Primary lymphomas of the small intestine: East‐west contrast

Hamid Al-Mondhiry

Research output: Contribution to journalReview article

33 Citations (Scopus)

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. Histologicaily, 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.

Original languageEnglish (US)
Pages (from-to)89-105
Number of pages17
JournalAmerican Journal of Hematology
Volume22
Issue number1
DOIs
StatePublished - Jan 1 1986

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Small Intestine
Lymphoma
Abdominal Pain
Immunoproliferative Small Intestinal Disease
Middle East
Lymphoma, Large B-Cell, Diffuse
Age Distribution
Celiac Disease
B-Cell Chronic Lymphocytic Leukemia
Duodenum
Developed Countries
Social Class
Non-Hodgkin's Lymphoma
Immunoglobulin A
Developing Countries
Fingers
Diarrhea
Neoplasms
Mucous Membrane
Serum

All Science Journal Classification (ASJC) codes

  • Hematology

Cite this

Al-Mondhiry, Hamid. / Primary lymphomas of the small intestine : East‐west contrast. In: American Journal of Hematology. 1986 ; Vol. 22, No. 1. pp. 89-105.
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Primary lymphomas of the small intestine : East‐west contrast. / Al-Mondhiry, Hamid.

In: American Journal of Hematology, Vol. 22, No. 1, 01.01.1986, p. 89-105.

Research output: Contribution to journalReview article

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AB - 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. Histologicaily, 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.

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