Ph染色体阳性的成人急性淋巴细胞白血病临床分析

目的 探讨Ph染色体阳性成人急性淋巴细胞白血病(Ph+ aALL)的临床特点,预后相关因素及治疗转归.方法 回顾性分析1995年1月至2009年12月收治的117例初治Ph+ aALL患者的临床资料,并随访生存期.结果 117例Ph+ aALL占同期727例初治成人ALL患者的16.1％.免疫表型大多数为前B型(64.1％)和普通型(31.3％);37.5％的患者伴有髓系抗原表达,98.4％的患者CD34表达阳性.总的完全缓解(CR)率为62％,中位缓解期6个月,中位生存期9个月.核型分析单纯t(9;22)异常占53％,t(9;22)附加其他染色体异常占47％,两组的CR率分别为59.6％和62.5％(P=0.768),中位生存期分别为7和4个月(P=0.158),3年总生存率为27.3％和14.4％(P=0.271).共同表达髓系抗原组与不表达髓系抗原组的CR率分别为56.0％和61.5％(P=0.750),中位生存期分别为5个月和4个月(P =0.182),3年总生存率分别为16.0％和15.0％(P=0.354).加用伊马替尼治疗和常规化疗组的CR率分别为81.3％和58.3％ (P =0.083),中位生存期分别为9.5和6个月(P=0.003),3年总生存率分别为52.2％和10.3％ (P =0.029).缓解后接受移植组和缓解后接受常规化疗巩固治疗组的中位生存期分别为15和6个月(P =0.000),3年总生存率分别为62.0％和10.3％(P=0.000).缓解后接受伊马替尼巩固维持治疗组和缓解后接受异基因造血干细胞移植组的中位生存期分别为12和15个月(P =0.300),3年总生存率分别为64.7％和62.0％(P=0.505).结论 Ph+ aALL约占aALL的16.1％,是一组预后不良的群体,常规化疗的CR率低,缓解期和生存期短;附加染色体异常和共同表达髓系抗原对CR率和预后无显著影响.在诱导治疗中加用伊马替尼能够显著提高Ph+ aALL患者的CR率.缓解后接受伊马替尼巩固维持治疗和接受异基因造血干细胞移植均可显著改善Ph+ aALL患者的长期生存率.

Clinical study of Philadelphia chromosome-positive adult acute lymphoblastic leukemia

Objective To study the clinical characteristics,risk factors and therapeutic outcome of Philadelphia chromosome-positive adult acute lymphoblastic leukemia( Ph + aALL).Methods The clinical data of 117 newly diagnosed adults with Ph + ALL in our hospital between January 1995 and December 2009were retrospectively analyzed.And their prognoses were followed up.Results There were 117 ( 16.1％ ) of 727 aALL patients diagnosed as Ph+ aALL.Among the 117 cases,64.1％ patients were classified as pre-B immunophenotype and 31.3％ as common B immunophenotype,37.5％ patients with co-expression of myeloid antigens (CD13 or CD33),and 98.4％ patients with positive CD34.The complete remission (CR) rate after 1 or 2 cycles of induction chemotherapy was 62.2％ in Ph+ aALL group versus 82％ in Ph - aALL group ( P =0.000).The median disease-free survival time of Ph+ group was 6 months and the median survival time was 9 months.Sole karyotype abnormality subgroup t (9;22) accounted for 53％ of all Ph+ aALL patients and additional karyotype abnormality subgroup,t (9 ; 22 ) plus other chromosome variation,accounted for 47％.Patients in sole karyotype abnormality subgroup had slightly lower CR rate ( 59.6％ vs 62.5％,P =0.768 ),longer median survival time (7 months vs 4 months,P =0.158),and higher 3-year overall survival rate (27.3％ vs 14.4％,P =0.271 ).For the myeloid antigen co-expressed patients and the only lymphocytic antigen expressed ones,CR rate was 56.0％ and 61.5％ ( P =0.750),the median survival time was 5 months and 4 months ( P =0.182 ),and the 3-year overall survival rate was 16.0％ and 15.0％ ( P =0.354),respectively. In the imatinib plus combination chemotherapy treatment group,81.3％ patients achieved CR,compared with that of 58.3％ in patients treated with only traditional combination chemotherapy (P =0.083).The median survival time was 9.5 months and 6 months (P =0.003 ) in these two subgroup,and 3-year overall survival rate was 52.2％ and 10.3％ ( P =0.029),respectively.For the patients receiving allo-HSCT after CR and that receiving traditional consolidation chemotherapy,the median survival time was 15 months and 6 months ( P =0.000),and the 3-year overall survival rate was 62.0％ and 10.3％ ( P =0.000 ),respectively.For the patients receiving imatinib as consolidation-maintenance treatment and that receiving allo-HSCT,the median survival time was 12 months and 15 months (P =0.300),and the 3-year overall survival rate was 64.7％ and 62％ ( P =0.505 ),respectively.Conclusion Of all adult ALL patients,the Ph + subgroup accounted for about 16.1％,which have unfavorable prognosis such as lower CR rate and shorter survival duration under traditional chemotherapy.Neither additional chromosome abnormalities to t(9;22) nor co-expression of myeloid antigen had negative effect on CR rate and survival duration.Addition of imatinib to the therapy was beneficial to improve the CR rate and survival duration.Either receiving imatinib as consolidation-maintenance treatment or alIo-HSCT after complete remission can improve long-term survival rate of Ph + adult ALL group significantly.