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Case Reports in Hematology
Volume 2015, Article ID 201536, 5 pages
http://dx.doi.org/10.1155/2015/201536
Case Report

Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation

1Division of Haematology and Oncology, McGill University Health Centre (MUHC), Montreal General Hospital, 1650 Cedar Avenue, Montreal, QC, Canada H3G 1A4
2Department of Medicine, Division of Internal, McGill University Health Centre (MUHC), Montreal General Hospital, 1650 Cedar Avenue, Montreal, QC, Canada H3G 1A4
3Department of Medicine, Division of Respirology, McGill University Health Centre (MUHC), Montreal General Hospital, 1650 Cedar Avenue, Montreal, QC, Canada H3G 1A4

Received 1 December 2014; Revised 22 January 2015; Accepted 25 January 2015

Academic Editor: Simon D. Wagner

Copyright © 2015 B. Routy et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm characterized by the proliferation and dissemination of histiocytes. These in turn may cause symptoms ranging from isolated, infiltrative lesions to severe multisystem disease. Pulmonary Langerhans cell histiocytosis (PLCH) presents as a localized polyclonal proliferation of Langerhans cells in the lungs causing bilateral cysts and fibrosis. In adults, this rare condition is considered a reactive process associated with cigarette smoking. Recently, clonal proliferation has been reported with the presence of BRAF V600E oncogenic mutation in a subset of PLCH patients. Spontaneous resolution was described; however, based on case series, smoking cessation remains the most effective way to achieve complete remission and prevent long term complications related to tobacco. Herein, we report the case of an adult woman with biopsy-proven PLCH presenting with thoracic (T8) vertebral bone destruction. Both the lung and the bone diseases regressed following smoking cessation, representing a rare case of synchronous disseminated PCLH with bone localization. This observation underscores the contribution of cigarette smoking as a systemic trigger of both pulmonary and extrapulmonary bone lesions. A review of similar cases in the literature is also presented.