Primary Intestinal Lymphoma: Clinical Manifestations and Possible Effect of Environmental Factors

Abstract

Lymphoma of the small intestine can occur as a late secondary manifestation of disseminated lymphoma, or far less commonly, as a primary lesion originating in the small intestine or mesenteric lymph nodes [1–6]. The latter disease can be further subdivided into two categories: a) Solitary lymphoma that occurs most commonly in the terminal ileum and is encountered more frequently in young children, and b) Diffuse or multifocal small intestinal lymphoma, occurring most commonly in proximal small intestine encountered primarily in the older age groups. In the latter entity malabsorption has been described [3–5]. The syndrome of malabsorption in small intestine lymphoma as described by Edelman et al. (1966), occurs only rarely in the more developed countries of the Western world [7]. These authors, reviewing the medical literature of the West, were able to find only 48 cases, of which they regarded 32 as well established and 16 as probable. On the other hand, the syndrome of malabsorption in small intestinal lymphoma is not rare among certain populations whose common denominator appears to be that they are under-privileged in terms of nutrition, hygiene and medical care [7–11]. In Israel the syndrome is relatively prevalent among Arabs and among first and second generations of Jewish immigrants from mid-eastern and North Africa countries and is virtually non exsistent among Jews of European origin [7–10]. This syndrome was also described in other Mediterranean populations as, North Africa, Italy, Spain as well as in Iran, Mexico, South America and in the African-Cape-Coloured people [11–13]. The evidence thus far would seem to suggest that predisposition to primary small intestinal lymphoma is environmentally determined. One could also speculate about the unique vulnerability of certain populations due to genetic factors or a combination of environmental and genetic influences. In this work we summarized the present day knowledge on this entity and analysed the possible effects of environmental factors on its development. The possibility of abnormalities in the lymphoreticular system and its possible effect on the disease will also be discussed.