Sinus histiocytosis with massive lymphadenopathy: remission with chemotherapy

A case of sinus histiocytosis with massive lymphadenopathy (SHML) is reported, in which the patient had severe life-threatening complications resulting from SHML. The disease process was extensive involving the respiratory tract, skeleton, integument, orbit and eyelid, uveal tract, and the lymphatic system. Treatment with cytotoxic agents (chlorambucil, prednisone) resulted in resolution of the airway obstruction and was effective in managing these severe complications with minimal observed toxicity.