Clinical features and outcomes of systemic amyloidosis with gastrointestinal involvement: a single-center experience

Abstract

Background/AimsThe 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.MethodsWe 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.ResultsAmong 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).ConclusionsThe 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.

Amyloidosis of the colon

“…In another report, the most common symptoms of gastrointestinal amyloidosis were diarrhea (45.8%), anorexia (37.5%), weight loss and nausea and/or vomiting (29.2%) and the histologically confirmed gastrointestinal tract site was the stomach (55.0%), colon (45.0%) and rectum (35.0%). Patients with gastrointestinal involvement had a greater frequency of organ involvement [1].…”

“…The underlying cause of the amyloidosis should be treated and this may result in lasting regression of the gastrointestinal amyloidosis [9-11]. The prognosis of patients with AL amyloidosis (amyloid light-chain amyloidosis) and gastrointestinal involvement was poorer than those without gastrointestinal involvement, and they presented with more organ involvement and more advanced disease than those without organ involvement [1].…”

“…Gastrointestinal amyloidosis is relatively rare in western countries occurring in 3-8% of patients with systemic amyloidosis, but a recent report from Korea found 15.5% gastrointestinal involvement [1]. The most frequent clinical manifestation in all patients with amyloidosis are weight loss and gastrointestinal bleeding [2].…”

“…The most frequent clinical manifestation in all patients with amyloidosis are weight loss and gastrointestinal bleeding [2]. However, diarrhea is seen in patients with secondary amyloidosis [1]. The majority of patients with amyloidosis (80%) have primary amyloidosis, and most of these patients are over 50 years old [2].…”

A case of multiple myeloma initially presenting as hematemesis

“…Gastrointestinal manifestation of amyloidosis is often asymptomatic (2). A few cases of amyloidosis-induced gastrointestinal hemorrhage have been reported (3)(4)(5)(6)(7), but amyloidosis-induced hematemesis as an initial symptom in multiple myeloma has been rarely reported.…”