Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy can present with or without systemic disease. It is a benign histioproliferative disorder characterized by generalized lymphadenopathy, weakness, anemia, and rarely extranodal involvement. While RDD most commonly affects lymph nodes, extranodal involvement of multiple organs has been reported, including the central nervous system (CNS).
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