慢性中性粒细胞白血病27例临床分析

Clinical study of 27 cases of chronic neutrophilic leukemia

目的探讨慢性中性粒细胞白血病(CNL)的诊断、治疗和预后。方法回顾性分析1985-2015年我院收治的27例CNL患者的实验室特点、治疗方法和预后。结果 27例CNL患者血液中中性粒细胞持续增多,平均40.7×109/L,骨髓粒细胞系增生并以成熟中性粒细胞为主,杆状核粒细胞占(21.1±8.0)%,分叶核粒细胞占(44.4±17.1)%,中性粒细胞碱性磷酸酶(NAP)积分增高平均235.0分,绝大多数患者骨髓红细胞系统明显受抑;25例患者使用马利兰或羟基脲治疗后症状有所改善、脾脏缩小及血液中白细胞数下降;2例疗效不佳,仍然有中性粒细胞增多及脾肿大。结论 CNL是一种罕见类型的慢性白血病,以骨髓成熟中性粒细胞浸润为特点,化疗和酶抑制剂等疗法对其有一定作用。

Objective To evaluate the diagnosis, treatment and prognosis of chronic neutrophilic leukemia(CNL). Methods The laboratory features, methods of treatment and prognosis in 27 patients with CNL were retrospectively analyzed between 1985-2015. Results All patients demonstrated sustained neutrophilia in the blood, with an average of 40.7×109/L. Marrow examination showed mature neutrophil proliferation, with rod-shaped neutrophils accounting for(21.1±8.0)% and segmented neutrophils for(44.4±17.1)%. Neutrophil alkaline phosphatase(NAP) activity was increased as an average of 235.0. Bone marrow erythroid system was significantly inhibited in the majority of patients.Twenty-five patients responded to therapy by busulfan or hydroxyurea with control of leukocytosis and reduction in splenomegaly. The curative effects of the other two cases were poor, and they still had sustained neutrophilia and splenomegaly. Conclusion CNL was a rare type of chronic leukemia with characteristics of mature neutrophils infiltration in bone marrow. The therapies of chemotherapy and enzyme inhibitors may play a role in CNL.