alpha-heavy chain disease, Mediterranean lymphoma, and immunoproliferativesmall 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 int estinal disease is endemic, whereas in other parts of the world (including Northwestern Europe and North America) celiac sprue, and other sprue-like s yndromes refractory to dietary gluten withdrawal, predominate. All of these syndromes appear to involve chronic stimulation of intestinal mucosa-assoc iated lymphoid tissue and are associated with a heightened risk of malignan t transformation. The clinicopathological features of these diseases, and d istinction of the Middle Eastern syndromes from those more common in the We stern hemisphere, have been reviewed. (C) 1999 by Am. Cell. of Gastroentero logy.