母细胞性浆细胞样树突状细胞肿瘤一例临床病理分析

Blastic plasmacytoid dendritic cell neoplasm： a case report and review of literature

目的 探讨母细胞性浆细胞样树突状细胞肿瘤（BPDCN）的临床病理学特征、诊断与鉴别诊断.方法 对1例BPDCN患者的临床病理特征、免疫表型以及治疗和预后情况进行分析,复习文献资料.结果 患者为女性,发病年龄32岁,皮肤斑块或结节.活组织检查形态学显示侵及皮肤和骨髓,镜下肿瘤细胞由小至中等大小幼稚细胞组成,细胞质稀少,核形不规则,染色质细腻,核仁不明显.免疫组织化学示肿瘤细胞CD4、CD56、CD123表达阳性,CD3、CD20、CD34、TdT、MPO表达阴性.结论 BPDCN是一种罕见的高度恶性的淋巴造血系统肿瘤,除侵及皮肤外还侵犯骨髓和淋巴结.该病预后差,应注意与其他淋巴造血系统肿瘤相鉴别.

Objective To investigate the feature,pathological diagnosis and differential diagnosis of blastic plasmacytoid dendritic cell neoplasm （BPDCN）.Methods The clinical and histological findings,immunophenotype,treatment and prognosis of one case with BPDCN were evaluated with review of the relevant literature.Results The female patient with age of 32 years presented as cutaneous lesions.Pathologic findings showed skin and bone marrow were involved.Microscopically,the tumors were composed of diffuse blasts with small to medium size,scant cytoplasm,highly irregular nuclear contours,dispersed nuclear chromatin and faint indistinct nucleolus.Immunohistochemical characteristics of the tumors were the positive expression of CD4,CD56 and CD123,and negative expression of CD3,CD20,CD34,TdT,MPO.Conclusion BPDCN is a rare and highly aggressive hematopoietic neoplasm.Except for the skin,bone marrow and lymph node can also be involved.The differential diagnosis is necessary from other haematopoietic and lymphoid tumors.