Kong Qing-Tao, Zhang Min, Sang Hong, Chen Jun, Yan Wenliang, Hu Wenxing, Deng Dequan, Xie Qimei
Jinling Hospital, Nanjing University.
Dermatol Online J. 2014 Dec 16;21(3):13030/qt3b65n3px.
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a recently described, rare hematologic malignancy with prominent skin involvement. The prognosis of this disease is poor in most cases, with rapid progression despite chemotherapy administration. The first reported case of this disease was in 1994, and less than 200 cases worldwide have been described in the literature to date. Moreover coexistence of BPDCN and MDS is extremely rare. In this study, we describe a typical patient with BPDCN in China who presented with cutaneous involvement as the first manifestation associated with MDS; a brief review of literature is also given.
母细胞性浆细胞样树突状细胞肿瘤（BPDCN）是一种最近才被描述的罕见血液系统恶性肿瘤，皮肤受累较为突出。大多数情况下，这种疾病的预后较差，尽管进行了化疗，病情仍会迅速进展。该疾病的首例报告病例于1994年出现，迄今为止，全球文献报道的病例不到200例。此外，BPDCN与骨髓增生异常综合征（MDS）并存极为罕见。在本研究中，我们描述了一名中国的典型BPDCN患者，该患者以皮肤受累为首发表现，并伴有MDS；同时也对相关文献进行了简要回顾。
