大颗粒淋巴细胞白血病的临床特征分析  

Analysis of clinical characteristics for large granular lymphocytic leukemia

作　　者：杨文娟[1] 齐军元[1] 李增军[1] 刘薇[1] 易树华[1] 徐燕[1] 赵耀中[1] 邱录贵[1] 

机构地区：[1]中国医学科学院北京协和医学院血液学研究所血液病医院淋巴瘤诊疗中心,天津300020

出　　处：《中华医学杂志》2014年第4期276-279,共4页National Medical Journal of China

摘　　要：目的探讨大颗粒淋巴细胞白血病（LGL）的临床特征及实验室特点。方法回顾性分析中国医学科学院北京协和医学院血液学研究所血液病医院淋巴瘤诊疗中心2004年3月至2013年5月确诊的35例LGL患者的临床及实验室特征。结果LGL起病大多隐匿，中位确诊年龄51岁，以贫血相关症状就诊者多见。11例（31．4％）患者表现为睥脏轻中度肿大，1例（2．8％）患者合并类风湿关节炎。血常规检查有贫血的患者占77．1％（27／35），外周血LGL细胞绝对值波动于（0．82～23．7）×109／L。19例（54．2％）患者表现为典型的CD3^＋CD57^＋CD56-惰性T-LGL。2例患者表现为复杂染色体核型。8例行T细胞受体可变区β链（TCRVβ）检测的患者全部表现为阳性。侵袭性LGL患者的中位总生存时间明显短于惰性LGL患者（16个月比未达到，P=0．000）。单因素分析，有B症状的患者的中位总生存时间明显低于无B症状的患者（19比45个月，P=0．039），初诊时血小板减少的患者中位总生存时间显著低于血小板正常的患者（16比42个月，P=0．000）。多因素分析时，B症状（P=0．736）和血小板减少（P=0．977）均不是LGL的独立预后因素。结论我国LGL以类风湿关节炎起病者明显低于国外报道，TCRVβ检测在这一疾病诊断和鉴别诊断中具有重要意义，侵袭性LGL患者的生存期明显低于惰性患者，更需积极治疗。Objective To explore the clinical and laboratory characteristics for large granular lymphocytic leukemia （LGL）. Methods A total of 35 LGL patients were retrospectively analyzed from March 2004 to May 2013 at our department. Results Their median age of diagnosis was 51 years. The anemia-related symptoms included splenomegaly （n = 11, 31.4%）. And one patient （2.8%） with a history of rheumatoid arthritis, Anemia was the most frequent hematological abnormality （ n = 27, 77.1% ）. The range of LGL count in peripheral blood was （ 0. 82 - 23.7 ） x 109/L. Among them, 19 patients （54. 2% ） showed the CD3^＋CD57^＋CD56-indolent LGL phenotype. Two patients showed complex karyotype. T cell receptor β chain variable region （TCRVβ） analysis was positive in 8 patients. The median overall survival of aggressive LGL patients was significant shorter than that of indolent patient（ 16 months vs not reached, P = 0. 000）. Univariate analysis showed the median overall survival of patients with B symptom was significant shorter than that without B symptom （I9 vs 45 months, P = 0. 039）; the median overall survival of patients with thrombacytopenia was significant shorter than that with platelet normal （ 16 vs 42 months,P = 0. 000 ）. Multivariate analysis showed B symptom （ P = 0. 736） and thromboeytopenia （ P = 0. 977）at diagnosis were not prognostic factors. Conclusions Chinese LGL patient with rheumatoid arthritis is infrequent compared with foreign reports. TCRVβ analysis is important for its diagnosis. The overall survival of aggressive LGL patients is signicantly inferior to indolent patients, and need earlier intensive therapy in order to achieve longer survival.

关 键 词：白血病 大颗粒淋巴细胞 疾病特征 

分 类 号：R733.7[医药卫生—肿瘤]