Table of Contents

HK J Paediatr (New Series)
Vol 24. No. 4, 2019

HK J Paediatr (New Series) 2019;24:227-230

Case Report

A Child with Blastic Plasmacytoid Dentritic Cell Neoplasm Complicated with Perianal Necrotising Fasciitis
兒童幼稚漿細胞樣樹突細胞瘤併發肛週壞死性筋膜炎一例

WYK Chan, RYY Leung, AKS Chiang, DKL Cheuk


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.

幼稚漿細胞樣樹突細胞瘤是一種少見進行性造血淋巴樣腫瘤,源自於2型樹突細胞,主要見於成人和老年人。其臨床表現通常在面部、頭部、軀幹或四肢的皮膚出現病灶。在此,作者描述6歲女孩一例,具有不常見的臨床表現:膿毒性休克和肛週壞死性筋膜炎,需要接受緊急剖腹探查術、結腸切除術、會陰清創術、和長期結腸造口術。女童在接受急性淋巴細胞白血病方案的化療後,病情已完全得到緩解。

Keyword : Blastic plasmacytoid dendritic cell neoplasm; Necrotising fasciitis; Perianal

關鍵詞:幼稚漿細胞樣樹突細胞瘤、壞死性筋膜炎、肛週

 
 

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