40例毛细胞白血病临床特征分析

Analysis of clinical features in 40 patients with hairy cell leukemia

目的:研究我国毛细胞白血病(HCL)的临床及实验室检查特点。方法:回顾性总结40例HCL患者的临床及相关实验室检查资料。结果:①脾大是HCL的最主要的症状体征,而全血细胞减少者比例较少,在经典型中也仅占1/3左右;变异型白细胞计数常增高;②电镜检查核糖体-板层复合物的阳性率仅有20%～30%;③多色流式细胞学表型分析发现典型的毛细胞表达CD19、CD20、CD22、CD25、CD103、CD11c和sIgM,是诊断的重要依据;④核苷类似物治疗HCL疗效优于其他治疗,值得推荐;⑤变异型HCL比例较高,表现不典型,且治疗效果差。结论:我国HCL中全血细胞减少者比例较低,电镜核糖体-板层复合物的阳性率低,而毛细胞免疫表型与国外类似,核苷类似物治疗效果也较好。变异型HCL比例高于国外,治疗反应差。

Objective：To investigate the clinical and laboratory characteristics of Chinese patients with hairy-cell leukemia（HCL）.Method：Forty patients with HCL were retrospectively analysed.Result：Splenomegaly was the most common physical finding.But pancytopenia only accounted for 1/3 even in classical HCL and leukocytosis was frequent in HCL.Ribosomal-lamellae complexes（RLC） were found only in about 20% to 30% of HCL cases.Most HCL cells expressed CD19,CD20,CD22,CD25,CD103,CD11c and sIgM.These immunophenotype characteristics were important for diagnosis.The purine nucleoside analogues presented better therapeutic effects than other regimens.The variant type of HCL was common and with poor response to therapy.Conclusion：Pancytopenia is infrequent in Chinese patients with HCL.The classical RLC is rare under electronmicroscope.The immunophenotype of HCL cells in Chinese patients is similar to that in western patients.Purine nucleoside analogues achieve better therapeutic effects.The variant type of HCL is common but with poor therapeutic response.