Pathology · Immunopathology (Hypersensitivity, Autoimmunity, Immunodeficiency, Amyloidosis)

In AL amyloidosis (primary amyloidosis), the fibril precursor protein is:

  • A Serum amyloid A protein (an acute phase reactant)
  • B Transthyretin (TTR)
  • C Beta-2-microglobulin
  • D Immunoglobulin light chain fragments (kappa or lambda)
Correct answer: D. Immunoglobulin light chain fragments (kappa or lambda)

Explanation

AL (amyloid light chain) amyloidosis derives from monoclonal immunoglobulin light chains produced by a plasma cell dyscrasia (myeloma or Waldenström's macroglobulinemia). The light chains (more often lambda than kappa) misfold into beta-pleated sheets forming amyloid fibrils. AA amyloidosis uses serum amyloid A (from chronic inflammation); hereditary amyloidosis uses transthyretin; dialysis-related amyloidosis uses beta-2-microglobulin.

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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