变异型毛细胞白血病1例报告并文献复习

目的：探讨变异型毛细胞白血病(HCL-V)的临床特征、诊断治疗及预后情况。方法：报告1例HCL-V的临床特征、诊断、治疗及预后情况，结合文献报道进行讨论。结果：该患者起病20d人院，人院时查有肝功能损害，人院后弥漫性血管内凝血持续存在，外周血白细胞进行性增高，人院第10天确诊为HCL-V，予氟达拉滨化疗及支持对症处理后，患者出现多脏器功能衰竭，病情恶化，于发病第40天死亡。结论：HCL-V有其独特的临床、病理学以及免疫学特征，诊断治疗上较为困难，部分预后极差。

Hairy-cell leukemia variant:a case report and review of the literatures

objective: To study the clinical features, differential diagnosis, therapy and prognosis of hairy-cell leukemia variant (HCL-V). Method:A case with HCL-V was reported and the literatures were reviewed. Result:A 37 years old woman was admitted because of edema of the face and feet, elevated lymphocyte count, liver function abnormalities and disseminated intravascular coagulation(DIC). She was diagnosed HCL-V on the tenth day and then treated with fludarabine, who died of multiple organ system failure finally.Conclusion: HCL-V is a unique clinicopathologic entity characterized by special immunophenotype and eletron microscopy features, which is difficult to be diagnosed and some of them with the poor prognosis.