Multisystem involvement Langerhans cell histiocytosis in an adult: A case report
- 26 October 2020
- journal article
- research article
- Published by Baishideng Publishing Group Inc. in World Journal of Clinical Cases
- Vol. 8 (20), 4966-4974
- https://doi.org/10.12998/wjcc.v8.i20.4966
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 F-18-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
This publication has 25 references indexed in Scilit:
- Langerhans cell histiocytosis in adults: Advances in pathophysiology and treatmentCancer Science, 2018
- Langerhans-Cell HistiocytosisThe New England Journal of Medicine, 2018
- Role of 18F-FDG PET/CT in patients affected by Langerhans cell histiocytosisJapanese Journal of Radiology, 2017
- Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineagesBlood, 2016
- RecurrentNRASmutations in pulmonary Langerhans cell histiocytosisEuropean Respiratory Journal, 2016
- Recurrent BRAF mutations in Langerhans cell histiocytosisBlood, 2010
- Endocrine manifestations of Langerhans cell histiocytosis diagnosed in adultsPituitary, 2010
- Cell-Specific Gene Expression in Langerhans Cell Histiocytosis Lesions Reveals a Distinct Profile Compared with Epidermal Langerhans CellsThe Journal of Immunology, 2010
- Endocrine manifestations in Langerhans cell histiocytosisTrends in Endocrinology & Metabolism, 2007
- Langerhans cell histiocytosis in adultsReport from the International Registry of the Histiocyte SocietyEuropean Journal of Cancer, 2003