慢性中性粒细胞白血病伴意义未明单克隆免疫球蛋白增高1例并文献复习

Chronic neutrophilic leukaemia associated with monoclonal gammopathy of undetermined significance:a case report and literature review

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。

Objective：To study the rare blood disease of chronic neutrophilic leukaemia （CNI.） with elevated monoclonal gammopathy. Method： One case diagnosed as CNI. with elevated monoclonal gammopathy was reported and the related literature were reviewed. Result： The patient was characterized by sustained mature neutrophilic leu- kocytosis, splenomegaly,high neutrophil alkaline phosphatase score,absence of Ph chromosome and BCR-ABL fu- sion transcripts,granulocytic hyperplasia without evidence of dysplasia or striking reticulin fibrosis in bone marrow histology,and exclusion of underlying disorders manifesting with neutrophil leukoeytosis such as leukemoid reac tion and other hematologic clonal disorders. The significance of increased monoclonal gammopathy remain undeter mined,of which lymphoma had been excluded, not yet diagnosed with multiple myeloma. Conclusion：CNI. is a rare myeloproliferative disease,it is important to distinguish the relation and treatment of CNL and monoclonal gam- mopathy.