母细胞性浆细胞样树突状细胞肿瘤临床和病理特征分析

Clinical and Pathological Features of Blastic Plasmacytoid Dendritic Cell Neoplasm

目的:归纳并分析母细胞性浆细胞样树突状细胞肿瘤(BPDCN)的临床资料,为进一步认识该类疾病提供依据。方法:回顾性分析11例BPDCN患者的临床表现、免疫表型及病理特点、治疗与预后。结果:11例明确诊断BPDCN的患者中,男性8例、女性3例,中位年龄44(6-81)岁。临床主要以皮疹、包块等为首发症状,并伴有淋巴结、骨髓受累。肿瘤性母细胞性浆细胞样树突状细胞(pDC)表达HLA-DR、CD4、CD56、CD123,不表达cCD3、cMPO、cCD79a,部分病例可表达CD38、CD99、CD36。临床上手术切除、多次化疗失败的患者复发快,患者生存期短。首次化疗达到完全缓解的患者pDC细胞不表达CD56,且经骨髓移植后具有较长的生存期。结论:BPDCN免疫表型具有异质性,CD56是区分肿瘤性和正常pDC细胞的良好标志物;化疗缓解后进行造血干细胞移植的BPDCN患者预后较好。

Objective:To summarize and analyze the clinical features of blastic plasmacytoid dendritic cell neoplasm(BPDCN),so as to enhance the understanding of this disease.Methods:The clinical manifestations,immunophenotype,pathological features,treatment and prognosis of 11 cases of BPDCN were retrospectively analyzed.Results:Among the 11 patients diagnosed with BPDCN,there were 8 males and 3 females,with a median age of 44(6-81)years.The main clinical symptoms were rash and mass,accompanied by lymph node and bone marrow involvement.The neoplastic plasmacytoid dendritic cells(pDC)were positive for HLA-DR,CD4,CD56 and CD123,but negative for cCD3,cMPO and cCD79a;In some cases,they were also positive for CD38,CD99 and CD36.Patients who have underwent surgical resection and those who experienced multiple chemotherapy failures tend to have rapid recurrence and shorter survival time.Patients who achieved complete remission(CR)after the first chemotherapy exhibit no expression of CD56 on pDC cells,and tend to have a longer survival time after bone marrow transplantation.Conclusion:The immunophenotype of BPDCN is heterogeneous.CD56 is a reliable marker to distinguish neoplastic pDC cells from reactive pDC cells.The BPDCN patients who underwent hematopoietic stem cell transplantation(HSCT)after achieving remission from initial chemotherapy tend to have a better prognosis.