Základnou charakteristikou Langerhansovej histiocytózy je proliferácia klonov histiocytov. Ide o raritnú diagnózu s incidenciou 1-2 prípady na 1 milión obyvateľov dospelej populácie. Langerhansova histiocytóza je ochorenie detského veku. Literatúra opisuje len niekoľko prípadov tejto choroby s výskytom v gastrointestinálnom trakte. Prinášame kazuistiky dvoch pacientov s manifestáciou Langerhansovej histiocytózy v tenkom a hrubom čreve. Patogenéza ochorenia nie je známa. Diagnostika sa opiera o pozitivitu Birbeckových granúl v elektromikroskopickom obraze, avšak definitívnu diagnózu potvrdí až prítomnosť CD1a antigénu.

Langerhans cell histiocytosis is an idiopathic disorder characterized by the proliferation of Langerhans cells. Literature describes incidence 1-2 cases per 1 million in the adult population. Langerhans cell histiocytosis is a rare disease of the paediatric population. Involvement of the gastrointestinal tract is very rare in LCH, especially in adults, with only a few isolated case reports available in literature. We present two cases of this rare disorder with manifestation in the small intestine and colon. The pathogenesis of histiocytosis is unknown. From the histopathological viewpoint, the demonstration of Birbeck granules by electron microscopy, but expression of the CD1a antigen on lesional cells also provides the basis for a definitive diagnosis.

Lymphadenopathy with progresive weight loss