Primary lymphomas of the small intestine: East‐west contrast

Abstract

Primary small intestinal lymphoma (PSIL) represents a heterogenous group of disorders with variable clinical and pathologic features and a characteristic age, socioeconomic, and geographic distribution. In developed countries, PSIL usually occurs as a localized ileal tumor, shows a bimodal age distribution, and most frequently presents with abdominal pain and obstructive symptoms. Histologically, most of these tumors are diffuse histiocytic, lymphocytic, or undifferentiated lymphomas. Other variants of PSIL, collectively referred to as immunoproliferative small intestinal disease, occur most often among young patients of poor socioeconomic status in Third World countries, mostly in the Middle East and Mediterranean area. They are characterized by involvement of long loops of the upper small intestine and commonly present with abdominal pain, diarrhea, malabsorption, and clubbing of the fingers. A subgroup of these patients shows a serological abnormality with the appearance of part of the alpha heavy chain of IgA in the serum. Histologically, the lesion appears as a dense diffuse lymphoplasmacytic infiltrate of the mucosa of the upper jejenum or duodenum. A form of malignant lymphoma of true histiocytic origin complicates long-standing celiac disease. The contrasting clinical, epidemiological, histopathological, and immunological features of these variants of PSIL raise interesting questions about the pathogenesis of small bowel lymphoma.

Immunoproliferative small intestinal disease (IPSID): a model for mature B-cell neoplasms

“…Geographically, the majority of cases reported were from the Middle East, North and South Africa, and the Far East. [14][15][16][17][18] From 19 Clinically, intermittent diarrhea and colicky abdominal pain are the most frequent symptoms. [20][21][22] Other symptoms and signs are mainly related to malabsorption.…”

Digital clubbing in a patient with progressive mantle cell lymphoma

“…In the report by Dutta et al on 29 cases with DC, one patient had non-Hodgkin's lymphoma, but there were no further details [8]. No MCL was mentioned in these series [1,2,3,8].…”

“…Al-Mondhiry [2] reported clubbing of fingers and toes in 48-75% of patients with primary small intestinal lymphoma arising in the Middle East and the Mediterranean area. Mediterranean lymphoma is characterized by diffuse mucosal lymphoplasmacytic infiltration of the jejunum and duodenum, diarrhea, malabsorption, and alpha heavy-chain protein in 20-90% of patients [2].…”

“…Mediterranean lymphoma is characterized by diffuse mucosal lymphoplasmacytic infiltration of the jejunum and duodenum, diarrhea, malabsorption, and alpha heavy-chain protein in 20-90% of patients [2]. Among 132 patients with primary intestinal lymphomas reported by Al-Bahrani et al [1], 73% had classical diffuse Mediterranean lymphoma, 17% had localized non-Mediterranean lymphoma, and 9% had Burkitt's lymphoma.…”

Burkitt's lymphoma of stomach: A case report and review of literature

“…The most common site involved is the stomach (48-63%) followed by the small intestine (20-40%) and colon [2,4]. Contrary to this, in the middle east, the small intestine is the site in more than half of cases [5]. According to international working formulation (IWF) classification, diffuse large cell (DLC), intermediate or high grade or diffuse histiocytic (Rapport) is the most common histology seen [2].…”