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 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 types Amyloid 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
Disease name
Type of amyloid in the tissue
Precursor
Multiple Myeloma AL
Ig lambda (or kappa chains)

10. Secondary systemic amyloidosis
Disease name
Type of amyloid in the tissue
Chronic inflammatory disease. AA
Hemodialysis associate amyloidosis in chronic renal failure.
A-beta 2 micro globulin.
(Aβ2- micro globulin)
Rheumatoid arthritis

11. Localized amyloidosis : mainly due to aging
Disease name
Type of amyloid in the tissue
Senile cerebral amyloidosis: Alzheimer's disease
Amyloid beta protein
(Aß)
Type 2 diabetes
Islet amyloid polypeptide
( AIAPP) in islet of Langerhans

12. Disease and amyloid Aβ2 micro globulin Alzheimer's disease
Multiple Myeloma ( Primary) AL
Rheumatoid arthritis/ TB
( secondary, reactive) AA
Senile systemic amyloidosis, familial polyneuropathies
TTR
Acute Familial Mediterranean Fever
Hemodialysis associated
Aβ2 micro globulin
Alzheimer's disease
Amyloid beta
Medullary CA thyroid
calcitonin

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
Congo red ( normal light)
Brick red
Congo red ( polarized light)
Apple green birefringence

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
Kidney
Nephrotic syndrome, protenuria, renal failure
Tongue , GIT
Macroglossia, malabsorpton
Heart
Cardiomegaly, heart failure
Bone marrow involvement in Multiple Myeloma.
Fracture/ Multiple myeloma

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 of M-band in electrophoresis

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

23. Disease and amyloid Aβ2 micro globulin Alzheimer's disease
Multiple Myeloma ( Primary) AL
Rheumatoid arthritis/ TB
( secondary, reactive) AA
Senile systemic amyloidosis, familial polyneuropathies
TTR
Acute Familial Mediterranean Fever
Hemodialysis associated
Aβ2 micro globulin
Alzheimer's disease
Amyloid beta
Medullary CA thyroid
calcitonin

24. Thank you