Vol. 1, Issue 1, Part A (2019)

Rosai-Dorfam disease (RDD) also known as sinus histiocytosis with massive lymphadenopathy (SHML) is a rare self-limited and benign disease. The clinical features in the classical form include slowly enlarging painless massive lymphadenopathy, fever, leukocytosis, anemia, hypergammaglobulinemia and elevated erythrocyte sedimentation rate (ESR). We report a case of 53 year old female who presented with generalized lymphadenopathy and clinical suspicion of lymphoma. The diagnosis of RDD was made primarily by the fine needle aspiration cytology (FNAC) findings. Emperipolesis was the key feature for diagnosis. The diagnosis was confirmed by the histopathological examination too. This case report, therefore establishes the usefulness of FNAC as a simple diagnostic tool for RDD, thereby avoiding more invasive approaches and unnecessary investigations. It also aims at creating an awareness about this rare entity among the clinicians, general practitioners and also pathologists especially in patients presenting with generalized massive lymphadenopathy. Finally, our case report adds on to the existing literature on cytologic diagnosis of RDD.