Fatal intestinal hemorrhage complicating ileal lymphoma after cyclosporine for unresponsive celiac disease

Unresponsive celiac disease may benefit from immunosuppressive therapy. Malignant small intestinal lymphoma is the most serious complication of celiac disease, also being noted as a complication of immunosuppressive therapy. The diagnosis of small intestinal lymphoma complicating celiac disease is notoriously difficult. Perforation is the most common complication of small intestinal lymphoma, frank hemorrhage being unusual. We report a case of massive, fatal hemorrhage from small intestinal lymphoma complicating unresponsive celiac disease treated with cyclosporine. The patient was presented with severe diarrhea and nutritional deterioration. Unresponsive celiac disease was diagnosed on the basis of clinical and histologic criteria with no response while on a gluten-free diet, corticotherapy and octreotide acetate injections. Cyclosporine therapy was advised. The patient had a remarkable clinical response. After 3 months from the start of the cyclosporine therapy, the patient returned with massive intestinal bleeding. The patient underwent emergency surgery diagnosing an enteropathy-associated lymphoma. We conclude that cyclosporine therapy for unresponsive celiac disease should be considered in select, severely ill patients only after a full-thickness biopsy of the small intestinal wall to disclose a latent super-imposed lymphoma, which course may be accelerated by immunosuppressive therapy.