-HSCT)(16例)。

结果

59例患者中,32例(58.3%)在第1个诱导周期后达到完全缓解(CR)。外周血白细胞(WBC)计数<30×10⁹/L、30-99.9×10⁹/L和≥100×10⁹/L的患者,CR率分别为75.0%(18/24)、56.3%(9/15)和26.3%(5/19)(P=0.006),2年总生存概率(OSs of 2-yrs)分别为24.7%、22.5%和21.1%(P=0.180)。根据FAB分类,56例分为L1、L2和双表型急性白血病(BAL)亚组,其CR率分别为66.7%(6/9)、63.2%(24/38)和22.2%(2/9)(P=0.029),OSs of 2-yrs分别为22.2%、27.0%和22.0%(P=0.623)。按EGIL免疫表型分组,ALL、髓系抗原阳性ALL和BAL患者的CR率分别为61.1%(11/18)、60.6%(20/33)和12.5%(1/8)(P=0.039),OSs of 2-yrs分别为22.7%、21.0%和18.8%(P=0.643)。55例已知核型的患者中,正常、单纯t(9;22)异常、t(9;22)伴其他异常组的CR率分别为71.4%(5/7)、70.8%(17/24)和37.5%(9/24)(P=0.046),OSs of 2-yrs分别为42.9%、34.0%和7.3%(P=0.000)。合并败血症的患者2年OSs显著低于未合并败血症者(0%对38.8%,P=0.005)。接受伊马替尼巩固化疗的患者2年OSs显著高于未接受者(48.0%对11.2%,P=0.001),allo-HSCT患者的2年OSs显著高于单纯化疗者(54.2%和8.5%,P=0.000)。

结论

WBC≥100×10⁹/L、FAB或FACM诊断为BAL、t(9;22)伴其他染色体异常的BCR/ABL360888725-ALL均对治疗结果产生不利影响,其他染色体异常和败血症与较低的2年OSs相关。伊马替尼治疗和allo-HSCT均可提高BCR/ABL(+)-ALL患者的2年OSs。

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