Rosai-Dorfman Disease: Successful Long-term Results by Combination Chemotherapy with Prednisone, 6-Mercaptopurine, Methotrexate, and Vinblastine: A Case Report

Abstract

Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease (RDD), is a rare clinicopathologic entity of unknown etiology. The majority of patients do not require treatment, demonstrating spontaneous resolution or stable disease. However, in some cases the disorder runs a progressive course and/or threatens vital structures and functions, which dictates some form of intervention. It is not clear what constitutes the best approach for those cases. We report on a girl with an extensive and progressive form of the disease who was cured with combination chemotherapy, showing no evidence of recurrence after a follow-up of 5.5 years.