Alpha-heavy Chain Disease, Mediterranean Lymphoma, and Immunoproliferative Small Intestinal Disease: a Review of Clinicopathological Features, Pathogenesis, and Differential Diagnosis

There are a number of clinical syndromes associated with chronic diarrhea, malabsorption, and lymphoplasmacytic proliferation of the small intestine. In Middle-Eastern and Mediterranean countries immunoproliferative small intestinal disease is endemic, whereas in other parts of the world (including Northwestern Europe and North America) celiac sprue, and other sprue-like syndromes refractory to dietary gluten withdrawal, predominate. All of these syndromes appear to involve chronic stimulation of intestinal mucosa-associated lymphoid tissue and are associated with a heightened risk of malignant transformation. The clinicopathological features of these diseases, and distinction of the Middle Eastern syndromes from those more common in the Western hemisphere, have been reviewed.