费城染色体阳性急性淋巴细胞白血病的临床研究

[Clinical study of Philadelphia chromosome-positive acute lymphoblastic leukemia].

作者信息

Bao Li, Jiang Bin, Huang Xiao-jun, Wang De-bing, Qiu Jing-ying, Lu Xi-jing, Chen Huan, Lu Dao-pei

机构信息

Institute of Hematology, People's Hospital, Peking University, Beijing 100044, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2005 Jan;26(1):31-4.

PMID:15946506

Abstract

OBJECTIVE

To study the clinical characteristics and therapeutic outcome of Ph+ acute lymphoblastic leukemia (ALL).

METHODS

Thirty previously untreated cases of Ph+ B-ALL were diagnosed in our institute. The patients were treated with combination chemotherapy of CODP +/- L regimen, Imatinib (400 approximately 600 mg/d) was continuously given to those who couldn't reach CR. Fourteen patients received allogeneic hematopoietic stem cell transplantation (allo-HSCT) after CR, while 16 received consolidation of intensive chemotherapy.

RESULTS

Thirty (32.6%) of 92 ALL patients were diagnosed as Ph+ ALL, with a median age of 25.5 (14 - 60). Among them Ph+ as the sole anomaly was seen in 16 patients, and Ph+ with additional chromosome abnormalities in 14. Besides the B cell markers, 23 (76.7%) patients had CD34+ and 13 (43.3%) CD13+ and/or CD33+. Nineteen of the Ph+ ALL patients underwent molecular analysis; 13 (68.4%) expressed P190 and 6 (31.6%) P210. Increased WBC (> 30 x 10(9)/L) was found in 22/30 cases while WBC > 100 x 10(9)/L in 9/30 cases. The chemotherapy complete remission rate was 68.8% in patients with only Ph+ versus 28.6% in those with additional chromosome abnormalities. All seven refractory/relapsed patients reached CR with Imatinib therapy. The total complete remission rate was 73.3% in all Ph+ ALL patients. The median remission duration was shorter in patients with additional chromosome than in those with only Ph+ (1 vs 7 months, P < 0.05), and so was the survival period (7 vs 9 months, P > 0.05). The remission duration was significantly longer in patients received allo-HSCT than in those received chemotherapy only (8 vs 0.5 month, P < 0.05), and so was the survival period (12.5 vs 6 months, P < 0.05).

CONCLUSION

Additional chromosome abnormalities negatively affect the prognosis and therapeutic effect of Ph+ ALL patients. Imatinib is effective for the induction therapy of Ph+ ALL. The survival period of patients who received allo-HSCT was obviously longer than those who received chemotherapy only.

摘要

目的

研究Ph+急性淋巴细胞白血病（ALL）的临床特征及治疗效果。

方法

我院确诊30例初治Ph+ B-ALL患者。患者接受CODP+/-L方案联合化疗，对未达完全缓解（CR）者持续给予伊马替尼（400～600mg/d）。14例患者CR后接受异基因造血干细胞移植（allo-HSCT），16例接受强化巩固化疗。

结果

92例ALL患者中30例（32.6%）诊断为Ph+ ALL，中位年龄25.5岁（14～60岁）。其中16例患者Ph+为唯一异常，14例Ph+合并其他染色体异常。除B细胞标志物外，23例（76.7%）患者有CD34+，13例（43.3%）有CD13+和/或CD33+。19例Ph+ ALL患者进行分子分析；13例（68.4%）表达P190，6例（31.6%）表达P210。30例患者中22例白细胞升高（>30×10⁹/L），9例白细胞>100×10⁹/L。单纯Ph+患者化疗完全缓解率为68.8%，合并其他染色体异常者为28.6%。7例难治/复发患者接受伊马替尼治疗后均达CR。所有Ph+ ALL患者总完全缓解率为73.3%。合并其他染色体异常患者的中位缓解期短于单纯Ph+患者（1个月对7个月，P<0.05），生存期也较短（7个月对9个月，P>0.05）。接受allo-HSCT患者的缓解期明显长于单纯化疗患者（8个月对0.5个月，P<0.05），生存期也较长（12.5个月对6个月，P<0.05）。