Multisystem involvement Langerhans cell histiocytosis in an adult: A case report.

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.

• Authors: Bei-Bei Wang, Jun-Ru Ye, Yun-Lei Li, Yi Jin, Zhong-Wei Chen, Jian-Min Li, Yu-Ping Li
• Journal: World journal of clinical cases (World J Clin Cases) Vol. 8 Issue 20 Pg. 4966-4974 (Oct 26 2020) ISSN: 2307-8960 [Print] United States
• PMID: 33195668 (Publication Type: Case Reports)
• Copyright: ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.