Yuksel SECKİN, Mehmet Ali ERDOGAN, Yasir Furkan CAGİN, Nese KARADAG SOYLU, Oguzhan YİLDİRİM, Saadet ALAN, Yilmaz BİLGİC, Ali Rıza ÇALIŞKAN

Gastrointestinal amyloidosis occurring in three different patterns: Case series

Systemic amyloidosis is a rare disease characterized by extracellular accumulation of amyloid protein in one or more organs. Inpatients with systemic amyloidosis, the most frequently affected organs are kidney and heart, followed by the nervous system,soft tissues, and lungs. Small bowel and liver involvement are also frequent in systemic amyloidosis. Gastrointestinal (GI) findingsare common, and the degree of organ involvement determines the symptoms. Patients usually have nonspecific findings suchas abdominal pain, nausea, diarrhea, and dysphagia, which may delay the appropriate diagnosis. Liver involvement occurs in themajority of patients, but the symptoms typically do not happen unless a marked hepatic amyloid deposition occurs. Diagnosis is bytissue biopsy. Treatment and prognosis depend on the underlying disease. GI system involvement is a sign of poor prognosis. In thiscase series, five patients who were diagnosed with gastrointestinal system amyloidosis in our clinic are presented.

PDF

___

1. Park MA, Mueller PS, Kyle RA. et. al. Primary (AL) hepatic amyloidosis: Clinical features and natural history in 98 patients. Medicine (Baltimore) 2003;82:291-8.
2. Gertz MA, Comenzo R, Falk RH. et al. Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (al): a consensus opinion from the 10 th international symposium on amyloid and amyloidosis. Am J Hematologly 2005;328:319-28.
3. Tada S, Fuchigami T, Okada M. et al. Gastrointestinal amyloidosis radiologic features by chemical types. Radiology 1994;190:37-42.
4. Merlini G, Comenzo RL, Seldin DC. et al. Immunoglobulin light chain amyloidosis. Expert Review of Hematology 2014;7:143-56.
5. Pinney JH, Lachmann HJ. Systemic AA amyloidosis. Subcellular Biochemistry 2012;65:541-64.
6. Westermark P, Westermark GT. Review. Reflections on amyloidosis in papua new guinea. Philos Trans R Soc Lond B Biol Sci 2008;360:3701-5.
7. Rowe K, Pankow J, Nehme F, et al. Gastrointestinal amyloidosis: review of the literature. Cureus 2017;9:e1228.
8. Tada S, Iida M, Iwashita A. et al. Endoscopic and biopsy findings of the upper digestive tract in patients with amyloidosis. Gastrointest Endosc 1990;36:10-4.
9. Tada S, Iida M, Yao T. et al. Endoscopic features in amyloidosis of the small intestine: clinical and morphologic differences between chemical types of amyloid protein. Gastrointest Endosc 1994;40:45-0.
10. Iwata T, Hoshii Y, Kawano H. et al. Hepatic amyloidosis in Japan: histological and morphometric analysis based on amyloid proteins. Hum Pathol 1995;26:1148-53.
11. Kyle RA, Greıpp PR. Amyloidosis (AL). Clinical and laboratory features in 229 cases. Mayo Clinic proc 1983;58:665-83.
12. Iida T, Yamano H, Nakase H. Systemic amyloidosis with gastrointestinal involvement: Diagnosis from endoscopic and histological views. Journal of Gastroenterology and Hepatology (Australia) 2018;33:583-90.
13. Poullos PD, Stollman N. Gastrointestinal Amyloidosis: Approach to Treatment. Current treatment options in Gastroenterol 2003;6:17-25.
14. Lim AY, Lee JH, Jung KS. et al. Clinical features and outcomes of systemic amyloidosis with gastrointestinal involvement: a single-center experience. The Korean J İnt Med 2015;30:496-505.
15. Lachmann HJ, Goodman HJB, Gilbertson JA. et al. Natural history and outcome in systemic aa amyloidosis. N Engl J Med 2007;356:2361-71.