慢性淋巴细胞白血病IgV(H)基因突变研究进展

慢性淋巴细胞白血病(CLL)免疫球蛋白重链易变区[IgV(H)]基因突变的研究已成为目前CLL异质性研究的热点。根据IgV(H)基因突变情况将CLL分为两型,本文从两型生物学特性、CD38表达、细胞遗传学改变、预后、性别、细胞形态和治疗等方面的异质性作一综述。     

慢性淋巴细胞白血病IgV（H）基因突变研究进展

慢性淋巴细胞白血病（CLL）免疫球蛋白重链易变区[IgV(H)]基因突变的研究已成为目前CLL异质性研究的热点。根据IgV(H)基因突变情况将CLL分为两型，本文从两型生物学特性，CD38表达，细胞遗传学改变。预后，性别，细胞形态和治疗等方面的异质性作一综述。     

慢性淋巴细胞白血病IgV（H）基因突变研究进展

慢性淋巴细胞白血病(CLL)免疫球蛋白重链易变区[IgV(H)]基因突变的研究已成为目前CLL异质性研究的热点。根据IgV(H)基因突变情况将CLL分为两型，本文从两型生物学特性、CD38表达、细胞遗传学改变、预后、性别、细胞形态和治疗等方面的异质性作一综述。     

慢性淋巴细胞白血病正调节基因1与慢性淋巴细胞白血病相关性的研究进展

慢性淋巴细胞白血病正调节基因1(CLLU1)位于染色体12q21.33-12q22,是一种新近发现的高水平表达于慢性淋巴细胞白血病(CLL)的基因.CLLU1与CLL的临床分期、免疫球蛋白重链可变区(IgVH)基因突变、CD38和zeta链相关蛋白-70等多种预后因素相关,可作为判断CLL预后的重要指标,对CLL临床靶向治疗具有一定的指导价值.     

慢性淋巴细胞白血病患者IgVH基因突变研究

目的 探讨我国慢性淋巴细胞白血病(CLL)患者IgVH基因各家族成员的发生频率以及突变情况.方法 应用多重PCR技术检测29例CLL患者的IgVH基因突变,纯化PCR扩增产物后直接测序,测序结果应用IMGT/V-QUEST数据库进行比对分析,明确有无IgVH突变及突变位置.结果 21例患者有体细胞突变,占CLL患者的72.4%;8例无突变.VH3家族16例,占CLL患者的55.2%,其中体细胞突变13例,占81.2%;VH4家族11例,占CLL患者的37.9%,其中突变7例,占63.6%;1例(3.5%)IgVH 2-5*10发生体细胞突变;1例(3.5%)IgVH 7-4-1*02无突变;未发现VH1、VH5和VH6家族成员.结论 我国CLL患者IgVH基因家族表达比例与西方国家存在显著差异,可能与种族和环境因素有关,这也可能是西方国家与我国CLL发病率差异的原因之一.     

慢性淋巴细胞白血病的治疗现状

慢性淋巴细胞白血病(CLL)是由于一种淋巴细胞克隆性增殖,逐步积累而浸润骨髓、血液、淋巴结和其它器官,最终导致造血功能衰竭的一种恶性疾病。CLL通常发生干60岁以上的老年人,男性多于女性。无症状患者的预期寿命与一般人群差异不大,但应定期随访。瘤可宁是标准治疗药物,能延缓有症状患者的进展;瘤可宁治疗无效时常使用氟达拉滨单药或联合其它制剂进行治疗;单克隆抗体也是治疗CLL的一种较有希望的药物;此外,同种移植能使部分CLL患者获得治愈。     

基因atm与慢性淋巴细胞白血病

共济失调毛细血管扩张突变基因(atm)为肿瘤抑制基因,位于人类染色体的11q22-23,主要参与DNA损伤识别和修复,并在DNA双链断裂诱导的信号级联转导通路中起到枢纽作用。慢性淋巴细胞白血病(CLL)可出现高频率的atm基因杂合性缺失和核苷酸突变,并与其发病及侵袭性病程有关。本文将对atm基因的特点、作用机制及其在慢性淋巴细胞白血病中作用的研究进展作一综述。     

