Analysis of Clinical Features in 21 Patients with Hairy Cell Leukemia
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摘要: 目的 探讨初诊毛细胞白血病(hairy cell leukemia,HCL)患者的临床特点和治疗疗效。方法 回顾性分析我院初诊的21例患者临床及相关实验室检查资料。结果脾大是HCL最主要的症状体征,其次表现为全血细胞减少、骨髓增生活跃；少数患者电子显微镜可见核糖体－板层复合物；典型的毛细胞表达CD11c、CD25、CD123、CD103,同时还表达CD20、CD22、CD52和Cyclin D1,尤其CD103是HCL较特异的表型。核苷类似物治疗HCL疗效优于其他治疗,值得临床推荐。结论初诊的HCL临床特征与国外的基本相似；国内以氟达拉滨为主的联合化疗方案,无明显的骨髓蓄积抑制作用,且疗效高于国外。Abstract: Objective To investigate the clinical and therapeutic effects of hairy cell leukemia(HCL). Methods The clinical and laboratory characteristics of twenty-one patients with HCL were retrospectively analysed. Results Splenomegaly was the most common physical phenomenon.The others were pancytopenia and active bone marrow hyperplasia.Ribosomal-lamellae only accounted for a few patients.Classic HCL cell expressed CD11c,CD25,CD123,CD103, and companied with CD20,CD22,CD52 and Cyclin D1 expression.Especially,CD103 was HCL specific immunophenotype.The purine nucleoside analogues presented better therapeutic effects than other regiments. Conclusion The HCL clinical characteristics are similar to that in western patients.In Chinese patients fludarabine-based combination regimens has no significant accumulation of bone marrow inhibition,and the therapeutic effect is higher than that in foreign patients.
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Key words:
- Hairy cell leukemia /
- Clinical feature /
- Immunophenotype /
- Nucleoside analogues
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[1] Grever MR.How I treat hairy cell leukemia [J].Blood,2010,115(1)：21-8. [2] Else M,Dearden CE,Matutes E,et al.Long-term follow-up of 233 patients with hairy cell leukaemia,treated initially with pentostatin or cladribine,at a median of 16 years from diagnosis[J].Br J Haematol,2009,145(6)：733- 40. [3] Tadmor T.Purine analog toxicity in patients with hairy cell leukemia[J].Leuk Lymphoma,2011,52(Suppl 2)：38-42. [4] Dearden CE,Else M,Catovsky D,et al.Long-term results for pentostatin and cladribine treatment of hairy cell leukemia[J].Leuk Lymphoma,2011,52(Suppl 2)：21-4.
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