Ett tillstånd som orsakas av lymfocythyperplasi i tunntarmen och mesenterialkörtlarna. Dessa lymfocyter producerar ett avvikande kedjeprotein av tunga kedjor. Patienter med detta sjukdomstillstånd har i allmänhet även lymfom eller utvecklar lymfom inom några få år. Sjukdomen beskrevs ursprungligen i medelhavsområdet.
A condition that is caused by HYPERPLASIA of LYMPHOCYTES in the small intestine (INTESTINE, SMALL) and the mesenteric LYMPH NODES. These lymphocytes produce an anomalous alpha heavy chain protein. Generally, these IPSID patients have either concurrent LYMPHOMA or develop lymphoma within a few years. The disease was first described in the Mediterranean region and is characterized by malabsorption; WEIGHT LOSS; DIARRHEA; and STEATORRHEA.
Inga svenska synonymer finns.
IPSID — Heavy Chain Disease, IgA Type — Lymphoma, Mediterranean — Mediterranean Lymphoma — alpha-Chain Disease — Disease, alpha-Chain — Diseases, alpha-Chain — alpha Chain Disease — alpha-Chain Diseases