Vnitr̆ní lékar̆ství
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Vnitr̆ní lékar̆ství · Nov 2008
Case Reports[Langerhans cell histiocytosis in adult patients--a disease with many faces. Experience of a centre and an overview of the disease symptoms].
Over a period of 18 years, 17 patients with proven Langerhans cell histiocytosis (LCH) were treated at the Haematological Clinic in Brno. In 13 of them, the disease was diagnosed at adult age, and 4 patients were referred to the centre with LCH diagnosed at early child age. One of these 4 patients suffered from repeated recurrences of the disease at adult age and was diagnosed with progressive neurodegenerative damage of the CNS at the age of 25 which in its terminal phase resulted in the patient's immobility, loss of sphincter control, incapacity to communicate and death at the age of 32. ⋯ Therefore each new osteolytic lesion should be submitted for histological exam, as well as each pathologic formation, because diagnosing the disease without a microscopic and immunohistochemical exams is not possible. In the case of occurrence of diabetes insipidus at adult age, LCH should be considered as one of the possible underlying diseases. LCH pulmonary involvement should be considered in patients with an interstitial pulmonary process and the examinations should be focused accordingly (thoracoscopy with sampling for histological exams or bronchoalveolar lavage) plus the indispensable immunohistochemical examination.