Yang Yunning, Xu Yuanyuan, Nie You, Wang Hongwei, Long Shan, Li Xiaosong
Department of Oncology, Fourth Medical Centre of Chinese PLA General Hospital, Beijing, China.
Department of Pathology, Fourth Medical Centre of Chinese PLA General Hospital, Beijing, China.
J Int Med Res. 2019 Oct;47(10):5281-5288. doi: 10.1177/0300060519871860. Epub 2019 Sep 17.
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare haematological malignancy that derives from plasmacytoid dendritic cells. The cancer is characterized by aggressive development and dismal prognosis, and due to limited prospective study, there is currently no established standard treatment. In this study, the case of a 77-year old female patient with BPDCN, who presented with a cutaneous lesion on the right of her face, is described. The lesion developed into a serious ulcer due to rapid disease progress, thus, a surgical excision was performed. As the patient refused to receive radiotherapy or haematopoietic stem cell transplantation following surgery, a new palliative combination chemotherapy was administered, comprising gemcitabine, nedaplatin and bleomycin. The therapy gave satisfactory results in terms of short-term treatment response and was well-tolerated. Published literature regarding BPDCN is also reviewed.
母细胞样浆细胞样树突状细胞肿瘤(BPDCN)是一种罕见的血液系统恶性肿瘤,起源于浆细胞样树突状细胞。该癌症具有侵袭性发展和预后不良的特点,由于前瞻性研究有限,目前尚无既定的标准治疗方法。在本研究中,描述了一名77岁患有BPDCN的女性患者的病例,该患者面部右侧出现皮肤病变。由于疾病进展迅速,病变发展为严重溃疡,因此进行了手术切除。由于患者术后拒绝接受放疗或造血干细胞移植,给予了一种新的姑息性联合化疗方案,包括吉西他滨、奈达铂和博来霉素。该疗法在短期治疗反应方面取得了满意的结果,且耐受性良好。同时还对关于BPDCN的已发表文献进行了综述。