慢性中性粒细胞白血病2例并文献复习

Chronic neutrophilic leukemia:two cases report and literature review

目的:研究对慢性中性粒细胞白血病(CNL)的特点。方法:报告2例CNL并结合文献进行复习。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、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,其中1例伴有单克隆免疫球蛋白血症,2.5年后转化为急性白血病。结论:CNL是一种少见的骨髓增殖性疾病,多发生于老年人,预后差,治疗无标准方案,年轻患者应进行异基因造血干细胞移植以达到治愈目的。

Objective：To study the feature of chronic neutrophilic leukemia （CNL）. Methods; Two cases diagnosed as CNL were reported and the related articles were reviewed. Results; The patients were characterized by sustained mature neutrophilic leukocytosis, splenomegaly, high neutrophil alkaline phosphatase score, absence of Ph chro- mosome and bcr - abl fusion transcripts, granulocytic hyperplasia without evidence of dysplasia or striking retieulin fi- brosis in bone marrow histology, and exclusion of underlying disorders manifesting with neutrophil leukoeytosis such as leukemoid reaction and other hematologic clonal disorders. One case was associated with monoclonal gammopathy and finally developed transformation of acute leukemia after 2.5 years. Conclusion ： CNL is a rare myeloproliferative dis- ease and generally affects elderly patients. Most patients have poor prognosis. Ahhouth the optimal treatment of CNL remains to be defined, curative therapy including allogeneic hematopoietic stem cell transplantation should be attempted in young pations with CNL.