Primary amyloidosis

4 Interesting Facts of Primary amyloidosis

  1. Primary systemic light chain amyloidosis is a plasma cell dyscrasia caused by a pathologic clonal population of plasma cells in the bone marrow that produce monoclonal light chains that misfold and aggregate into a fibrillar pattern
    • These amyloid fibrils’ monoclonal light chains have a high affinity for visceral organs, soft tissue, or nervous system
    • Deposition causes end-organ dysfunction in various organs (eg, kidney, heart, liver) and autonomic or peripheral nervous system dysfunction 
  2. Symptoms are diverse and include unexplained fatigue, edema, dyspnea, unintentional weight loss, orthostatic hypotension, and paresthesias 
  3. Serum and urine protein electrophoresis, serum and urine immunofixation electrophoresis, and serum free light chain testing show results similar to those in multiple myeloma and monoclonal gammopathy of undetermined significance 
    • Monoclonal spike is seen on serum electrophoresis in about half of cases
    • Elevated circulating serum free light chain levels, due to abnormalities of the κ or λ chains or the κ/λ ratio, are common
  4. Differentiated from multiple myeloma by histologic identification of amyloid deposits in affected tissues via tissue and bone marrow biopsy 
    • Most expeditious and safest way to confirm amyloidosis is to perform a needle aspiration of subcutaneous abdominal fat and stain both the fat and the bone marrow biopsy cellular specimen for amyloid

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