Ph阳性急性淋巴细胞白血病21例临床分析

PHILADELPHIA CHROMOSOME POSITIVE ACUTE LYMPHOBLASTIC LEUKEMIA AN ANALYSIS OF 21 CASES

目的 :分析 Ph阳性急淋的细胞遗传学、临床疗效及预后 ,并对比成人和儿童患者的临床特点。方法 :总结 2 1例初治 Ph阳性急淋患者的 CR率 ,以及不同巩固方案的疗效 ,同时对白血病细胞的生物学特征进行研究。结果 :儿童与成人患者在疗效以及预后方面无显著性差异 ( P<0 .0 5 ) ;本组患者 CR率为 76 % ;在各种不同巩固方案中 ,大剂量 Ara-C DF S为 ( 10 .944± 1.774)月 ,中位生存时间 19月 ,未用大剂量 Ara-C DFS为 ( 2 .840± 0 .95 5 )月 ,中位生存时间 8月 ,P值分别为 0 .0 0 3、0 .182 ;CTX/VP1 6使用者 DFS为 ( 7.6 6 7± 1.85 8)月 ,中位生存时间 19月 ,未用 CTX/VP1 6DFS为 ( 2 .75 3± 1.0 46 )月 ,中位生存时间 8月 ,P值分别为 0 .0 395、0 .0 32 6。结论 :儿童与成人Ph阳性急淋临床表现相似 ,预后较差。巩固强化治疗使用大剂量 Ara-C、含 CTX/VP1 6的方案可能取得相对较好的疗效 ,但由于例数尚少 。

Objective:To analyze the cytogenetic characteristics,the outcome and prognosis of philadelphia (Ph) chromosome positive (+) acute lymphoblastic leukemia (ALL),and to compare adult Ph +ALL with children Ph +ALL.Methods:The complete remission (CR) rate of 21 newly diagnosed Ph positive ALL patients were analyzed,and the effectiveness of different consolidation regimens in the treatment of Ph +ALL were observed.The biological features of leukemic cells were explored by immunochemistry and cytogenetics.Results:There was no difference in the outcome and prognosis between adult Ph +ALL patients and childhood patients( P <0.05).In the 21 Ph +ALL patients,the CR rate was 76%.All patients received different consolidation regimens.With high dose Ara C,the disease free survival was 10.944±1.774 months,compared with 2.840±0.955 months with non receiving high dose Ara C( P =0.003),the median survival was 19 months compared with 8 months with non receiving high dose Ara C ( P =0.182).With cyclophospha mide/etoposide,the disease free survival was 7.667±1.858 months,compared with 2.753±1.046 months with non receiving cyclophosphamide/etoposide ( P =0.0395),the mdeian survival was 19 months compared with 8 months with non receiving cyclophosphamide/etoposide ( P =0.0326).Conclusion:The clinical features of childhood Ph +ALL were similar to those of adult Ph +ALL.Incorporating high dose Ara C or cyclophosphamide/etoposide containing regimens in consolidation therapies may be effective.