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Amyloidosis
Dr. Amitabha Basu
What is amyloid (amylin : starch-like)?
►Def : Homogeneous, Pink, extra cellular ,
pathologic material.
Nature of Amyloid
A. Physical Nature : non branching protein
fibrils 7.5 -10 nm in diameter. Beta pleated
sheet.
B. Chemical nature :
95% Protein Fibril
Beta-Pleated Sheet
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)
Pathogenesis
►Precursor of AA = SAA ( Serum Amyloid
Associate protein from liver): chronic
inflammation.
►Precursor of AL = Immunoglobulin from
plasma cells = ? Multiple Myeloma .
Amyloidosis
►Def : Disease characterized by deposition of
amyloid in the tissue.
Classifications of Amyloidosis
A. Systemic amyloidosis
1. Primary amyloidosis
2. Secondary amyloidosis
B. Localized amyloidosis
1. Senile cerebral
2. Senile cardiac
3. Type 2 diabetes.
Primary systemic amyloidosis
Disease name Type of
amyloid in the
tissue
Precursor
Multiple
Myeloma
AL Ig lambda
(or kappa
chains)
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 AA
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
Disease and amyloid
Multiple Myeloma ( Primary) AL
Rheumatoid arthritis/ TB
( secondary, reactive)
AA
Senile systemic amyloidosis,
familial polyneuropathies
TTR
Acute Familial
Mediterranean Fever
AA
Hemodialysis associated Aβ2 micro globulin
Alzheimer's disease Amyloid beta
Medullary CA thyroid calcitonin
Special stain and amyloid
►Lugals iodine: Used only on gross specimen
►PAS: Stain P component ( in Biopsy
specimen)
►Congored : Most important (in Biopsy
specimen)
Special stain and amyloid
►Lugals iodine : Brown in gross specimen of
heart.
Special stain and amyloid
Congo red ( normal
light)
Brick red
Congo red ( polarized
light)
Apple green
birefringence
Morphology
►Gross of any organ with amyloidosis :
Waxy in nature.
Amyloidosis of kidney ; amyloid deposit
in the mesengeum of the glomerular
tuft
Amyloid in heart : name the stain
and viewing light
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
Laboratory findings
1. Biopsy from gingiva and rectum and
special stain.
2. Diagnosis of Multiple Myeloma associated
amyloid (AL).
1. Presence of M-band in electrophoresis.
2. Bens-Jones protein in the urine.
3. Increased light chain and monoclonal Ig.
Presence of
M-band in
electrophoresis
Disease and Amyloid
►Primary amyloidosis: AL,
►Secondary amyloidosis : AA,
►Hereditary (familial) amyloidosis: ATTR
Disease and amyloid
Multiple Myeloma ( Primary) AL
Rheumatoid arthritis/ TB
( secondary, reactive)
AA
Senile systemic amyloidosis,
familial polyneuropathies
TTR
Acute Familial
Mediterranean Fever
AA
Hemodialysis associated Aβ2 micro globulin
Alzheimer's disease Amyloid beta
Medullary CA thyroid calcitonin
Thank you

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7598047.ppt

  • 2. What is amyloid (amylin : starch-like)? ►Def : Homogeneous, Pink, extra cellular , pathologic material.
  • 3. Nature of Amyloid A. Physical Nature : non branching protein fibrils 7.5 -10 nm in diameter. Beta pleated sheet. B. Chemical nature : 95% Protein Fibril
  • 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 A. Systemic amyloidosis 1. Primary amyloidosis 2. Secondary amyloidosis B. Localized amyloidosis 1. Senile cerebral 2. Senile cardiac 3. 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 AA
  • 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 Multiple Myeloma ( Primary) AL Rheumatoid arthritis/ TB ( secondary, reactive) AA Senile systemic amyloidosis, familial polyneuropathies TTR Acute Familial Mediterranean Fever AA 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 1. Biopsy from gingiva and rectum and special stain. 2. Diagnosis of Multiple Myeloma associated amyloid (AL). 1. Presence of M-band in electrophoresis. 2. Bens-Jones protein in the urine. 3. Increased light chain and monoclonal Ig.
  • 22. Disease and Amyloid ►Primary amyloidosis: AL, ►Secondary amyloidosis : AA, ►Hereditary (familial) amyloidosis: ATTR
  • 23. Disease and amyloid Multiple Myeloma ( Primary) AL Rheumatoid arthritis/ TB ( secondary, reactive) AA Senile systemic amyloidosis, familial polyneuropathies TTR Acute Familial Mediterranean Fever AA Hemodialysis associated Aβ2 micro globulin Alzheimer's disease Amyloid beta Medullary CA thyroid calcitonin