Alpha-chain disease and related small-intestinal lymphoma: a memorandum

Primary intestinal lymphomas are remarkably frequent in the Mediterranean region and South-West Asia. They are usually found in young persons from the lower socio-economic strata of the population. These conditions sometimes present a premalignant phase characterized by plasmacytic infiltration of the small intestine. It has been reported that early treatment of cases with antibiotics is followed by complete remission, suggesting that some environmental factors may be responsible for the disease. Some patients have an abnormal alpha-chain protein in their serum. This Memorandum reviews the present knowledge of the clinical, immunological, epidemiological, and therapeutic aspects of this condition.