Langerhans Cell Histiocytosis In Bone: A Case Report

Year 2021, Volume: 3 Issue: 4, 201 - 206, 29.10.2021

Orkide Kutlu , Gizem Meral Hasan Eruzun , Özlem Buluz Ayça Zeynep Kutlu Suat Erol Çelik

https://doi.org/10.46310/tjim.876279

Abstract

Langerhans cell histiocytosis (LCH) is a rare disease in which histiocytic infiltrations can be seen in bone, skin, lymph nodes, lungs, liver, spleen, bone marrow, central nervous system and endocrine glands. Pulmonary LCH has been closely associated with smoking while there is no data on genetic, viral or neoplastic etiology. In LCH with multiple system involvement, unifocal/multifocal infiltrations occur in two or more organs together with systemic symptoms such as weight loss and fever. In histology, Langerhans cells that do not contain phagocytic material in their cytoplasm, have a folded "coffee bean" appearance in their nucleus, express histiocyte markers CD1a, S100 and C207 and contain Birbeck granules under electron microscope. In treatment, if there is a risk of collapse in spinal or femoral bone lesions, surgery and radiotherapy can be applied for stabilization; if necessary, chemotherapy can be applied in multisystem disease. Here, we reported a LCH patient with a malignant shaped lytic lesion in the thoracic spine and adjacent bone.

Keywords

Langerhans Cell Histiocytosis, Histiocytosis In Bone, Histiocytosis

References

• De Young B, Egeler RM, Rollins BJ. Langerhanscellhistiocytosis. In: WHO Classification of Tumours of SoftTissueand Bone, 4th ed, Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F (Eds), International AgencyforResearch on Cancer, Lyons 2013. p.356
• Senechal B, Elain G, Jeziorski E, et al. Expansion of regulatory T cells in patientswithLangerhanscellhistiocytosis. PLoSMed 2007; 4:e253.
• Berres ML, Lim KP, Peters T, et al. BRAF-V600E expression in precursorversusdifferentiateddendriticcellsdefinesclinicallydistinct LCH risk groups. J ExpMed 2014; 211:669.
• Minkov M, Pötschger U, Grois N, et al. Bone marrowassessment in Langerhanscellhistiocytosis. Pediatr Blood Cancer 2007; 49:694.
• Götz G, Fichter J. Langerhans'-cellhistiocytosis in 58 adults. Eur J MedRes. 2004;9(11):510.
• Huang W, Yang X, Cao D, Xiao J, Yang M, Feng D, Huang Q, Wu Z, Zheng W, Jia L, WuS Eosinophilicgranuloma of spine in adults: a report of 30 cases and outcome.ActaNeurochir (Wien)2010;152(7):112.
• Phillips M, Allen C, Gerson P, McClain K. Comparison of FDG-PET scans to conventional radiography and bone scans in management of Langerhans cel lhistiocytosis. Pediatr Blood Cancer 2009; 52:97.
• Badalian‐Very G, Vergilio JA, Degar BA, et al. Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood. 2010;116:1919‐1923.
• Kobayashi M, Tojo A. Langerhans cell histiocytosis in adults: Advances in pathophysiology and treatment. Cancer Sci. 2018;109(12):3707-3713.
• Go H, Jeon YK, Huh J, et al. Frequent detection of BRAF(V600E) mutations in histiocytic and dendritic cell neoplasms. Histopathology. 2014;65(2):261‐272.
• Héritier S, Hélias‐Rodzewicz Z, Lapillonne H, et al. Circulating cell‐free BRAFV600E as a biomarker in children with Langerhans cell histiocytosis. Br J Haematol. 2017;178(3):457‐ 467.
• Allen CE, Ladisch S, McClain KL. How I treat Langerhans cell histiocytosis. Blood 2015; 126:26.
• Haroche J, Cohen‐Aubart F, Emile JF, et al. Dramatic efficacy of vemurafenib in both multisystemic and refractory Erdheim‐Chester disease and Langerhans cell histiocytosis harboring the BRAF V600E mutation. Blood. 2013;121:1495‐1500.
• Diamond EL, Subbiah V, Lockhart AC, et al. Histiocytosis: analysis of data from the histology‐independent, phase 2, open‐label VE‐BASKET Study. JAMA Oncol. 2018;4:384‐ 388.
• Papapanagiotou M, Griewank KG, Hillen U, et al. Trametinib‐induced remission of an MEK1‐mutated langerhans cell histiocytosis. JCO Precis Oncol. 2017;1:1‐5.