Rosai-Dorfman Disease: A Case Report and Literature Review

Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare disorder that typically manifests as lymphadenopathy and systemic symptoms whose etiology remains poorly elucidated. The diagnosis is based on immunohistochemistry. Its treatment is poorly defined but the prognosis is usually favorable. Here we report a 14 year old boy who presented with massive bilateral cervical lymphadenopathy. Histopathological examination demonstrated lymphophagocytosis (emperipolesis) consistent with a diagnosis of Rosai-Dorfman disease. The clinical and histological aspects of the disease are discussed as a rare cause of lymphadenopathy. Key Words: Rosai-Dorfman disease, emperipolesis, case, Iran.