260例CD5+慢性B淋巴增殖性疾病患者免疫表型分析

The immunophenotypic characteristics of 260 patients with CD5 ＋ B cell lymphoproliferativedisorders

【目的探讨我国CD5＋B淋巴增殖性疾病（B—LPD）免疫表型特点。方法采用流式细胞术分析B—LPD患者骨髓和（或）外周血细胞免疫表型特点，比较各免疫标志表达差异，并结合临床特点进行回顾性分析。应用荧光原位杂交（FISH）检测t（11；14）以诊断或排除套细胞淋巴瘤。结果①共260例患者纳入研究，其中慢性淋巴细胞白血病（CLL）186例（71．5％），套细胞淋巴瘤（MCL）38例（14．6％），其他B．LPD包括脾边缘区淋巴瘤（5例）、毛细胞白血病（3例）、B幼淋巴细胞白血病（2例）共10例（3．8％）；另外26例（10．0％）患者尚无法确诊亚类，暂定为不能分类的B．LPD（BLPD．U）。MCL患者均经FISH检测t（11；14）阳性而诊断，BLPD—U均为t（11；14）阴性。②所有患者均表达CDl9、CD20和CD5。依据CLL免疫表型积分系统，186例CLL患者积分均为4—5分，CLL患者CD23、sIgM、FMC7、CD22和CDllc阳性率分别为99．5％、11．3％、10．2％、44．1％和51．1％；MCL患者免疫表型积分均为1～3分，上述抗原阳性率分别为50．O％、50．0％、81．6％、92．1％和5．3％；BLPD．U患者上述抗原阳性率分别为73．1％、34．6％、88．5％、92．3％和69．2％；其他B．LPD患者上述抗原阳性率分别为50％、60％、100％、90％和70％。③CLL与MCL比较，CD23、sIgM、FMC7、CD22和CDllc表达在两者间差异均有统计学意义（尸值均〈0．01）；MCL与BLPD．U比较，仅CDllc表达在两者间差异有统计学意义（P〈0．01）；MCL与其他B．LPD比较，CDllc的表达在两者间差异有统计学意义（P〈0．01）。西方人群中，MCL中CD23表达率为7％～21％，sIgM的表达率为82％-100％，其免疫标志与本研究类似。结论我国MCL患者CD23阳性率显著高于西方人群，而sIgM表达率显著低于西方人群。CDllc可作为MCL与CLL及其他CD5＋B-LPD鉴别的有用标志。

Objective To explore the immunophenotypic characteristics of CD5 ＋ B cell lymphoproliferative disorders （B-LPD） of Chinese patients. Methods Immunophenotyping of bone marrow and （or） of peripheral blood was performed in patients with B-LPD by four color multiparameter flow cytometry analysis using a panel of monoclonal antibodies, and the patients clinical data were retrospectively analyzed. The difference in immunophenotypes and the related clinical features were retrospectively analyzed. Fluorescence in situ hybridization （FISH） for t （ 11; 14） detection was applied to diagnose or exclude mantle cell lymphoma. Results ①A total 260 CD5＋B-LPD patients were enrolled in this study, including 186 chronic lymphocytic leukemia （CLL）, 40 mantle cell lymphoma （MCL）, other B-LPD including 5 splenic marginal zone lymphoma （SMZL）, 2 B-cell prolymphocytic leukemia （B-PLL）, 3 hairy cell leukemia （HCL）. The other 26 cases （10%） were not classified and defined as unclassifed B- LPD （BLPD- U）. MCL patients were all positive for t （ 11; 14） detected by FISH, while allthe BLPD-U patients were negative for t （11;14）. ②All patients expressed CD19, CD20 and CD5. According to the immunophenotypic score system, 186 CLL patients scored 4-5, 99.5% of patients with CD23＋, 11.3% with sIgM＋, 10.2% with FMC7＋, 44.1% with CD22＋ and 51.1% with CDllc＋. MCL patients scored 2-3, with 50% expressing CD23 and sIgM, 81.6% expressing FMC7, 92.1% expressing CD22 and 5.3% expressing CDllc. In aspect of BLPD-U and other B-LPD, the expression of CD23, sIgM, FMC7, CD22 and CDllc were 73.1% and 50%, 34.6% and 50%, 88.5% and 100%, 92.3% and 90%, 69.2% and 70%, respectively. ③In comparison of CLL with MCL, there was a significant difference in the expression of CD23, sIgM, FMC7, CD22 and CDllc between the two groups （P〈0.01）. Between MCL and BLPD-U, similar expression type of CD23, sIgM, FMC7 and CD22 was found except CD1 lc, which was highly expressed in BLPD-U （P〈0.001）. The difference of CDllc expression was also statistically significant between MCL and other B-LPD （P 〈 0.01 ）. In comparison of MCL with other B-LPD, there was a significant difference in the expression of CD1 lc （P 〈 0.01 ）. The expression of CD23 and slgM in MCL are 7%-21% and 82%- 100% respectively in Western population, while the expression of other immunophenotypic markers is similar with our study. Conclusion The significant high incidence of CD23 and low incidence of sIgM compared to the Western population was observed in Chinese patients, and CD1 lc coud serve as a useful marker to distinguish MCL from CLL and other CD5＋ B-LPD.