慢性中性粒细胞白血病5例临床分析

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：对5例CNL患者的临床表现，实验室特点，治疗方法和预后进行分析。结果：5例CNL患者中，4例脾脏肿大；所有患者血液中中性粒细胞持续增多，骨髓粒系增生并以成熟中性粒细胞为主，中性粒细胞碱性磷酸酶（NAP）积分增高，Ph染色体或bcr/abl融合基因阴性；使用马利兰及羟基脲治疗后患者症状有所改善，脾脏缩小及血液中白细胞数下降；1例患者诊断后7年发生急性变。结论：CNL是一种少见类型的慢性白血病，其特点与慢性髓细胞白血病不同；马利兰及羟基脲对其有一定疗效；预后具有异质性。

Clinical study on 5 cases of chronic neutrophilic leukemia

Objective:To evaluate the diagnosis, treatment and prognosis of chronic neutrophilic leukemia (CNL).Method:The clinical manifestations, laboratory features, methods of treatment and prognosis in 5 cases of CNL were analyzed. Result:Splenomegaly was present in 4 cases. All patients demonstrated sustained neutrophilia in the blood. Marrow examination showed mature neutrophil proliferation. Neutrophil alkaline phosphatase (NAP) activity was increased. No patient had the Philadelphia chromosome or the bcr/abl fusion gene. All responded to therapy with busulfan and hydroxyurea with control of leukocytosis and reduction in splenomegaly. One patient manifested blastic transformation after 7 years in diagnosis.Conclusion:CNL was a rare clinicopathologic entity that could distinguished from chronic myelogenous leukemia. The clinical course was heterogeneous.