A Rare case of rosai-dorfman disease in an adult male associated with auto-immune hemolytic anemia

Abstract

Rosai-Dorfman disease (RDD) is a rare benign histiocytic proliferative disorder predominantly of the lymph nodes, which mostly occurs in children and young adults typically presenting with lymphadenopathy. Our case is of a 63 year-old African-American male who presented with subjective fever, weight loss, bilateral axillary and inguinal lymphadenopathy as well as auto-immune hemolytic anemia. The histological analysis showed emperipolesis and histiocytes that were positive for S-100 and CD-68 consistent with RDD. After steroid treatment and splenectomy, patient's symptoms and hemolytic anemia had resolved. Our case is the first case of RDD reported to be associated with auto-immune hemolytic anemia in an adult.