CASE REPORT   

Chirurgia 2005 February;18(1):35-8

Copyright © 2005 EDIZIONI MINERVA MEDICA

language: Italian

Chronic intestinal pseudo-obstruction secondary to AL amyloidosis associated with severe gastroparesis: case report

Lovisetto F., Zonta S., Alessiani M. Cobianchi L., Sgarella A., Fraipont G., Palladini G., Del Bello A., Dionigi P.

Amyloidosis is a disease characterized by the intercellular deposition of amyloid, an insoluble protein composed of AL type or AA type non-brenching fibrils of diameter 10 nm approximately. The AL amyloid is constituted by light chains of immunoglobulins while the AA amyloid is the main component of amyloid in the inflammatory chronic diseases. In this article we report the case of a patient who developed chronic intestinal pseudo-obstruction, a disorder characterized by signs and symptoms of intestinal obstruction in the absence of a mechanical cause, associated with severe gastric dismotility as a consequence of AL type amyloid deposition in the intestinal wall. This clinical picture acquires a special evidence since, although the involvement of the gastro-enteric distrect is not uncommon in systemic amyloidosis, the main finding of severe gastro-enteric dismotility disorder in amyloidosis AL is extremely rare. The deposit of amyloid AL in the gastric and intestinal wall generally leads to chronic bleeding whereas the amyloid AA causes intestinal functional disorder such as paresis and decreased nutritional intake. The review of the recent literature has showed that there were only 2 other reports dealing with patients showing this atypical association between this syndrome and amyloidosis AL. In spite of medical and surgical treatment, no clinical improvement was observed in the patient that we describe. The patient is maintained on total parenteral nutrition.