Monosystem Multifocal Langerhans Cell Histiocytosis (Multifocal Eosinophilic Granulomas of the Bone) in a 36-Year Old Patient: Case Report, Therapeutic Doubts and Review of Literature

Siniša Roginić, Damir Buković, Ksenija Pešek, Dubravko Tršinski

Abstract


Our aim is to provide review of available studies on Langerhans cell histiocytosis (LCH) and discuss treatment for polyostotic monosystem form of disease based on our clinical experience. LCH is an enigmatic disease with insufficiently understood etiology, pathophysiology, and variety of clinical presentations ranging from solitary eosinophilic granuloma to severe multisystem disease. It is marked by formation of granuloma in practically any organ. We present rare case of multifocal bone disease in 36-year old patient without visceral involvement. Treatment protocols for adult LCH patients, especially for uncommon form in our case have not yet been defined. Our therapeutical trial with corticosteroids showed limited success with numerous side-effects. We conclude that LCH treatment can commence only after diagnosis and staging of the disease. Other factors like patient’s age, comorbidity, general condition, severity of symptoms and contraindications for therapy modalities should also be considered. In our experience expectative approach has better clinical outcome than immunosuppressive therapy in patients suffering from polyostotic multifocal form LCH with mild symptoms.


Keywords*


Langerhans cell histiocytosis, eosinophilic granuloma, corticosteroids, polyostotic, treatment

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