母细胞性浆细胞样树突状细胞肿瘤临床与实验室特点分析

Characteristics of blastic plasmacytoid dendritic cell neoplasm

目的对1例母细胞性浆细胞样树突状细胞肿瘤(BPDCN)患者的临床表型、形态学特点、免疫学表型、染色体核型等进行分析,以提高临床和实验室人员对此类病例的认识。方法分析1例BPDCN患者的临床表型、骨髓细胞形态学、免疫表型与遗传学结果,并结合文献总结BPDCN的临床特征。结果BPDCN患者骨髓涂片瑞氏-吉姆萨染色结果:骨髓增生明显活跃,原始细胞37.5%,该类细胞中等大小;核圆形、椭圆形或不规则形,染色质细致,核仁隐显不一;胞质量中等,灰蓝色,无颗粒,部分细胞胞质中有沿细胞膜分布的空泡和伪足;原始细胞过氧化物酶(POX)染色阴性;过碘酸-希夫(PAS)染色为红色颗粒状阳性。骨髓流式细胞学检测结果:异常细胞群占有核细胞的13.15%,强表达HLA-DR、CD56、CD123,表达CD33、CD7、CD4,部分表达CD38,不表达cCD3、cCD22、cMPO、CD3、CD10、CD34、CD117。染色体核型分析结果为46,XY(20)。骨髓细胞形态学和免疫表型结果均符合BPDCN的诊断标准。结论临床表型、细胞形态学和免疫表型对于诊断BPDCN都有重要意义,其中免疫表型必不可少。

Objective To report a case of blastic plasmacytoid dendritic cell neoplasm(BPDCN),and to detail the characteristics of its clinical manifestation,cell morphology,immunophenotype and karyotype analysis,which may show new light on the diagnosis of the disease.Methods The case was diagnosed by its clinical manifestation,cell morphology and immunophenotype.Literature review was performed to reveal the clinical characteristics of BPDCN.Results The results of bone marrow smear demonstrated the presence of a dramatically active myeloid hyperplasia,and the presence of the blast cell was 37.5%,with the characteristic of median size,circular or elliptic or irregular nucleus,loose chromatin,0 to 3 nucleolus,dispersed gray-blue cytoplasm and vacuolation which was often observed at the edge of cytoplasm.Peroxidase(POX)staining showed the blast cells were negative,while periodic acid-schiff(PAS)staining displayed that they were positive.Flow cytometric analysis of the bone marrow revealed the presence of the abnormal cells was 13.15%,and they were strongly positive for HLA-DR,CD56,CD123,positive for CD33,CD7,CD4,partially positive for CD38,and negative for cCD3,cCD22,cMPO,CD3,CD10,CD34,CD117.The result of karyotype analysis was 46,XY(20).The patient was diagnosed as BPDCN with the clinical manifestation,cell morphology and immunophenotype.Conclusions The characteristics of clinical manifestation,cell morphology and immunophenotype are of clinical significance to diagnose BPDCN,and the characteristics of immunophenotype is indispensable.