基因atm与慢性淋巴细胞白血病

共济失调毛细血管扩张突变基因（atm）为肿瘤抑制基因，位于人类染色体的11q22-23，主要参与DNA损伤识别和修复，并在DNA双链断裂诱导的信号级联转导通路中起到枢纽作用。慢性淋巴细胞白血病（CLL）可出现高频率的atm基因杂合性缺失和核苷酸突变，并与其发病及侵袭性病程有关。本文将对atm基因的特点、作用机制及其在慢性淋巴细胞白血病中作用的研究进展作一综述。     

慢性淋巴细胞白血病的治疗现状

慢性淋巴细胞白血病(CLL)是由于一种淋巴细胞克隆性增殖，逐步积累而浸润骨髓、血液、淋巴结和其它器官，最终导致造血功能衰竭的一种恶性疾病。CLL通常发生于60岁以上的老年人，男性多于女性。无症状患者的预期寿命与一般人群差异不大，但应定期随访。瘤可宁是标准治疗药物，能延缓有症状患者的进展；瘤可宁治疗无效时常使用氟达拉滨单药或联合其它制剂进行治疗；单克隆抗体也是治疗CLL的一种较有希望的药物；此外，同种移植能使部分CLL患者获得治愈。     

慢性淋巴细胞白血病微小残留病研究进展

近年来,很多技术用于慢性淋巴细胞白血病微小残留病的检测,本组比较目前常用的流式细胞术及聚合酶链反应这两种技术的优缺点,并进一步探讨微小残留病在慢性淋巴细胞白血病预后中的意义。     

慢性淋巴细胞白血病DNA甲基化谱的研究进展

各种类型肿瘤具有特征性的DNA甲基化模式。本文就慢性淋巴细胞白血病(CLL)DNA甲基化谱、DNA甲基化在CLL发病机制、预后评估中的作用等方面作一综述,阐明CLL与基因DNA甲基化的关系。     

203例慢性淋巴细胞白血病患者预后相关因素分析

目的 探讨慢性淋巴细胞白血病(CLL)患者预后的主要影响因素.方法 回顾性分析2000年至2007年就诊于中国医学科学院血液病医院并获得有效随访的203例CLL患者临床资料,收集可能影响预后的因素,以Kaplan-Meier法绘制生存曲线,用Log-rank检验进行单因素分析,运用COX回归模型评估独立预后因素.结果 全组CLL患者中位随访时间为48.0(3.0～156.0)个月,5年总体生存(OS)率为(87.3±2.4)%,10年OS率为(77.4 ±3.3)%,死亡48例(23.6%).单因素分析显示临床分期为晚期、有B症状、结外器官受累、受累淋巴区≥3个、肝脏肿大、Hb<100 g/L、BPC<100 ×109/L、外周血淋巴细胞计数(ALC)>50 ×109/L、形态学表现为混合细胞型、病程中出现分期进展、对治疗无反应、并发感染、并发第二肿瘤或类型转化为不良预后因素.多因素分析显示受累淋巴区≥3个和彤态学表现为混合细胞型为独立的不良预后因素,根据这两项结果重新分组,低危、中危、高危组患者5年OS率分别为(89.8±3.5)%、(66.4±7.2)%、(15.0±13.8)%.各组间差异均具有统计学意义(P值均<0.05).结论 初诊时受累淋巴区数和CLL细胞形态学特征有助于评估CLL患者的预后.     

慢性淋巴细胞白血病治疗进展

本文综述慢性淋巴细胞白血病(CLL)治疗指征和药物选择方面的研究进展,归纳了目前较为一致的意见;介绍了新药嘌呤类似物在CLL的应用和难治性CLL的治疗方法。     

慢性淋巴细胞白血病治疗进展

本文综述慢性淋巴细胞白血病治疗指征和药物选择方面的研究进展，归纳了目前较为一一致的意义；介绍了新药嘌呤类似物在ＣＬＬ的应用和难治性ＣＬＬ的治疗方法。     

慢性淋巴细胞白血病/小淋巴细胞淋巴瘤的研究进展

慢性淋巴细胞白血病(CLL)/小淋巴细胞淋巴瘤(SLL)为高度异质性的成熟B淋巴细胞恶性克隆性疾病.目前对于CLL/SLL的相关研究,特别是对其发病机制的更加深入的研究,以及不断完善的预后评估指标方面,均取得了较大的进展.为了给CLL/SLL的临床诊疗提供参考,笔者拟就CLL/SLL的发病机制、诊断、分期及预后评估、治疗的相关研究进展进行综述.     

