• Ina Volis, Ido Livneh, Yaniv Zohar, and Ayelet Raz-Pasteur.
• Department of Internal Medicine "A", Rambam Health Care Campus, Haifa 3109601, Israel; The Rappaport Faculty of Medicine and Research Institute, Technion - Israel Institute of Technology, Haifa 3109602, Israel; Department of Cardiology, Rambam Health Care Campus, Haifa, Israel. Electronic address: ina.volis@gmail.com.
• Am. J. Med. Sci. 2022 Sep 1; 364 (3): 353358353-358.

AbstractLangerhans cell histiocytosis (LCH) is an uncommon clonal proliferation of myeloid progenitor cells, it is especially rare in adults. We present a case of multi-system LCH in a 53-year-old woman, the sole symptom of which was prolonged, non-resolving hip pain for 18 months prior to the diagnosis. Initial evaluation included imaging studies aimed at identifying a presumed local etiology. X-ray demonstrated non-specific arthritic changes on the left femur. Computed tomography (CT) and magnetic resonance imaging (MRI) scans identified a lytic lesion at the same location, warranting a systemic workup. After non-invasive investigations failed to reveal the underlying etiology, a biopsy was performed, revealing cores of Langerhans cells that stained positive for both CD1a and langerin. These findings verified the surprising, uncommon diagnosis of LCH. A comprehensive workup was conducted in order to determine the extent of the disease and its molecular nature - revealing a BRAFV600E-positive, high-risk, multi-system LCH with skeletal, lung and liver involvement.Copyright © 2022 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.

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