Rosai Dorfman’s Disease (RDD) Destombes also called sinus histiocytosis with massive lymphadenopathy is a lymphoproliferative pathology usually benign, of unknown etiology, and of low incidence. It is a rare, but wel...Rosai Dorfman’s Disease (RDD) Destombes also called sinus histiocytosis with massive lymphadenopathy is a lymphoproliferative pathology usually benign, of unknown etiology, and of low incidence. It is a rare, but well-defined clinicopathological entity, which predominantly affects children and adolescents without distinction of gender or race. It manifests itself generally by bulky cervical lymphadenopathies, most often bilateral, painless, of benign but persistent evolution and a long-term fever associated with a non-specific biological inflammatory syndrome. Extra lymph node locations are described in association with lymph node involvement or isolation. The definitive diagnosis of Rosai Dorfman’s disease is histological, characterized by histiocytic infiltration with emperipolesis essentially of lymphocytes. The etiopathogenesis of the condition remains unclear, based on infectious and immunological hypotheses without clear evidence. The management is not well codified, it combines depending on the case, surgery;corticosteroids;antimetabolites and interferon alpha. Studies devoted to this condition in tropical Africa are rare. We report the clinical and progressive features of 5 observations of Rosai Dorfman’s disease.展开更多
文摘Rosai Dorfman’s Disease (RDD) Destombes also called sinus histiocytosis with massive lymphadenopathy is a lymphoproliferative pathology usually benign, of unknown etiology, and of low incidence. It is a rare, but well-defined clinicopathological entity, which predominantly affects children and adolescents without distinction of gender or race. It manifests itself generally by bulky cervical lymphadenopathies, most often bilateral, painless, of benign but persistent evolution and a long-term fever associated with a non-specific biological inflammatory syndrome. Extra lymph node locations are described in association with lymph node involvement or isolation. The definitive diagnosis of Rosai Dorfman’s disease is histological, characterized by histiocytic infiltration with emperipolesis essentially of lymphocytes. The etiopathogenesis of the condition remains unclear, based on infectious and immunological hypotheses without clear evidence. The management is not well codified, it combines depending on the case, surgery;corticosteroids;antimetabolites and interferon alpha. Studies devoted to this condition in tropical Africa are rare. We report the clinical and progressive features of 5 observations of Rosai Dorfman’s disease.
