SHM Converge Scientific Abstract ePoster Gallery-Everything Everywhere All at Once: Immunoglobulin Light-Chain Amyloidosis

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This case describes a 73-year-old veteran with hypertension, remote stroke, and PTSD who presented with 3 months of dyspnea on exertion and 6 months of progressive, severe edema that spread from the legs to the thighs, groin, hands, and face. Despite normal vital signs, exam showed inspiratory crackles and marked anasarca with a 40-pound weight gain.<br /><br />Key laboratory findings suggested systemic amyloidosis: creatinine 1.09 mg/dL, albumin 1.1 g/dL, urine protein/creatinine ratio 5500 mg/g, and a markedly abnormal serum kappa/lambda ratio of 0.04. Renal biopsy confirmed AL (immunoglobulin light chain) amyloidosis, with Congo red staining showing apple-green birefringence in glomerular capillary loops, vessel walls, and peritubular capillaries. PET imaging later showed diffuse left ventricular FDG uptake, consistent with cardiac involvement.<br /><br />The patient developed multiple complications typical of AL amyloidosis, including refractory volume overload, symptomatic anemia from gastrointestinal bleeding, and recurrent syncope most consistent with autonomic dysfunction. EGD demonstrated distal esophageal AVMs, and colon biopsy showed erythematous, friable lesions with amyloid deposition in vessel walls. He ultimately had 12 additional hospitalizations over the next 9 months.<br /><br />The discussion emphasizes that AL amyloidosis is a systemic disease caused by misfolded light chains produced by clonal plasma cells. It commonly affects the heart and kidneys but can also cause orthostatic hypotension, peripheral neuropathy, GI dysmotility, and bleeding. GI involvement is associated with a poor prognosis, and duodenal biopsy has the highest diagnostic yield. Because organ damage is often irreversible, early recognition is critical. Treatment aims to slow progression, not reverse established disease. In this case, the hospital team discussed goals of care early, and the patient later entered hospice.

Asset Subtitle

Cory D. Ritter

Meta Tag

Author List Cory D. Ritter, Isaac Lill, Lakshmi B. Konduru

Category Clinical Vignettes

Concept AL Amyloidosis

Concept Early Diagnosis

Concept Cardiac Involvement

Concept Renal involvement

Concept Gastrointestinal Involvement

Distinguished Non-Finalist

Presenter Organization Baylor College of Medicine

Presenting Author Cory D. Ritter

Track Adult

Keywords

AL amyloidosis

systemic amyloidosis

light chain amyloidosis

nephrotic syndrome

anasarca

cardiac involvement

renal biopsy

Congo red staining

gastrointestinal bleeding

autonomic dysfunction

AL Amyloidosis

Early Diagnosis

Cardiac Involvement

Renal involvement

Gastrointestinal Involvement