9760/3 Immunoproliferative disease, NOS


Definitions

Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: C88.9
           

A clonal disorder, also known as immunoproliferative small intestinal disease (IPSID) or Mediterranean lymphoma, characterised by the secretion of a defective alpha heavy chain. It predominantly affects young people in the Mediterranean region. It involves the small intestine, and patients usually present with malabsorption syndrome, abdominal pain, 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 small intestinal morphologic changes are consistent with a mucosa-associated lymphoid tissue lymphoma (MALT lymphoma). Alpha heavy chain disease frequently progresses to large B-cell lymphoma, and normally has a poor prognosis.

This diagnostic term is not contained in the 2008 WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues 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



. It is synonymous with alpha heavy chain disease.