摘要目的 提高对母细胞性浆细胞样树突细胞肿瘤(blastie plasmacytoid dendritic cell neoplasm,BPDC)的认识.方法 报道2例BPDC患者,并复习文献总结该病临床及实验窒检杏特点,介绍肿瘤细胞起源的最新进展.结果 2例患者均以皮肤结节起病,肿瘤细胞表达CD4和CD56,不表达髓系、T细胞以及B细胞特异性标志.对初始治疗敏感,但迅速复发,病程分别为26、11个月.结论 BPDC是少见的淋巴瘤亚型,具有独特的免疫表型,病程呈侵袭性,预后差.近期研究表明肿瘤细胞起源于浆细胞样树突细胞前体细胞.

abstractsObjective To identify the clinical and pathological features of blastic plasmacytoid dendritic cell neoplasm (BPDC). Methods The characteristics of BPDC hematodermic neoplasm were discussed with a report of two new cases and review the literatures. Results Both patients presented with skin nodules and the tumors were CD+4 and CD+56. Lineage specific markers for B- and T-cell were negative and the tumors did not express myeloperoxidase. Systemic chemotherapy resulted in complete remission, but the disease relapsed quickly and were unresponsive to further chemotherapy. The patients died 26 months and 11 months respectively after diagnosis. Conclasion BPDC hematodermic neoplasm is a rare subtype of lymphoma with distinct clinicopathologic and immunophenotypic features. The disease often has a fulminant course with a poor prognosis. More recent studies suggest that there is a derivation from a plasmacytoid dendritic cell precursor.