RID3846 Langerhans cell histiocytosis

synonym Langerhanszell-Histiozytose histiocytosis X

definition Group of disorders characterized by monoclonal proliferation of CD1+ histiocytes. The demonstration of the langerhans cells birbeck granules is the gold standard of the phenotype. eosinophilic granuloma makes up 60-80% of all the cases. [MeSH]