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Amyloidosis

Amyloidosis. Dr. Amitabha Basu. What is amyloid (amylin : starch-like)?. Def : Homogeneous, Pink, extra cellular , pathologic material. Nature of Amyloid. Physical Nature : non branching protein fibrils 7.5 -10 nm in diameter. Beta pleated sheet. Chemical nature : 95% Protein Fibril .

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Amyloidosis

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  1. Amyloidosis Dr. Amitabha Basu

  2. What is amyloid (amylin : starch-like)? • Def : Homogeneous, Pink, extra cellular , pathologic material.

  3. Nature of Amyloid • Physical Nature : non branching protein fibrils 7.5 -10 nm in diameter. Beta pleated sheet. • Chemical nature : 95% Protein Fibril

  4. Beta-Pleated Sheet

  5. Chemical nature • Amyloid = 95% Protein Fibril + 5% Glycoprotein P component - pentagonal molecule • Many different chemical types of amyloid • Common typesAmyloid associate ( AA)Amyloid light chain (AL)

  6. Pathogenesis • Precursor of AA = SAA ( Serum Amyloid Associate protein from liver): chronic inflammation. • Precursor of AL = Immunoglobulin from plasma cells = ? Multiple Myeloma .

  7. Amyloidosis • Def : Disease characterized by deposition of amyloid in the tissue.

  8. Classifications of Amyloidosis • Systemic amyloidosis • Primary amyloidosis • Secondary amyloidosis • Localized amyloidosis • Senile cerebral • Senile cardiac • Type 2 diabetes.

  9. Primary systemic amyloidosis

  10. Secondary systemic amyloidosis

  11. Localized amyloidosis : mainly due to aging

  12. Disease and amyloid

  13. Special stain and amyloid • Lugals iodine: Used only on gross specimen • PAS: Stain P component ( in Biopsy specimen) • Congored : Most important (in Biopsy specimen)

  14. Special stain and amyloid • Lugals iodine : Brown in gross specimen of heart.

  15. Special stain and amyloid

  16. Morphology • Gross of any organ with amyloidosis : Waxy in nature.

  17. Amyloidosis of kidney ; amyloid deposit in the mesengeum of the glomerular tuft

  18. Amyloid in heart : name the stain and viewing light

  19. Clinical manifestation

  20. Laboratory findings • Biopsy from gingiva and rectum and special stain. • Diagnosis of Multiple Myeloma associated amyloid (AL). • Presence of M-band in electrophoresis. • Bens-Jones protein in the urine. • Increased light chain and monoclonal Ig.

  21. Presence ofM-band in electrophoresis

  22. Disease and Amyloid • Primary amyloidosis: AL, • Secondary amyloidosis : AA, • Hereditary (familial) amyloidosis: ATTR

  23. Disease and amyloid

  24. Thank you

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