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Systemic light chain amyloidosis presenting as jejunal perforation
Hung-Chih Chen1, Ta-Chuan Yu2, Keng-Man Chiang2
1Internal Medicine, National Taiwan University Hospital, Taipei City, Taiwan.
Abstract:
Systemic light chain (AL) amyloidosis is a clonal plasma cell dyscrasia characterised by extracellular deposition of misfolded amyloid fibrils composed of immunoglobulin light chains. These amyloid fibrils disrupt tissue architecture, ultimately leading to organ dysfunction. Clinical symptoms vary among cases, depending on the organs involved. The heart and kidney are the most commonly affected vital organs, whereas jejunal involvement is rare.We report a case of jejunal perforation associated with systemic AL amyloidosis during treatment of recurrent uterine cancer. The deposition of amyloid fibrils has also been identified in uterine cancer tissue specimens. Tracing back to her history, postprandial abdominal distension developed 3 years before jejunal perforation. This case highlights both the chronic and acute gastrointestinal manifestations of AL amyloidosis; however, diagnosis and treatment remain challenging in patients with such complex comorbidities.
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