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多灶性朗格汉斯细胞组织细胞增多症：一名青春期女性嗜酸性肉芽肿伴颅内扩展、巨大淋巴结病及爱泼斯坦-巴尔病毒感染的独特表现

Multifocal Langerhans Cell Histiocytosis: A Unique Presentation of Eosinophilic Granuloma with Intracranial Extension, Massive Lymphadenopathy, and Epstein-Barr Viral Infection in an Adolescent Female.

作者信息

Foster Kent Wade, Robinson Shamika Kayo, Tung-Hahn Eleanor, Gibbons George, Cruse Carl Wayne, Mudaliar Kumaran, Tung Rebecca, Kim Wendy

机构信息

Department of Biology, Brigham Young University, Provo, Utah, USA.

Division of Dermatology, Loyola University Chicago Stritch School of Medicine, Maywood, Illinois, USA.

出版信息

Case Rep Dermatol. 2021 Jan 25;13(1):28-35. doi: 10.1159/000511488. eCollection 2021 Jan-Apr.

DOI:10.1159/000511488
PMID:33613231
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7879306/
Abstract

We report the case of an otherwise healthy 17-year-old female who presented for surgical removal of an enlarging "atypical cyst" on her scalp. During subtotal excision, only friable serosanguinous translucent ribbons of tissue were found. A histopathologic diagnosis of Langerhans cell histiocytosis (LCH) was rendered and imaging studies revealed extradural invasion of the tumor. Within weeks, the patient also developed progressive lymphadenopathy with grossly elevated levels of Epstein-Barr virus viral capsid antigen antibody levels. This report aims to highlight a unique presentation of LCH with discussion of workup, management, and avoidance of potential surgical complications.

摘要

我们报告了一例病例，患者为一名17岁的健康女性，因头皮上一个不断增大的“非典型囊肿”前来接受手术切除。在次全切除过程中，仅发现了易碎的浆液血性半透明组织条带。病理组织学诊断为朗格汉斯细胞组织细胞增生症（LCH），影像学研究显示肿瘤硬膜外侵犯。数周内，患者还出现了进行性淋巴结病，爱泼斯坦-巴尔病毒病毒衣壳抗原抗体水平显著升高。本报告旨在强调LCH的一种独特表现，并讨论其检查、管理以及避免潜在手术并发症的问题。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b8de/7879306/f6cf20dca6eb/cde-0013-0028-g07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b8de/7879306/c7b61f617e44/cde-0013-0028-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b8de/7879306/5406e280be42/cde-0013-0028-g02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b8de/7879306/8b19dd198d58/cde-0013-0028-g03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b8de/7879306/5eb64c6f2010/cde-0013-0028-g04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b8de/7879306/fde220cc148b/cde-0013-0028-g05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b8de/7879306/4ad97deb1482/cde-0013-0028-g06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b8de/7879306/f6cf20dca6eb/cde-0013-0028-g07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b8de/7879306/c7b61f617e44/cde-0013-0028-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b8de/7879306/5406e280be42/cde-0013-0028-g02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b8de/7879306/8b19dd198d58/cde-0013-0028-g03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b8de/7879306/5eb64c6f2010/cde-0013-0028-g04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b8de/7879306/fde220cc148b/cde-0013-0028-g05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b8de/7879306/4ad97deb1482/cde-0013-0028-g06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b8de/7879306/f6cf20dca6eb/cde-0013-0028-g07.jpg

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