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

AL amyloidosis differs from AA amyloidosis in that AL amyloid fibrils are derived from:

  • A Serum amyloid A protein, an acute-phase reactant
  • B Immunoglobulin light chain variable regions produced by a plasma cell dyscrasia
  • C Transthyretin (prealbumin) mutant or wild-type protein
  • D Beta-2 microglobulin in patients on long-term dialysis
Correct answer: B. Immunoglobulin light chain variable regions produced by a plasma cell dyscrasia

Explanation

AL (primary) amyloidosis fibrils are derived from the N-terminal variable domains of immunoglobulin light chains (kappa or lambda), produced by a clonal plasma cell population (myeloma or monoclonal gammopathy). AA amyloidosis is derived from serum amyloid A, an acute-phase protein elevated in chronic inflammatory states. ATTR amyloidosis involves transthyretin; Abeta-2M involves beta-2 microglobulin.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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