Deng Wenjun, Yang Minghua, Kuang Feimei, Liu Yingting, Zhang Hui, Cao Lizhi, Xie Min, Yang Liangchun
Department of Pediatrics, Xiangya Hospital, Central South University, Changsha, Hunan 410008, P.R. China.
Mol Clin Oncol. 2017 Oct;7(4):709-715. doi: 10.3892/mco.2017.1370. Epub 2017 Aug 8.
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a newly characterized, rare malignant tumor of the skin and hematopoietic system. BPDCN occurs mainly in the elderly, whereas it is rarer among children, and has variable clinical manifestations. Optimal chemotherapeutic regimens for the treatment of BPDCN have not yet been determined and this tumor has a poor prognosis. In this study, two pediatric cases of BPDCN, including a 7-year-old female and a 9-year-old male patient, diagnosed at the Xiangya Hospital of Central South University over the past 2 years, were retrospectively reviewed. Both cases exhibited multiple organ involvement, although the clinical manifestations differed; they were diagnosed with BPDCN based on the clinical manifestations, pathological and immunohistochemical findings, which included positivity for CD4, CD56 and CD123. A high-risk acute lymphocytic leukemia (ALL) chemotherapy regimen was administered to both patients. The patient in the first case achieved a complete remission, but unfortunately her parents refused follow-up treatment and she succumbed to the disease 9 months after the initial diagnosis. The second patient was treated for a total of three courses with a chemotherapy regimen including daunorubicin, cytarabine and etoposide, followed by two courses of the high-risk ALL chemotherapy regimen; unfortunately, a remission was not achieved and the patient was scheduled to receive hematopoietic stem cell transplantation. Thus, not all pediatric BPDCN patients may be able to achieve complete remission following chemotherapy with the high-risk ALL regimen, and other treatment options must be investigated in the future.
母细胞性浆细胞样树突状细胞肿瘤（BPDCN）是一种新发现的、罕见的皮肤和造血系统恶性肿瘤。BPDCN主要发生于老年人，儿童中较为少见，临床表现多样。治疗BPDCN的最佳化疗方案尚未确定，且该肿瘤预后较差。在本研究中，回顾性分析了过去2年在中南大学湘雅医院确诊的2例儿童BPDCN病例，包括1例7岁女性和1例9岁男性患者。两例均表现为多器官受累，尽管临床表现有所不同；根据临床表现、病理及免疫组化结果确诊为BPDCN，免疫组化结果包括CD4、CD56和CD123阳性。两例患者均接受了高危急性淋巴细胞白血病（ALL）化疗方案。第一例患者达到完全缓解，但不幸的是其父母拒绝后续治疗，她在初次诊断后9个月死于该疾病。第二例患者总共接受了三个疗程的化疗方案，包括柔红霉素、阿糖胞苷和依托泊苷，随后接受了两个疗程的高危ALL化疗方案；不幸的是，未达到缓解，该患者计划接受造血干细胞移植。因此，并非所有儿童BPDCN患者采用高危ALL方案化疗后都能实现完全缓解，未来必须探索其他治疗选择。
