Cao Xin, Xu Wei, Wu Yu-Jie, Qiao Chun, Liu Qiong, Fan Lei, Miao Kou-Rong, Li Jian-Yong
Department of Hematology, the First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing 210029, China.
Zhonghua Xue Ye Xue Za Zhi. 2009 Jul;30(7):450-3.
To analyze the prognostic factors for chronic lymphocytic leukemia (CLL) with typical and atypical immunophenotype. The parameters analyzed included sex, age, Binet stages, absolute lymphocyte count (ALC), immunoglobulin heavy-chain variable region (IgVH) gene mutation status, ZAP-70 protein, CD38 expression and cytogenetic aberrations.
According to the clinical guideline and scoring system for CLL in Britain, among 77 patients, 61 patients with score 5 called typical immunophenotype CLL, 16 with score 4 or 3 were atypical immunophenotype CLL. Multiparameter flow cytometry was employed for immunophenotypic analysis in 77 CLL patients for CD5, CD19, CD23, FMC7, sIg, CD20, CD79b expression and ZAP-70 protein and CD38. IgVH mutation status was detected by multiplex RT-PCR and sequencing of the purified PCR amplification products. Fluorescence in situ hybridization (FISH) and a panel of probes were used to detect cytogenetic aberrations.
There was no significant difference between the two groups in sex, age, ZAP-70 and IgVH mutation status (P=0.398, P=0.189, P=0.268 and P=0.131, respectively). The incidence of ALC> or =50 x 10(9)/L, Binet B + C, CD38> or =30% in atypical CLL patients (43.8%, 87.5% and 43.8%, respectively) were higher than that in typical group (16.4%, 36.1% and 16.4%, respectively) (P=0.026, P<0.01 and P=0.026, respectively). The proportion of typical patients (26.8%) with a 13q14 deletion as sole abnormality was higher than that of atypical patients (7.6%), and that with deletion of 11q22 or 17p13 was lower than that of atypical patients (12.2% vs 46.2%) (P=0.022).
There were obvious differences between the typical immunophenotype CLL and atypical CLL in ALC, Binet stages, CD38 expression level and cytogenetic aberrations.
分析具有典型和非典型免疫表型的慢性淋巴细胞白血病(CLL)的预后因素。分析的参数包括性别、年龄、Binet分期、绝对淋巴细胞计数(ALC)、免疫球蛋白重链可变区(IgVH)基因突变状态、ZAP-70蛋白、CD38表达及细胞遗传学异常。
根据英国CLL的临床指南和评分系统,77例患者中,61例评分为5分的患者为典型免疫表型CLL,16例评分为4分或3分的患者为非典型免疫表型CLL。采用多参数流式细胞术对77例CLL患者进行免疫表型分析,检测CD5、CD19、CD23、FMC7、sIg、CD20、CD79b表达及ZAP-70蛋白和CD38。通过多重RT-PCR及对纯化的PCR扩增产物进行测序检测IgVH突变状态。采用荧光原位杂交(FISH)及一组探针检测细胞遗传学异常。
两组在性别、年龄、ZAP-70及IgVH突变状态方面无显著差异(P分别为0.398、0.189、0.268和0.131)。非典型CLL患者中ALC≥50×10⁹/L、Binet B + C期、CD38≥30%的发生率(分别为43.8%、87.5%和43.8%)高于典型组(分别为16.4%、36.1%和16.4%)(P分别为0.026、P<0.01和0.026)。仅13q14缺失作为唯一异常的典型患者比例(26.8%)高于非典型患者(7.6%),而11q22或17p13缺失的典型患者比例低于非典型患者(12.2%对46.2%)(P = 0.022)。
典型免疫表型CLL与非典型CLL在ALC、Binet分期、CD38表达水平及细胞遗传学异常方面存在明显差异。