中性粒细胞异常增殖相关的BCR/ABL^-慢性白血病的临床特征  

Clinical features of BCR/ABL^- chronic leukemia associated with abnormal neutrophilia

作　　者：吕泰霞[1] 王建宁[2] 

机构地区：[1]南京医科大学第二附属医院检验医学中心,江苏南京210011 [2]南京医科大学第二附属医院血液科,江苏南京210011

出　　处：《现代肿瘤医学》2017年第8期1295-1299,共5页Journal of Modern Oncology

摘　　要：目的:探讨与中性粒细胞异常增殖相关的BCR/ABL-慢性白血病的临床特征。方法:报告3例不同类型与中性粒细胞异常增殖相关的BCR/ABL-慢性白血病并结合文献进行讨论。结果:3例均为老年男性,表现为以中性粒细胞为主的白细胞异常增多、BCR/ABL融合基因阴性。例1诊断为慢性中性粒细胞白血病(CNL),脾大,外周血以成熟中性粒细胞增高为特征,胞浆内中毒颗粒易见,无明显病态造血,中性粒细胞碱性磷酸酶(NAP)染色强阳性,骨髓粒系增生明显活跃。例2诊断为不典型慢性髓细胞白血病(a CML),血涂片示不成熟粒细胞比例增高伴粒系病态造血,尤其核染色质异常浓聚更为明显,骨髓象表现为髓系增生和病态造血,原始细胞轻度增多。例3诊断为慢性粒单核细胞白血病(CMML),外周血成熟单核细胞数量和比例异常偏高,粒系病态如核分叶过多、环状核和假性Pelger-Huet畸形等易见,骨髓髓系增生明显活跃伴病态造血,无原始细胞增多。结论:中性粒细胞异常增殖相关的BCR/ABL-慢性白血病较为少见,易于误诊,细胞形态学特征是诊断BCR/ABL-慢性白血病的基础。临床表现、多种实验室检查结果尤其是分子生物学等有助于正确诊断这类疾病。Objective： To explore the clinical features of BCR/ABL^-chronic leukemia associated with abnormal neutrophilia.Methods： Three cases of different kinds of BCR / ABL^-chronic leukemia associated with abnormal neutrophilia were reported and the related articles were reviewed.Results： Three patients were male elder and characterized by sustained neutrophilic leukocytosis,which were absence of BCR / ABL fusion transcripts.Case 1 was diagnosed as chronic neutrophilic leukemia（CNL） with splenomegaly.The characters of mature neutrophilic leukocytosis with no dysplastic features,toxic granulation of the neutrophils and strong positive of NAP-stain were noted in the peripheral blood（PB）.Bone marrow（BM） biopsy revealed hypercellular marrow due marked granulocytic hyperplasia.Atypical chronic myeloid leukemia（aCML） was made in case 2 by the following characteristics： PB smear showing left-shifted myeloid hyperplasia with marked granulocytic dysplasia including peculiar abnormal chromatin clumping,BM evaluation demonstrating myeloid hyperplasia and dysplasia with slightly blasts increase.We diagnosed case 3 as chronic myelomonocytic leukemia（CMML） whose presentation showed mature monocytosis with granulocytic dysplasia such as peculiar abnormal nuclear hypersegmentation,ring nucleus,and pseudo Pelger-Huet abnormity in the peripheral blood.BM biopsy revealed a hypercellularity with markedly increased myeloid and dysplasia without an expansion of blasts.Conclusion： CNL,a CML and CMML were considered extremely rare and may lead to be misdiagnosed.Making diagnosis is mainly based on the cellular characters of morphology for the BCR / ABL^-chronic leukemia.The various combinations of clinical manifestation and multiple laboratory data including molecular genetics may likely contribute to right diagnosis.

关 键 词：异常中性粒细胞增多 BCR/ABL融合基因 慢性中性粒细胞白血病 不典型慢性髓细胞白血病 慢性粒单核细胞白血病 

分 类 号：R733.7[医药卫生—肿瘤]