慢性NK细胞增多症患者临床及实验室特征分析——八例报告并文献复习

Chronic natural killer cell lymphocytosis： eight cases report and literature review

目的 提高对慢性NK细胞增多症（CNKL）的认识.方法 结合相关文献和8例患者资料对CNKL患者的临床及实验室特征进行分析.结果 8例患者确诊时中位年龄55.5（28～ 75）岁,大多数患者临床症状轻微,4例患者诊断时无临床症状.血常规异常主要表现为白细胞[中位值11.8（4.5 ～20.7）×109/L]及淋巴细胞比例[中位值0.78（0.51～0.89）]增高,少数患者伴有贫血或血小板减少.流式细胞术检测外周血NK细胞（CD3-CD 16＋细胞）计数增高[中位值5.7（2.4～9.6）×109/L].8例患者中1例失访,4例未给予治疗,3例患者分别给予苯丁酸氮芥、甲泼尼龙、泼尼松治疗,获得较好治疗反应.中位随访47（11～78）个月,7例患者病情稳定.结论 CNKL患者临床症状轻微,外周血NK细胞（CD3 CD16＋细胞）增多,预后较好.

Objective To identify the characteristics of chronic natural killer cell lymphocytosis （CNKL）.Methods The clinical data of eight cases defined by the World Health Organization classification was retrospectively analyzed and related literatures were reviewed.Results Half of the 8 patients were asymptomatic.Among them,the most common abnormalities were leukocytosis with the median of 11.8 （4.5-20.0） × 109/L and high proportion of lymphocytes [0.78 （0.51-0.89）],while anemia and thrombocytopenia only in 1 patient respectively.The absolute CD3-CD16＋ NK cell count with the median of 5.7 （2.4-9.6） × 109/L increased in peripheral blood.By the end of follow-up,except one case was lost,the other seven patients were in stable condition,including four cases without any medications and three patients receiving chlorambucil or glucocorticoid.Conclusions As an indolent chronic lymphoproliferative disease,CNKL was presented with mild clinical symptoms and increased number of CD3 CD16 ＋ NK cells in peripheral blood.Patients with symptoms could be treated with immunosuppressive therapy while those asymptomatic could be followed up without intervention.