母细胞性浆细胞样树突细胞肿瘤二例并文献复习

Blastic plasmacytoid dendritic cell neoplasm： two cases report and review of literatures

目的提高对母细胞性浆细胞样树突细胞肿瘤（blastic 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室检查特点，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。

Objective 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 CD4^＋ and CD56^＋. 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. Conclusion 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.