Clinical features and outcomes of systemic amyloidosis with gastrointestinal involvement: a single-center experience
2
조회
0
다운로드
3
인용
0
참고문헌
키워드
초록
Background/Aims: The gastrointestinal (GI) tract often becomes involved in patients with systemic amyloidosis. As few GI amyloidosis data have been reported, we describe the clinical features and outcomes of patients with pathologically proven GI amyloidosis. Methods: We identified 155 patients diagnosed with systemic amyloidosis between April 1995 and April 2013. Twenty-four patients (15.5%) were diagnosed with GI amyloidosis using associated symptoms, and the diagnoses were confirmed by direct biopsy. Results: Among the 24 patients, 20 (83.3%) had amyloidosis light chain (AL), three (12.5%) had amyloid A, and one (4.2%) had transthyretin-related type amyloidosis. Their median age was 57 years (range, 37 to 72), and 10 patients were female (41.7%). The most common symptoms of GI amyloidosis were diarrhea (11 patients, 45.8%), followed by anorexia (nine patients, 37.5%), weight loss, and nausea and/ or vomiting (seven patients, 29.2%). The histologically confirmed GI tract site in AL amyloidosis was the stomach in 11 patients (55.0%), the colon in nine (45.0%), the rectum in seven (35.0%), and the small bowel in one (5.0%). Patients with GI involvement had a greater frequency of organ involvement (p = 0.014). Median overall survival (OS) in patients with GI involvement was shorter (7.95 months; range, 0.3 to 40.54) than in those without GI involvement (15.84 months; range, 0.0 to 114.53; p = 0.069) in a univariate analysis. A multivariate analysis of prognostic factors for AL amyloidosis revealed that GI involvement was not a significant predictor of OS (p = 0.447). Conclusions: The prognosis of patients with AL amyloidosis and GI involvement was poorer than those without GI involvement, and they presented with more organ involvement and more advanced disease than those without organ involvement.
Abstract
Background/Aims: The gastrointestinal (GI) tract often becomes involved in patients with systemic amyloidosis. As few GI amyloidosis data have been reported, we describe the clinical features and outcomes of patients with pathologically proven GI amyloidosis. Methods: We identified 155 patients diagnosed with systemic amyloidosis between April 1995 and April 2013. Twenty-four patients (15.5%) were diagnosed with GI amyloidosis using associated symptoms, and the diagnoses were confirmed by direct biopsy. Results: Among the 24 patients, 20 (83.3%) had amyloidosis light chain (AL), three (12.5%) had amyloid A, and one (4.2%) had transthyretin-related type amyloidosis. Their median age was 57 years (range, 37 to 72), and 10 patients were female (41.7%). The most common symptoms of GI amyloidosis were diarrhea (11 patients, 45.8%), followed by anorexia (nine patients, 37.5%), weight loss, and nausea and/ or vomiting (seven patients, 29.2%). The histologically confirmed GI tract site in AL amyloidosis was the stomach in 11 patients (55.0%), the colon in nine (45.0%), the rectum in seven (35.0%), and the small bowel in one (5.0%). Patients with GI involvement had a greater frequency of organ involvement (p = 0.014). Median overall survival (OS) in patients with GI involvement was shorter (7.95 months; range, 0.3 to 40.54) than in those without GI involvement (15.84 months; range, 0.0 to 114.53; p = 0.069) in a univariate analysis. A multivariate analysis of prognostic factors for AL amyloidosis revealed that GI involvement was not a significant predictor of OS (p = 0.447). Conclusions: The prognosis of patients with AL amyloidosis and GI involvement was poorer than those without GI involvement, and they presented with more organ involvement and more advanced disease than those without organ involvement.
참고문헌 (21)
- Merlini G, "Molecular mechanisms of amyloidosis", N Engl J Med, 349, pp.583~596 (2003)
- Ebert EC, "Gastrointestinal manifestations of amyloidosis", Am J Gastroenterol, 103, pp.776~787 (2008)
- Falk RH, "The systemic amyloidoses", N Engl J Med, 337, pp.898~909 (1997)
- Lachmann HJ, "Natural history and outcome in systemic AA amyloidosis", N Engl J Med, 356, pp.2361~2371 (2007)
- Petre S, "Review article: gastrointestinal amyloidosis: clinical features, diagnosis and therapy", Aliment Pharmacol Ther, 27, pp.1006~1016 (2008)
- Sipe JD, "Amyloid fibril protein nomenclature: 2012 recommendations from the Nomenclature Committee of the International Society of Amyloidosis", Amyloid, 19, pp.167~170 (2012)
- Cowan AJ, "Amyloidosis of the gastrointestinal tract: a 13-year, single-center, referral experience", Haematologica, 98, pp.141~146 (2013)
- Madsen LG, "Primary (AL) amyloidosis with gastrointestinal involvement", J Gastroenterol, 44, pp.708~711 (2009)
- Gertz MA, "Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis, Tours, France, 18-22 April 2004", Am J Hematol, 79, pp.319~328 (2005)
- Tada S, "Endoscopic and biopsy findings of the upper digestive tract in patients with amyloidosis", Gastrointest Endosc, 36, pp.10~14 (1990)
- Tada S., "Diagnosis of gastrointestinal amyloidosis with special reference to the relationship with amyloid fibril protein", Fukuoka Igaku Zasshi, 82, pp.624~647 (1991)
- Hayman SR, "Primary systemic amyloidosis: a cause of malabsorption syndrome", Am J Med, 111, pp.535~540 (2001)
- Obici L, "Clinical aspects of systemic amyloid diseases", Biochim Biophys Acta, 1753, pp.11~22 (2005)
- Menke DM, "Symptomatic gastric amyloidosis in patients with primary systemic amyloidosis", Mayo Clin Proc, 68, pp.763~767 (1993)
- James DG, "Clinical recognition of Al type amyloidosis of the luminal gastrointestinal tract", Clin Gastroenterol Hepatol, 5, pp.582~588 (2007)
- Gould M, "Small bowel amyloidosis", Curr Gastroenterol Rep, 15, pp.350~ (2013)
- Kumar S, "Revised prognostic staging system for light chain amyloidosis incorporating cardiac biomarkers and serum free light chain measurements", J Clin Oncol, 30, pp.989~995 (2012)
- Gertz MA., "Immunoglobulin light chain amyloidosis: 2013update on diagnosis, prognosis, and treatment", Am J Hematol, 88, pp.416~425 (2013)
- Kourelis TV, "Coexistent multiple myeloma or increased bone marrow plasma cells define equally high-risk populations in patients with immunoglobulin light chain amyloidosis", J Clin Oncol, 31, pp.4319~4324 (2013)
- Desikan KR, "Incidence and impact of light chain associated (AL) amyloidosis on the prognosis of patients with multiple myeloma treated with autologous transplantation", Leuk Lymphoma, 27, pp.315~319 (1997)
- Dember LM., "Amyloidosis-associated kidney disease", J Am Soc Nephrol, 17, pp.3458~3471 (2006)