203例慢性淋巴细胞白血病患者预后相关因素分析

Objective To explore prognostic factors in patients with chronic lymphocytic leukemia (CLL). Methods Two hundred and three CLL patients in our hospital between 2000 to 2007 were retro-spectively reviewed for prognostic factor analysis. Survival was analysed by Kaplan-Meier analysis, univariate analysis by Log-rank test and multivariate analysis by COX regression model. Results With a median follow-up time of 48.0 (3.0 - 156.0) months, the 5-year overall survival (OS) rate was (87.3 ± 2.4) % and 10-year OS rate was (77.4 ± 3.3) %. Forty-eight (23.6%) patients died. Univariate analysis indicated that ad-vanced clinical stage, B symptoms, extranodal involvement, number of lymph node regions involved ≥3, en-larged liver, Hb < 100 g/L, BPC < 100 × 109/L, absolute lymphocyte count (ALC) > 50 × 109/L, atypical cell morphology, progression to stage, non-respons to treatment, complicating infections and secondary cancer or disease transformation were associated with poor prognosis. And on multivariate analysis, lymph node re-gion involvod≥3 and atypical cell morphology were independent poor prognostic factors. Based on the two in-dependent poor prognostic factors, three risk groups were defined: low- (0 factor), intermediate-(one factor) and high-(two factors) groups. The 5 year OS rates were (89.8 ± 3.5) % , (66.4 ～ 7.2) % and (15.0 ±13.8)%, respectively, and the difference between them was statistically. Conclusion The number of lymph node region involved and cell morphology are useful for assessing CLL patients prognosis.     

203例慢性淋巴细胞白血病患者预后相关因素分析

Objective To explore prognostic factors in patients with chronic lymphocytic leukemia (CLL). Methods Two hundred and three CLL patients in our hospital between 2000 to 2007 were retro-spectively reviewed for prognostic factor analysis. Survival was analysed by Kaplan-Meier analysis, univariate analysis by Log-rank test and multivariate analysis by COX regression model. Results With a median follow-up time of 48.0 (3.0 - 156.0) months, the 5-year overall survival (OS) rate was (87.3 ± 2.4) % and 10-year OS rate was (77.4 ± 3.3) %. Forty-eight (23.6%) patients died. Univariate analysis indicated that ad-vanced clinical stage, B symptoms, extranodal involvement, number of lymph node regions involved ≥3, en-larged liver, Hb < 100 g/L, BPC < 100 × 109/L, absolute lymphocyte count (ALC) > 50 × 109/L, atypical cell morphology, progression to stage, non-respons to treatment, complicating infections and secondary cancer or disease transformation were associated with poor prognosis. And on multivariate analysis, lymph node re-gion involvod≥3 and atypical cell morphology were independent poor prognostic factors. Based on the two in-dependent poor prognostic factors, three risk groups were defined: low- (0 factor), intermediate-(one factor) and high-(two factors) groups. The 5 year OS rates were (89.8 ± 3.5) % , (66.4 ～ 7.2) % and (15.0 ±13.8)%, respectively, and the difference between them was statistically. Conclusion The number of lymph node region involved and cell morphology are useful for assessing CLL patients prognosis.     

