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HK J Paediatr (New Series)
Vol 24. No. 4,
2019
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HK J Paediatr (New Series) 2019;24:227-230
Case Report
A Child with Blastic Plasmacytoid Dentritic Cell Neoplasm Complicated with Perianal Necrotising Fasciitis
WYK Chan, RYY Leung, AKS Chiang, DKL Cheuk Department of Paediatrics and Adolescent Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Queen Mary Hospital, 102 Pokfulam Road, Pokfulam, Hong Kong SAR WYK Chan (陳祐祈) MBBS, MPH AKS Chiang (蔣國誠) MBChB, PhD DKL Cheuk (卓家良) MBBS, MPH Department of Pathology, Queen Mary Hospital, 102 Pokfulam Road, Pokfulam, Hong Kong SAR RYY Leung (梁毓恩) MBBS Correspondence to: Dr WYK Chan Email: wilsonykchan@graduate.hku.hk Received September 11, 2017
Abstract Blastic plasmacytoid dendritic cell neoplasm is an uncommon aggressive haemato-lymphoid neoplasm derived from type 2 dendritic cells which occur mainly in adults and elderly. It usually manifests as cutaneous lesions on face, scalp, trunk or limbs. In this report, we describe a 6-year old girl with unusual presentation of septic shock and perianal necrotising fasciitis, warranting urgent laparotomy, colectomy, perineal debridement and long term colostomy. She achieved complete remission with acute lymphoblastic leukaemia-based chemotherapy protocol. Keyword : Blastic plasmacytoid dendritic cell neoplasm; Necrotising fasciitis; Perianal Abstract in Chinese
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