The association of Behçet's disease and non-Hodgkin's lymphoma is very rare. The first case of primary intestinal lymphoma with intestinal Behçet's disease is reported. A 37 year-old woman had been treated for Behçet's disease with colchicine for 2 years. In July 1997, she developed uveitis and was treated by monthly intravenous pulses of cyclophosphamide for 6 months, but uveitis persisted; so cyclophosphamide was replaced by cyclosporin 300 mg/day. One month later, she suffered from diarrhea. Colonoscopy showed ileocoecal ulcerations. Histological examination of surgical biopsy revealed B large cell type lympocytic malignant lymphoma and vasculitis lesions compatible with intestinal Behçet's disease. Cyclosporin was stopped and treatment with prednisone was instituted. The relationship between non-Hodgkin's lymphoma and immunosuppressive drugs in Behçet's disease is discussed.
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