Langerhans cell histiocytosis (LCH) is a rare disease with the incidence in adult population about 1-2/1 000 000 person. Among children is the incidence a little higher. Twenty two patients with LCH were diagnosed and followed at the Department of Internal Medicine, Hematooncology, Brno. The disease was newly diagnosed in 17 adult patients, and 5 patients were referred to our department with LCH, recognised in children age. The diverse forms of manifestation of LCH in our patients are described and discussed in context with published date. The first manifestation of LCH in adult people could be: infiltration of infundibulum of hypophysis with the diabetes mellitus, later in the course of the disease, insufficient production of gonadotropin, TSH and somatostatin could be detected. Insufficient
somatostatin production was reason of failing in vitro fertilisation, this procedure was successful only after somatostatin substitution. In some patients the first manifestation is otitis externa, some years after this first symptom can occur destruction of procesus mastoideus. In some patients the first symptom of the disease are pathologic changes of the gums (similary like by parodontosis) with lost of the teeth. In some people cough and breathlessness are the first symptoms with micronodular and cystic changes on HRCT of lungs. Some patients have a bone pain caused by osteolytic bone destruction. The first symptoms of this disease are really divergent and therefore multidisciplinary approach is necessary for diagnosis and therapy of this disease. For the therapy of agresive multisystem LCH we have used 2-chlordeoxyadenosine. In all bud one patients long lasting remission were achieved with no toxicity IIIrd and IVth grade (WHO).