40例慢性淋巴细胞白血病临床分析  

Clinical Analysis on 40 Patients with Chronic Lymphocytic Leukemia

作　　者：许贞书[1] 张金燕[1] 战榕[1] 郑志宏[1] 吴顺泉[1] 陈志哲[1] 

机构地区：[1]福建医科大学附属协和医院血液科,福建福州350001

出　　处：《中国实验血液学杂志》2012年第3期583-586,共4页Journal of Experimental Hematology

基　　金：福建省高等学校新世纪优秀人才支持计划项目资助(编号JA10128)

摘　　要：本研究旨在分析慢性淋巴细胞白血病(CLL)的临床和实验室特点及其对预后的影响。对2004年1月至2010年12月收住本院的40例CLL患者进行了回顾性分析。结果表明,本病主要发生于老年男性,中位年龄66岁(42-80岁);25例(62.5%)可见典型CLL免疫表型,所有病例均表达CD19和CD5,表达CD38和Zap70的分别有7例(17.5%)和14例(35%);8例检测出IgVH基因突变,其中有4例为VH3家族;FISH检测出P53基因缺失6例,RB1缺失3例,12三体1例,正常核型5例;31例患者接受了含氟达拉滨的化疗,中位无进展生存(PFS)时间为48个月(95%CI:39-57个月),其中有27例(87.1%)获完全缓解(CR)+部分缓解(PR),而接受其他治疗方法的患者有9例,PFS仅为14个月(95%CI:10-18个月,P<0.001),只有3例(33.3%)获CR+PR。诊断时β2微球蛋白水平正常者,36个月的总生存率为78%(95%CI:69%-87%),明显高于β2微球蛋白水平异常者的总生存率47%(95%CI:35%-59%,P=0.004)。Zap70表达阳性的患者中有7例(50%)治疗后获CR+PR,疗效差于表达阴性者,后者中有23例(88.5%,P=0.006)治疗后获CR+PR。IgVH基因突变者,治疗后都获CR,无IgVH基因突变者,治疗后只有4例(66.7%)获CR。结论:CLL具有独特的临床特征和分子遗传学标记,如Zap70、CD38和IgVH基因突变,并与预后相关。氟达拉滨治疗方案能明显提高CLL患者的长期生存率。This study was aimed to analyze the clinical and laboratorial characteristics of patients with chronic lymphocytic leukemia（CLL）,as well as their relationship with outcomes of patients.The clinical and laboratorial data of 40 CLL patients admitted from 2004 to 2010 in our hospital were analyzed retrospectively.The results indicated that the most of CLL attacked the elderly male patients with median age 66（from 42 to 80）.Flow cytometric analysis showed that 25 cases were positive for typical immunophenotype of CLL.On the other hand,all the patients clearly expressed CD19 and CD5,7 cases（17.5%）and 14 cases（35%）were positive for the expression of CD38 and Zap70 respectively.8 cases harbored a mutated immunoglobulin heavy-chain（VH） gene,among them 4 cases belong to VH3 family.Interphase FISH analysis showed that P53 deletion,RB1 deletion,trisomy 12 and normal chromosome were detected in 6,3,1,and 5 cases,respectively.The median PFS in 31 patients received treatment of fludarabine based chemotherapy was 48 months（95% CI：39-57 months）,among them 27 cases（87.1%） achieved CR＋PR.While PFS was 14 months（95% CI：10-18 months,P〈0.001） in 9 patients received other treatment regimen,out of them only 3 ceses（33.3%） achieved CR＋PR.Patients with normal level of serum β2-microglobulin at diagnosis showed significantly higher overall survival（78%,95% CI：69%-87%） in 36 months than those with abnormal level of serum β2-microglobulin（47%,95% CI：35%-59%,P=0.004）.Significant difference in the rate of CR＋PR was noted in the Zap70 positive group（50%） and in negative group（88.5%,P=0.006）.All of 8 patients with IgVH mutation displayed CR after treatment,while 4 cases（66.7%） achived CR among 6 patients without IgVH mutation.It is concluded that CLL is characterized by high heterogeneity in both clinical features and molecular markers,which are associated with prediction of outcomes for patients.The treatment with fludarabine-based chemotherapy results in a major benefit

关 键 词：慢性淋巴细胞白血病 回顾性分析 Zap70 CD38 IGVH 氟达拉滨 

分 类 号：R733.72[医药卫生—肿瘤]