Middle Eastern intestinal lymphoma: report of a case and review of the literature.

A 20-year-old Persian man with Middle Eastern lymphoma is described, and 84 additional cases from the literature are reviewed. Basically, the disease is a malignant lymphoma which involves the upper small intestine (duodenum and proximal jejunum). It is associated with clubbing of the fingers, abdominal pain, weight loss, diarrhea, vomiting, and malabsorption, and frequently occurs in a younger age group than "Western Hemisphere" intestinal lymphoma. Some patients also have alpha heavy chain disease. The sex ratio is equal, and the disease occurs only in Middle Eastern and North African Moslems and Jews. Upper gastrointestinal radiographs are frequently diagnostic, and per oral small intestinal biopsy is nearly always diagnostic. Pathologically, the following features are characteristic for Middle Eastern lymphoma: partial or total villous atrophy with only mildly abnormal surface epithelium, sparsity of crypts, lymphatic dilatation, and infiltration of lamina propria by pleomorphic mononuclear cells which pepetrate the muscularis mucosa. The etiology and pathogenesis of this disease are unknown, but several hypotheses are discussed. Treatment by a variety of modalities is far from satisfactory, and the prognosis is much poorer than that observed in patients with the "Western" form of intestinal lymphoma. Other differences between Middle Eastern lymphoma and Western lymphoma are described in detail,