203例慢性淋巴细胞白血病患者预后相关因素分析

Objective To explore prognostic factors in patients with chronic lymphocytic leukemia (CLL). Methods Two hundred and three CLL patients in our hospital between 2000 to 2007 were retro-spectively reviewed for prognostic factor analysis. Survival was analysed by Kaplan-Meier analysis, univariate analysis by Log-rank test and multivariate analysis by COX regression model. Results With a median follow-up time of 48.0 (3.0 - 156.0) months, the 5-year overall survival (OS) rate was (87.3 ± 2.4) % and 10-year OS rate was (77.4 ± 3.3) %. Forty-eight (23.6%) patients died. Univariate analysis indicated that ad-vanced clinical stage, B symptoms, extranodal involvement, number of lymph node regions involved ≥3, en-larged liver, Hb < 100 g/L, BPC < 100 × 109/L, absolute lymphocyte count (ALC) > 50 × 109/L, atypical cell morphology, progression to stage, non-respons to treatment, complicating infections and secondary cancer or disease transformation were associated with poor prognosis. And on multivariate analysis, lymph node re-gion involvod≥3 and atypical cell morphology were independent poor prognostic factors. Based on the two in-dependent poor prognostic factors, three risk groups were defined: low- (0 factor), intermediate-(one factor) and high-(two factors) groups. The 5 year OS rates were (89.8 ± 3.5) % , (66.4 ～ 7.2) % and (15.0 ±13.8)%, respectively, and the difference between them was statistically. Conclusion The number of lymph node region involved and cell morphology are useful for assessing CLL patients prognosis.     

203例慢性淋巴细胞白血病患者预后相关因素分析

Objective To explore prognostic factors in patients with chronic lymphocytic leukemia (CLL). Methods Two hundred and three CLL patients in our hospital between 2000 to 2007 were retro-spectively reviewed for prognostic factor analysis. Survival was analysed by Kaplan-Meier analysis, univariate analysis by Log-rank test and multivariate analysis by COX regression model. Results With a median follow-up time of 48.0 (3.0 - 156.0) months, the 5-year overall survival (OS) rate was (87.3 ± 2.4) % and 10-year OS rate was (77.4 ± 3.3) %. Forty-eight (23.6%) patients died. Univariate analysis indicated that ad-vanced clinical stage, B symptoms, extranodal involvement, number of lymph node regions involved ≥3, en-larged liver, Hb < 100 g/L, BPC < 100 × 109/L, absolute lymphocyte count (ALC) > 50 × 109/L, atypical cell morphology, progression to stage, non-respons to treatment, complicating infections and secondary cancer or disease transformation were associated with poor prognosis. And on multivariate analysis, lymph node re-gion involvod≥3 and atypical cell morphology were independent poor prognostic factors. Based on the two in-dependent poor prognostic factors, three risk groups were defined: low- (0 factor), intermediate-(one factor) and high-(two factors) groups. The 5 year OS rates were (89.8 ± 3.5) % , (66.4 ～ 7.2) % and (15.0 ±13.8)%, respectively, and the difference between them was statistically. Conclusion The number of lymph node region involved and cell morphology are useful for assessing CLL patients prognosis.     

203例慢性淋巴细胞白血病患者预后相关因素分析

Objective To explore prognostic factors in patients with chronic lymphocytic leukemia (CLL). Methods Two hundred and three CLL patients in our hospital between 2000 to 2007 were retro-spectively reviewed for prognostic factor analysis. Survival was analysed by Kaplan-Meier analysis, univariate analysis by Log-rank test and multivariate analysis by COX regression model. Results With a median follow-up time of 48.0 (3.0 - 156.0) months, the 5-year overall survival (OS) rate was (87.3 ± 2.4) % and 10-year OS rate was (77.4 ± 3.3) %. Forty-eight (23.6%) patients died. Univariate analysis indicated that ad-vanced clinical stage, B symptoms, extranodal involvement, number of lymph node regions involved ≥3, en-larged liver, Hb < 100 g/L, BPC < 100 × 109/L, absolute lymphocyte count (ALC) > 50 × 109/L, atypical cell morphology, progression to stage, non-respons to treatment, complicating infections and secondary cancer or disease transformation were associated with poor prognosis. And on multivariate analysis, lymph node re-gion involvod≥3 and atypical cell morphology were independent poor prognostic factors. Based on the two in-dependent poor prognostic factors, three risk groups were defined: low- (0 factor), intermediate-(one factor) and high-(two factors) groups. The 5 year OS rates were (89.8 ± 3.5) % , (66.4 ～ 7.2) % and (15.0 ±13.8)%, respectively, and the difference between them was statistically. Conclusion The number of lymph node region involved and cell morphology are useful for assessing CLL patients prognosis.