Histiocitose das células de langerhans: relato de caso

Abstract

Langerhans cells are dendritic cells seen above the basal layer of squamous epithelia. They are cells specialized in the capture of antigens and in the induction of T lymphocyte responses. Langerhans cell histiocytosis is a rare disease and of unknown etiology. It is believed that its nature is a neoplastic of inflammatory origin. The clinicopathological aspect is traditionally divided into: class 1, Langerhans cell histiocytosis, formerly known as eosinophilic granuloma. Class 2, which includes non-malignant histiocytosis lesions. Class 3 includes malignant lesions of histiocytosis. Due to the variety of clinical manifestations, there are several forms of differential diagnosis, but the lack of knowledge regarding the etiopathogenesis hinders a more direct approach. In this work we bring the case of a patient diagnosed with Langerhans cell histiocytosis at 6 years old. A series of computed tomography scans of the skull were performed in which the presence of lytic lesions in the frontal and mastoid bones was found and identified numerous enlarged lymph nodes with central necrosis. During treatment, the patient presented recurrences and development of diabetes insipidus. After more than 10 years of treatment involving surgical procedures, chemotherapy and radiotherapy, the patient is cured and undergoes periodic follow-ups. Therefore, the removal of the lesion surgically, radiotherapy and chemotherapy using vinblastine together with the use of corticosteroids are indicated in the treatment of the disease.