Clinical and pathologic subtypes of primary intestinal lymphoma. Experience with 132 patients over a 14-year period

Primary lymphomas of the small intestine (PIL) show definite racial and geographic distribution. Clinical and laboratory data on 132 patients with PIL treated and followed up over a 14-year period in Baghdad, Iraq, are presented. Based on the pattern of involvement of the bowel and the histopathologic appearance of the tumor, the patients can be divided into three main groups. Ninety-seven patients had diffuse lymphoplasmacytic infiltration affecting large segments of the upper bowel. Abdominal pain, anorexia, weight loss, diarrhea, malabsorption, and clubbing of the fingers are the most common clinical findings. The barium appearance of the small intestine and peroral jejunal biopsy specimens are abnormal in nearly all cases. This clinicopathologic entity has been referred to in the literature as Mediterranean lymphoma (ML). Ten of 34 patients tested had free alpha-heavy chain in the serum. Twenty-three cases had other "Western" variants of lymphoma (18 lymphocytic and 5 plasmacytic). The lesions were localized, occurring most frequently in the lower ileum or ileocecal area. The most common presentation was intestinal obstruction. Twelve children had Burkitt's lymphoma (BL), presenting most commonly with abdominal masses and/or intestinal obstruction. These patients responded poorly to cyclophosphamide, and the disease disseminated early and extensively. None of the patients with the localized lymphomas or BL had free alpha chain in the serum.