SHM Converge Scientific Abstract ePoster Gallery-An Unusual and Complex Initial Presentation of Amyloidosis

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This case describes AL amyloidosis, a rare systemic disorder caused by misfolded immunoglobulin light chains produced by plasma cells. It commonly affects the kidneys, heart, and liver, while gastrointestinal involvement is less frequent and can make diagnosis difficult because symptoms are nonspecific and often attributed to isolated organ disease.<br /><br />The patient was a 69-year-old woman with HFpEF, chronic lymphedema, and hypertension who presented with one week of shortness of breath, leg swelling, vomiting described as “coffee-ground” material, and a sensation of food sticking in her throat and chest. On exam, she had bibasilar crackles, abdominal bloating, mild tenderness, and bilateral leg edema. Labs showed hyponatremia, elevated BUN and creatinine, leukocytosis, and proteinuria with bacteria/WBCs on urinalysis. CT imaging showed a distended stomach with gastric wall air, edematous peritoneum, and emphysematous bladder.<br /><br />She was initially treated for AKI and sepsis from cystitis, but further evaluation revealed ulceration of the gastric cardia and esophagus consistent with ischemia, a large pleural effusion requiring chest tube placement, gastroparesis requiring PEGJ tube feeding, and worsening renal failure requiring hemodialysis. Serum protein electrophoresis found a gamma-migrating paraproteinemia, and light chain testing showed a markedly reduced kappa/lambda ratio. An abdominal fat pad biopsy was Congo red positive with apple-green birefringence, confirming amyloid deposition. Additional workup identified a plasma cell neoplasm consistent with smoldering multiple myeloma, leading to a final diagnosis of AL amyloidosis.<br /><br />She was treated with daratumumab plus CyBorD and improved, eventually tolerating oral intake and showing normalized renal function. The case highlights the importance of considering systemic amyloidosis in patients with unexplained, persistent, or atypical organ dysfunction.

Asset Subtitle

Shimon Garrel

Meta Tag

Author List Manile A. Dastagir, Neha Puttagunta, Shimon Garrel, Shruti Varadarajan

Category Clinical Vignettes

Concept AL Amyloidosis

Concept Plasma Cell Disorder

Concept Immunoglobulin Light Chain

Concept Renal involvement

Concept Cardiac Involvement

Distinguished Non-Finalist

Presenter Organization SUNY Downstate Medical School

Presenting Author Shimon Garrel

Track Adult

Keywords

AL amyloidosis

systemic amyloidosis

plasma cell neoplasm

smoldering multiple myeloma

Congo red positive

light chain deposition

gastroparesis

renal failure

cardiac involvement

gastrointestinal ischemia

AL Amyloidosis

Plasma Cell Disorder

Immunoglobulin Light Chain

Renal involvement

Cardiac Involvement