母细胞性浆细胞样树突状细胞瘤一例报告

母细胞性浆细胞样树突状细胞瘤 （BPDCN） 是一种罕见的造血系统肿瘤, 恶性程度高, 进展迅速, 对化疗敏感, 但易复发耐药, 生存时间短。现报告我科收治的1例BPDCN患者, 以皮损为首发表现, 经皮肤活检确诊。初期对淋巴细胞肿瘤方案敏感但很快复发, 累及肺、 纵隔, 后予髓系肿瘤方案, 效果不佳, 最终死亡, 总生存期17个月。

A case of blastic plasmacytoid dendritic cell neoplasm

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematopoietic neoplasm with high malignancy and rapid progression. BPDCN is sensitive to chemotherapy, but it is prone to relapse and drug resistance, and the survival time is short. We report a case of BPDCN hospitalized in our department. In this case, the skin lesions were the first manifestation. The diagnosis of BPDCN was confirmed by the skin biopsy. Initially, this patient was sensitive to the lymphoma chemotherapy but the disease relapsed quickly, spreading to the lungs and mediastinum. Then, we treated the patient with myeloid regimen, which was no efficacy. The patient eventually died with an overall survival of 17 months.