title: Alpha-heavy chain disease, Mediterranean lymphoma, and immunoproliferative small intestinal disease: a review of clinicopathological features, pathogenesis, and differential diagnosis.
abstract: 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.
authors.affiliation: Department of Internal Medicine, Baylor University Medical Center, Dallas, Texas 75246, USA.
authors.forename: K D
authors.initials: KD
authors.lastname: Fine
authors.forename: M J
authors.initials: MJ
authors.lastname: Stone
mesh_terms: D003937:Diagnosis, Differential; D006801:Humans; D007161:Immunoproliferative Small Intestinal Disease / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D007421:Intestine, Small / Q000473:pathology; D009626:Terminology as Topic