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Updated: Jun 25, 2026
05:48
Rapid Generation of Amyloid from Native Proteins In vitro
Published on: December 5, 2013
Primary (AL) amyloidosis with gastrointestinal involvement
Lone Galmstrup Madsen1, Peter Gimsing, Frank V Schiødt
1Department of Gastroenterology, Rigshospitalet, Copenhagen, Denmark.
Scandinavian Journal of Gastroenterology
|February 27, 2009
Summary
Gastrointestinal amyloidosis (AL) causes significant weight loss and malabsorption. Despite treatment, this rare disease has a poor prognosis, highlighting the need for earlier diagnosis and better therapies.
Area of Science:
- Gastroenterology
- Hematology
- Oncology
Background:
- Immunoglobulin light-chain (AL) amyloidosis is a rare systemic disease.
- Gastrointestinal (GI) involvement is a significant manifestation of AL amyloidosis.
- GI symptoms often precede definitive diagnosis, leading to delayed management.
Purpose of the Study:
- To characterize gastrointestinal manifestations of AL amyloidosis.
- To analyze patient symptoms, biochemical profiles, and treatment outcomes.
- To understand the diagnostic delay and prognostic factors in GI AL amyloidosis.
Main Methods:
- Retrospective study of 11 patients with AL amyloidosis and malabsorption.
- Inclusion criteria: patients evaluated in a Gastroenterology Department (2000-2006).
- Data collected on symptoms, diagnosis, treatment, and survival.
Main Results:
- All 11 patients had histologically confirmed GI amyloidosis and GI symptoms.
- Weight loss (10/11) and diarrhea (5/11) were most common; malabsorption (steatorrhea) present in 4/7.
- Diagnosis delay averaged 7 months; hypoalbuminemia (9/11) and anemia (6/11) were frequent.
- Treatment included chemotherapy or stem-cell transplantation; 5/11 patients died within the observation period.
Conclusions:
- Gastrointestinal AL amyloidosis predominantly presents with weight loss and malabsorption.
- Prognosis remains grave despite current therapeutic strategies.
- Early diagnosis and improved management are crucial for better patient outcomes.
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