Rosai dorfman disease-A rare disease in India

Abstract

A 27 year old male presented with complaint of painless cervical lymphadenopathy which was progressively increasing in size and low grade fever on and off since one and a half year. Bilateral Cervical and axillary lymphnodes were palpable. The lymphnodes were firm, mobile and painless. Liver was palpable, non-tender and smooth in consistency. Hematological parameters and ESR in this case were in normal range. FNAC was suggestive of Hodgkin’s lymphoma but immunohistochemistry showed S-100 protein expression and lesion demonstrated emperipolesis. Hence the diagnosis of RDD was made. Patient was initially non-compliant to medical advise and presented after 5 years with similar complaints. He was managed using Rituximab and Chemotherapy with cyclophosphamide and Adriamycin. Also, oral prednisolone 100 mg was started. The patient responded very well to treatment over a period of two weeks and showed no signs of recurrence after 12 months follow up.