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A prospective study of 32 patients with primary upper small intestinal lymphoma in our region revealed 10 cases of alpha heavy-chain disease. Patients were mostly in the second and third decades of life and males predominated. Weight loss, diarrhea, and abdominal pain were the most common complaints and clubbing the most frequent physical findings. Laboratory tests revealed a malabsorption pattern on intestinal x-rays, and malabsorption of xylose, fat, and vitamin B12 was frequently noted. Dense plasmacytic infiltrate of the lamina propria of small bowel was the most frequent pathologic finding while true neoplasm of the lymphoid system (ie, immunoblastic sarcoma) was encountered in 20% of the cases.

About one hundred cases of alpha-chain disease have been reported to date from various Mediterranean and Middle-Eastern countries. We report eleven Iranian patients with primary lymphoma of the duodenum and upper small intestine and positive alpha-chain protein in their sera. The clinical, laboratory and pathological findings in these patients are discussed. The initial favourable response of some of these patients to antibiotic treatment is noted. It is concluded that the optimal treatment of alpha-chain disease is not yet well established, and the clinical improvement might not coincide with histological and immunological remission.