A case of sinus histiocytosis with generalized lymphadenopathy--Rosai-Dorfman disease--in a 54 year old male is presented. The diagnosis was made by biopsy of the lymph nodes and histopathological examination of the affected organ. Treatment with prednisone and cyclophosphamide induced clinical improvement. The diseases clinical advanced stage and organ localization and the immunosuppressive therapy resulted in a opportunistic infection--pneumocystidosis and death of the patient within half a year after starting of therapy.
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