Alpha Heavy Chain Disease

**Semantic type:** Neoplastic Process

**Definition:** A clonal disorder, also known as immunoproliferative small intestinal disease or Mediterranean lymphoma, characterized 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).

**Synonyms:** - Alpha heavy chain disease - IPSID - Immunoproliferative Small Intestinal Disease - Immunoproliferative disease, NOS - Immunoproliferative small intestinal disease - Immunoproliferative small intestinal diseases - Mediterranean Abdominal Lymphoma - Mediterranean Lymphoma - Mediterranean lymphoma