Gastrointestinal manifestations of amyloidosis; Systemic amyloidosis
Amyloidoses are a diverse group of diseases characterized by abnormal folding of proteins and the extracellular tissue deposition of fibrils of low molecular weight subunits of normal serum proteins which disrupt tissue structure and function. The conversion of peptides or proteins from their insoluble functional states into highly organized fibrillar aggregates showing a cross-beta super-secondary structure is termed as “amyloid.” At least 28 different human proteins have been identified as amyloidogenic. Amyloidogenic deposition can be localized or systemic. Acquired causes of systemic amyloidoses include: (1) primary or AL amyloidosis in which the fibrils are composed of fragments of monoclonal light chains and affected patients may have amyloidosis alone or in association with other plasma cell dyscrasias, and (2) reactive (secondary) or AA amyloidosis in which the fibrils are composed of...
References and Further Reading
- Cowan, A., Skinner, M., & Seldin, D. et al. (2012) Amyloidosis of the gastrointestinal tract: A 13-year single center referral experience. Haematologica, 97. (epub ahead of print).Google Scholar
- Merlini, G., Seldin, D., & Gertz, M. (2011). Amyloidosis: Pathogenesis and new therapeutic options. Journal of Clinical Oncology, 14, 1924–1933.Google Scholar
- Sattianayagam, P. T., Hawkins, P. N., & Gillmore, J. D. (2009). Systemic amyloidosis and the gastrointestinal tract. Nature Reviews. Gastroenterology & Hepatology, 6, 608–617.Google Scholar