9764/3 Immunoproliferative small intestinal disease


Mediterranean lymphoma


Small intestine
ICD-O-3 topography code: C17
ICD10: C88.3

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) 1
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


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
Click to access Pubmed
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.

Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: C88.3

/span>he term alpha heavy chain disease (alpha HCD) has been extensively used in the literature, since many cases are initially recognized by the presence of an abnormal alpha chain in the serum. However, the term immunoproliferative small intestinal disease (IPSID) was adopted by the WHO in 1978 and will be used here. IPSID is a variant of extranodal marginal zone lymphoma of mucosa associated lymphoid tissue (MALT)in which defective alpha heavy chains are secreted; it is also discussed in the section on extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT). It occurs in young adults and involves the gastrointestinal tract, resulting in malabsorption and diarrhoea. IPSID begins as a process sometimes reversible by antibiotics but may progress to diffuse large B-cell lymphoma (DLBCL).