摘要
目的:提高对儿童母细胞性浆细胞样树突细胞肿瘤(BPDCN)的临床及病理特征、诊断和治疗的认识。方法:分析我院1例4岁男孩患BPDCN的临床资料,并进行文献复习。结果:儿童BPDCN罕见,该例BPDCN患儿以皮肤和睾丸受累为主,未累及骨髓。予以ALL-CCLG-2008中危方案治疗,维持期增加培门冬酰胺酶治疗,效果显著,随访至今已无病生存5年半。结论:儿童BPDCN的化疗预后明显好于成人。是否需要高危急性淋巴细胞白血病方案化疗或造血干细胞移植,还需进一步临床病例积累。
Objective:To improve the understanding of the clinical and pathological features,diagnosis and treatment of blastic plasmacytoid dendritic cell neoplasm(BPDCN).Method:Clinical data of a 4-year-old boy with BPDCN in our hospital was analyzed and the related literatures were reviewed.Result:Pediatric BPDCN was very rare.Skin and testis were mainly involved in this case.The child was treated with ALL-CCLG-2008 intermediaterisk regimen and had been in complete remission for 5 year and a half.Conclusion:The prognosis of children with BPDCN is significantly better than that of adults.We should gather more clinical data of pediatric BPDCN to determinate whether high-risk acute lymphoblastic leukemia regimen or hematopoietic stem cell transplantation is necessary.
作者
丁丽丽
刘爱国
胡群
张柳清
张艾
王雅琴
余文
王松咪
陈萍
聂第敏
DING Lili;LIU Aiguo;HU Qun;ZHANG Liuqing;ZHANG Ai;WANG Yaqin;YU Wen;WANG Songmi;CHEN Ping;NIE Dimin(Department of Hematology,Tongji Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan,430030,China)
出处
《临床血液学杂志》
CAS
2019年第1期44-47,共4页
Journal of Clinical Hematology
关键词
母细胞性浆细胞样树突细胞肿瘤
儿童
治疗
预后
blastic plasmacytoid dendritic cell neoplasm
children
diagnosis
prognosis
