Rosai Dorfman Disease: A Case Report

Abstract

Rosai-Dorfman disease, also defined as sinus histiocytosis with massive lymphadenopathy (SHML), is represented by a distorted lymph node architecture with marked dilation of lymphatic sinuses occupied by numerous lymphocytes, as well as histiocytes with vesicular nuclei and abundant clear cytoplasm with phagocytized lymphocytes or plasma cells, also known as 'emperipolesis.' This disease of unknown cause develops with a favourable prognosis only if and when it is diagnosed and treated early. A dismal prognosis is exacerbated by a late diagnosis and widespread lymph node involvement. A 29-year-old Indian woman with a 4-month history of a painful unilateral cervical tumour and low-grade fever was diagnosed with Rosai-Dorfman disease.  Fine needle aspiration (FNA) biopsy of the cervical lymph node was used to make the final diagnosis. Finally, due to the specific cytological features of SHML, FNA biopsy may be sufficient to make the diagnosis in the majority of patients, eliminating potentially unnecessary invasive procedures. The disease can be diagnosed on the histopathologically and immunohistochemically.