Immunoproliferative small intestinal disease (IPSID) is a subtype of lymphoma of mucosa-associated lymphoid tissue. Notable for a high production of alpha-heavy chains, it is designated alpha-heavy-chain disease. IPSID is a debilitating disease that has a predilection for impoverished populations of developing countries. It has been documented primarily in subjects of Middle Eastern countries and thus was previously referred to as Mediterranean lymphoma. We report the case of a 42-year-old man from Senegal who presented with chronic diarrhea, dehydration, and weight loss. The endoscopic, pathologic, and serologic findings before, during, and after treatment with fludarabine phosphate are presented. We review the literature concerning current concepts on the etiology, pathogenesis, and management of IPSID.
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Treatment-Resistant Immunoproliferative Small Intestinal Disease (IPSID) Leading to Lymphoma.
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Department of Gastroenterology and Hepatology, Rutgers New Jersey Medical School, Newark, USA.
Article Synopsis
- Immunoproliferative small intestinal disease (IPSID) is a type of MALT lymphoma, mainly affecting the proximal small intestine and linked to chronic infection, causing symptoms like malabsorption and weight loss.
- A case study of a 50-year-old Nigerian woman highlights the diagnostic challenges of IPSID, as her symptoms were initially misattributed to other conditions despite multiple antibiotic treatments.
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Falsely elevated anti-tissue transglutaminase antibodies in patients with immunoproliferative small intestinal diseases: A case series.
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October 2023
Department of Gastroenterology and Human Nutrition, All India Institute of Medical Sciences, Ansari Nagar, 110 029, New Delhi, India.
Immunoproliferative small intestinal disease (IPSID) is an uncommon disease of the small intestine. There is a similarity in the clinical presentations of enteropathic diseases, including celiac disease, tropical sprue, IPSID, and Whipple's disease. A differentiation between them is based on the use of a highly specific serological test for celiac disease and specific histological characteristics.
View Article and Find Full Text PDFThe Evolution of Our Understanding of Immunoproliferative Small Intestinal Disease (IPSID) over Time.
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Section of Hematology and Bone Marrow Transplant, King Faisal Specialist Hospital and Research Center, Riyadh 11564, Saudi Arabia.
Immunoproliferative small intestinal disease (IPSID) is an uncommon disease with a higher prevalence in the developing world. IPSID diagnosis relies mainly on a tissue biopsy and a high index of suspicion. Treatment options are variable; however, they mainly include anthracycline-based chemotherapy with or without antibiotics in advanced stages.
View Article and Find Full Text PDFImmunoproliferative Small Intestinal Disease Diagnosed by Double-balloon Endoscopy with Biopsy Sampling.
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Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Japan.
We herein report an 80-year-old man diagnosed with immunoproliferative small intestine disease (IPSID) via small bowel endoscopy with a biopsy. He developed persistent diarrhea and subsequently presented with hypoproteinemia and moderate anemia. Transanal double-balloon endoscopy showed prominent villous edema in the middle and lower ileum, while a histological examination showed high lymphocyte/plasma cell infiltration in the mucosal layer.
View Article and Find Full Text PDFMucosa-Associated Lymphoid Tissue (MALT) Lymphoma in the Gastrointestinal Tract in the Modern Era.
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January 2022
Department of Gastroenterology, International University of Health and Welfare, Fukuoka 814-0001, Japan.
Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) typically arises from sites such as the stomach, where there is no organized lymphoid tissue. Close associations between and gastric MALT lymphoma or and immunoproliferative small intestinal disease (IPSID) have been established. A subset of tumors is associated with chromosomal rearrangement and/or genetic alterations.
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