Langerhans Cell Histiocytosis In Bone: A Case Report
Turkish Journal of Internal Medicine(2021)
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.
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Key words
langerhans cell histiocytosis,histiocytosis in bone,histiocytosis
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