Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Oct 26, 2020; 8(20): 4966-4974
Published online Oct 26, 2020. doi: 10.12998/wjcc.v8.i20.4966
Multisystem involvement Langerhans cell histiocytosis in an adult: A case report
Bei-Bei Wang, Jun-Ru Ye, Yun-Lei Li, Yi Jin, Zhong-Wei Chen, Jian-Min Li, Yu-Ping Li
Bei-Bei Wang, Jun-Ru Ye, Yun-Lei Li, Yu-Ping Li, Department of Pulmonary and Critical Care Medicine, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou 325000, Zhejiang Province, China
Yi Jin, Jian-Min Li, Pathology Department, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou 325000, Zhejiang Province, China
Zhong-Wei Chen, Radiology Department, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou 325000, Zhejiang Province, China
Author contributions: Wang BB and Ye JR reviewed the literature and contributed to manuscript drafting; Li YL, Jin Y, Chen ZW and Li JM performed data curation, formal analysis; Li YP designed the research and performed supervision.
Informed consent statement: The patient involved in this case report provided written informed consent to the publication of case details and the accompanying images. And the written consent is available for review by the editor of this journal.
Conflict-of-interest statement: None.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Yu-Ping Li, MD, Professor, Department of Pulmonary and Critical Care Medicine, The First Affiliated Hospital of Wenzhou Medical University, South Baixiang, Ouhai District, Wenzhou 325000, Zhejiang Province, China. wzliyp@163.com
Received: May 5, 2020
Peer-review started: May 5, 2020
First decision: May 15, 2020
Revised: May 26, 2020
Accepted: August 26, 2020
Article in press: August 26, 2020
Published online: October 26, 2020
Processing time: 172 Days and 19.2 Hours
Abstract
BACKGROUND

Langerhans cell histiocytosis (LCH) is a rare condition wherein Langerhans cells proliferate abnormally, adversely impacting organs including lymph nodes, bones, skin, lungs, and pituitary gland. The LCH disease course varies widely among patients from a self-limiting condition to one that progresses rapidly and culminates in death. It is uncommon for multisystem LCH to be observed in adults. Herein we describe a woman suffering from multi-system LCH involvement.

CASE SUMMARY

A 37-year old Chinese woman was admitted to the hospital in June 2019 suffering from dyspnea that had progressed over the course of 5 years. Her medical history included: central diabetes insipidus (DI) that had been treated via radiotherapy, desmopressin acetate, and bromocriptine; bilateral pneumothorax with two surgeries having been performed to remove bullae; and autoimmune hepatitis that had been unsuccessfully treated using a combination of methylprednisolone and mycophenolate mofetil. A chest computed tomography (CT) scan revealed the presence of multiple pulmonary cysts of varying sizes. We re-analyzed right pulmonary bullae samples that had been removed in 2014, performed a systematic 18F-FDG PET/CT analysis, and convened a multidisciplinary medical team to diagnose and treat this patient. As a result, we were able to eventually diagnose this patient with LCH that was not associated with BRAF-V600E mutations.

CONCLUSION

We hope to emphasize the importance of systemic evaluation and of cooperation between multidisciplinary physicians with the goal of improving awareness and detection of this orphan disease.

Keywords: Langerhans cell histiocytosis; Lung; Pituitary gland; Liver; Case report

Core Tip: Herein we describe the case of a young woman suffering from Langerhans cell histiocytosis (LCH) that affected organs including the lungs, liver, rib, pituitary gland, bilateral parotid gland, and bilateral submandibular glands. There have been very few previous reports of LCH with parotid gland and submandibular gland involvement. LCH is under- or misdiagnosed as a consequence of its variable and nonspecific presentation. Such misdiagnosis was evident in the patient described herein, and there was an extended period of time between initial symptom presentation and LCH diagnosis. This is a typical case of multi-system involvement LCH, and through this report we hope to help raise awareness of early LCH.