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WHO Classification of Tumours
Alpha heavy chain disease
Small intestine


Definition

Alpha heavy chain disease is s clonal disorder also known as immunoproliferative small intestinal disease (IPSID) or Mediterranean lymphoma and is characterised by the secretion of a defective alpha heavy chain. It predominantly affects young people in the Mediterranean region and typically involves the small intestine. Patients usually present with malabsorption syndrome, abdominal pain, diarrhoea, weight loss, and fever. There is extensive villous atrophy of the small intestinal mucosa, which is heavily infiltrated by small lymphocytes and plasma cells. The morphologic changes are consistent with a mucosa-associated lymphoid tissue lymphoma (MALT lymphoma)
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Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



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In some cases, chronic intestinal infection with Campylobacter jejuni imay result in chronic inflammation, a setting in which neoplastic transformation of a clone of abnormal B cells develops

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Lecuit M, Abachin E, Martin A, Poyart C, Pochart P, Suarez F, Bengoufa D, Feuillard J, Lavergne A, Gordon JI, Berche P, Guillevin L, Lortholary O (2004)
Immunoproliferative small intestinal disease associated with Campylobacter jejuni.
N Engl J Med 350: 239-48



. This is the most common heavy chain disease. It tends to progresses to large B-cell lymphoma, and normally has a poor prognosis.