慢性中性粒细胞白血病国内文献135例分析

 目的　总结国内慢性中性粒细胞白血病（CNL）的临床资料。方法　以"慢性中性粒细胞白血病"为主题词,检索中国知网（www. cnki. net）的中国期刊全文数据库（1994年至2008年）。结果　检索到CNL136例,男77例,女59例,男女比例1.305∶1,平均年龄57.8（8～87）岁。以头昏乏力、体检发现白细胞增高、上腹部不适或包块为主要症状,绝大多数脾大;外周血成熟中性粒细胞增高且持久,并以分叶核细胞为主,血清维生素B12及尿酸水平升高;骨髓增生明显或极度活跃,所有患者碱性磷酸酶（NAP）增高,Ph染色体和bcr-abl融合基因阴性。结论　CNL是罕见的一种慢性白血病,需与类白血病反应和慢性粒细胞白血病鉴别;CNL患者病程长短不一,有向急性白血病转化可能;目前以姑息治疗为主。

Analysis of domestic literature of 135 cases of chronic neutrophilic leukemia

Objective To summarize clinical data of chronic neutrophilic leukemia(CNL) in China.Methods Chronic neutrophilic leukemia was searched as key words in full text database of Chinese periodical(www. cnki. net)(from 1994 to 2008). Results 136 cases of CNL were found, including male 77 cases and female 59 cases. Male: female ratio was 1.305:1. The mean age was 57.8 (8 ～87) years old. The common symptoms was fatigue,granulocytosis and upper abdominal discomfort or tumour. The splenomegaly was found in the majority of cases. The mature neutrophilic granulocyte in peripheral blood was high and cast longer time with raised segmented and band staged of neutrophils. Serum assay showed a raised level of VitB12 and uric acid. Bone marrow aspirate showed a hypercellular marrow with marked granulocytic proliferation, consisting mainly of stab forms and segmented neutrophils. The NAP score was high. Both Ph chromosome and bcr-abl fusion gene was negtive. Conclusion CNL was a scarce chronic leukemia. It should be discriminated with leukemoid reaction and chronic granulocytic leukemia. The course of CNL patients was long or short. It could transform into acute leukemia and palliative therapy was mainly applied at present.