Kim Marie Jeong-Min, Nasr Ahmed, Kabir Bilaal, de Nanassy Joseph, Tang Ken, Menzies-Toman Danielle, Johnston Donna, El Demellawy Dina
*Faculty of Medicine §Faculty of Health Sciences, University of Ottawa Departments of †Pediatric Surgery ∥Pediatric Pathology ‡Children's Hospital of Eastern Ontario, Research Institute ¶Department of Pediatrics, Division of Hematology/Oncology, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada.
J Pediatr Hematol Oncol. 2017 Oct;39(7):528-537. doi: 10.1097/MPH.0000000000000964.
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive hematologic malignancy characterized by frequent skin involvement that most commonly affects older patients. BPDCN is known to have a poor prognosis. Our objective was to assess if outcome and disease prognosis were independently influenced by age when evaluated with clinical presentation, sex, and treatment regimens. We conducted a systematic review to identify BPDCN cases, to compare pediatric BPDCN cases with adult cases. A total of 125 publications were identified detailing 356 cases. Including 1 pediatric case from our institution, 74 were children, and 283 were adults aged 19 or over. Age was shown to be an independent prognostic factor predictive of more favorable outcomes across measures including initial response to therapy, likelihood of relapse, and overall survival at follow-up. The distribution of affected organs at diagnosis was similar across children and adults and type of clinical presentation did not disproportionately influence 1 age group's prognosis over the other. Acute lymphoblastic leukemia-type chemotherapy regimens were shown to be superior to other chemotherapy regimens (acute myeloid leukemia, lymphoma, acute lymphoblastic leukemia/lymphoma, other, or none) in inducing complete remission. Allogeneic stem cell transplantation was shown to increase mean survival time. Future research may be directed toward elucidating the further morphologic, cytogenetic, and cytochemical differences between younger and older BPDCN patients.
母细胞性浆细胞样树突状细胞肿瘤（BPDCN）是一种罕见的侵袭性血液系统恶性肿瘤，其特征是常累及皮肤，最常影响老年患者。已知BPDCN预后较差。我们的目的是评估在结合临床表现、性别和治疗方案进行评估时，年龄是否会独立影响预后和疾病转归。我们进行了一项系统评价，以识别BPDCN病例，并将儿童BPDCN病例与成人病例进行比较。共识别出125篇详细描述356例病例的出版物。包括我们机构的1例儿科病例，74例为儿童，283例为19岁及以上的成年人。结果显示，年龄是一个独立的预后因素，在包括对治疗的初始反应、复发可能性和随访总生存等指标方面，均预示着更有利的结果。儿童和成人在诊断时受累器官的分布相似，临床表现类型对一个年龄组预后的影响并不比另一个年龄组大。在诱导完全缓解方面，急性淋巴细胞白血病型化疗方案优于其他化疗方案（急性髓系白血病、淋巴瘤、急性淋巴细胞白血病/淋巴瘤、其他或无化疗方案）。异基因干细胞移植可延长平均生存时间。未来的研究可致力于阐明年轻和老年BPDCN患者在形态学、细胞遗传学和细胞化学方面的进一步差异。
