Projevy Histocytózy z Langerhansových buněk v ORL oblasti

Title in English Head and neck manifestation of Langerhans´ cell histiocytosis
Authors

SMILEK Pavel PAŽOURKOVÁ Marta

Year of publication 2010
Type Article in Periodical
Magazine / Source Vnitřní lékařství
MU Faculty or unit

Faculty of Medicine

Citation
Field ORL, ophthalmology, stomatology
Keywords Langerhans cell histiocytosis and head and neck manifestation; mastoidectomy; surgery; chemotherapy and radiotherapy
Description Head and neck region used to be involved in almost 90 % of patients with Langerhans cell histiocytosis (LCH). The base of treatment in multisystem disease is in systemic chemotherapy and radiotherapy; surgery has only palliative character. In localized lesions of LCH there is recommended surgery and topical application of steroids. Cases: patient 1 has been followed up for 33 years, she was treated with Vinblastin (more than 126 time 10 mg i.v.), Prednison, radiotherapy (focal dose was more than 100 Gy) and mastoidectomy. Today there is diabetes insipidus, peripheral palsy of facial nerve, advanced destruction of the temporal and occipital bone, atlantooccipital junction and intracerebral manifestation in right temporal lobe. Patient 2 has been treated for 4 years with the temporal bone involvement and pulmonary disease with mastoidectomy, Leustatin and radiotherapy. He is now in full remission. Patient 3 has been treated for 3 years with pulmonary involvement of LCH and chronic inflammation of external auditory meatus (the diagnosis has not been established yet from the ear.

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