目的:研究慢性中性粒细胞白血病(CNL)伴单克隆免疫球蛋白增高这种罕见血液病。方法:报告1例CNL伴单克隆免疫球蛋白增高患者,并复习国内外相关文献。结果:患者中性粒细胞明显增多,脾大,中性粒细胞碱性磷酸酶积分增高,无Ph染色体及BCR/ABL融合基因,骨髓粒系增生,无病态造血和明显纤维化依据,排除了如类白血病反应、其他克隆性血液病引起的中性粒细胞增多,合并单克隆免疫球蛋白增高意义未明,目前已排除淋巴瘤,尚不能诊断为多发性骨髓瘤。结论:CNL是一种少见的骨髓增殖性疾病,CNL与单克隆免疫球蛋白增高的相关性和治疗方法需要进一步明确。 Aims: To investigate the association of chronic neutrophilic leukemia (CNL) with monoclonal immunoglobulin in this rare blood disease. Methods: One case of CNL with monoclonal immunoglobulin was reported, and the related literature at home and abroad was reviewed. Results: Patients with neutrophils increased significantly, spleen, neutrophil alkaline phosphatase score increased, no Ph chromosome and BCR / ABL fusion gene, bone marrow myeloid hyperplasia, no pathological hematopoiesis and obvious fibrosis basis, ruled out Such as leukemia reaction, other clonogenic hematopathy caused by neutrophils, combined with monoclonal immunoglobulin increased significance of unknown, lymphoma has been ruled out, yet to be diagnosed as multiple myeloma. CONCLUSION: CNL is a rare myeloproliferative disease. The correlation between CNL and monoclonal immunoglobulin and its treatment need to be further clarified.