α-heavy chain disease, Mediterranean lymphoma, and immunoproliferative small intestinal disease A review of clinicopathological features, pathogenesis, and differential diagnosis

Abstract

There are a number of clinical syndromes associated with chronic diarrhea, malabsorption, and lymphoplasmacytic proliferation of the small intestine. In Middle-Eastern and Mediterranean countries immunoproliferative small intestinal disease is endemic, whereas in other parts of the world (including Northwestern Europe and North America) celiac sprue, and other sprue-like syndromes refractory to dietary gluten withdrawal, predominate. All of these syndromes appear to involve chronic stimulation of intestinal mucosa-associated lymphoid tissue and are associated with a heightened risk of malignant transformation. The clinicopathological features of these diseases, and distinction of the Middle Eastern syndromes from those more common in the Western hemisphere, have been reviewed.

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.…”

Immunoproliferative Small Intestinal Disease Associated with Overwhelming Polymicrobial Gastrointestinal Infection with Transformation to Diffuse Large B-cell Lymphoma

“…The majority of IPSID cases are seen in patients from the Middle East, North Africa, and Asia, although cases from Central and South America are described. 7 Adolescents and young adults (age range 10-35) are primarily affected with a male predominance of 2:1. 1 The most common predisposing factors are poor hygiene, and lack of adequate sanitation and clean drinking water.…”

“…3,11 Pathology shows extensive lymphoplasmacytic infiltration with blunting or absence of the villi and destruction of the crypts, and in later stages the infiltrate can be marked by atypical lymphoid cells. 1,7 In advanced disease, mesenteric, abdominal, and retroperitoneal lymph nodes can become involved, but involvement of other abdominal organs (liver, spleen) is uncommon. 7 The pathogenesis of IPSID is not fully elucidated, although is believed to result from chronic antigenic stimulation due to recurrent or chronic gastrointestinal infections.…”

“…1,7 In advanced disease, mesenteric, abdominal, and retroperitoneal lymph nodes can become involved, but involvement of other abdominal organs (liver, spleen) is uncommon. 7 The pathogenesis of IPSID is not fully elucidated, although is believed to result from chronic antigenic stimulation due to recurrent or chronic gastrointestinal infections. To date, no causative agent has been identified.…”

Severe Enteropathy Caused by α-Heavy Chain Disease Lacking Detectable M-proteins

“…The digestive type is a synonym for immunoproliferative small intestinal disease (IPSID) and primarily affects children and young adults (range, 10-35 years; mean, 25-30 years) of low socioeconomic status in developing countries (3). The clinical characteristics of the digestive type include weight loss, intermittent diarrhea, abdominal pain lasting for months to years, malabsorption and, in serious cases, intestinal obstruction, abdominal mass formation and ascites (4).…”