Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematological malignancy, there is no standard treatment and the prognosis is very poor. Affiliated Zhongshan Hospital of Dalian University report a case of 85-year-old BPDCN male patient treated with DVT regimen (decitabine combined with Venetoclax and thalidomide) and achieved complete remission. The patient with skin nodules and the pathology diagnosed BPDCN, the next generation sequencing of skin nodules showed mutations of IDH2 and ASXL1. DVT (decitabine combined with Venetoclax and thalidomide) has significant efficacy with rapid and deep remission for BPDCN, and the adverse effects is less, especially suitable for elderly patients who cannot tolerate intense chemotherapy.
母细胞性浆细胞样树突细胞肿瘤（BPDCN）是一种罕见的具有高度侵袭性的血液系统恶性肿瘤，目前无统一治疗方案，预后极差。大连大学附属中山医院报道1例85岁BPDCN男性患者应用DVT方案（地西他滨联合维奈克拉、沙利度胺）治疗获完全缓解的病例。患者皮肤结节起病，病理提示BPDCN，皮肤结节二代测序提示IDH2、ASXL1热点突变。地西他滨联合维奈克拉、沙利度胺的DVT非化疗治疗方案对于BPDCN疗效显著，起效快，缓解程度深，安全性好，尤其适用于无法耐受强化疗的老年患者。.