Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition A rare non-Langerhans cell histiocytosis characterized by infiltration of lymph nodes or extranodal tissues by non-malignant histiocytes displaying emperipolesis, a non-destructive phagocytosis of lymphocytes or erythrocytes. Most typical presentation is as a massive cervical lymphadenopathy in adolescents and young adults. Most frequent sites of extranodal disease are skin, soft tissue, bones, paranasal sinuses, orbit, salivary glands, and central nervous system. Symptoms are related to mass effect in the affected organs. Disease data Classification Disease Synonyms Destombes-Rosaï-Dorfman disease Choroba Destombesa, Rosaï i Dorfmana choroba Rosaï, Dorfmana i Destombesa Histiocytoza zatokowa z masywną limfadenopatią SHML Rosaï-Dorfman-Destombes disease SHML Sinus histiocytosis with massive lymphadenopathy ORPHA code 158014 OMIM code - ICD10 code D76.3 ICD11 code EK92 *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl