Amyloidosis

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Amyloidosis

• Dr. Amitabha Basu

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What is amyloid (amylin starch-like)?

• Def Homogeneous, Pink, extra cellular ,
  pathologic material.

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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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Beta-Pleated Sheet
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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)

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Pathogenesis

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

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Amyloidosis

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

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Classifications of Amyloidosis

• Systemic amyloidosis
• Primary amyloidosis
• Secondary amyloidosis
• Localized amyloidosis
• Senile cerebral
• Senile cardiac
• Type 2 diabetes.

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Primary systemic amyloidosis
Disease name Type of amyloid in the tissue Precursor
Multiple Myeloma AL Ig lambda(or kappa chains)
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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. (Aß2- micro globulin)
Rheumatoid arthritis AA
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Localized amyloidosis mainly due to aging
Disease name Type of amyloid in the tissue
Senile cerebral amyloidosisAlzheimer's disease Amyloid beta protein (Aß)
Type 2 diabetes Islet amyloid polypeptide ( AIAPP) in islet of Langerhans
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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 Aß2 micro globulin
Alzheimer's disease Amyloid beta
Medullary CA thyroid calcitonin
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Special stain and amyloid

• Lugals iodine Used only on gross specimen
• PAS Stain P component ( in Biopsy specimen)
• Congored Most important (in Biopsy specimen)

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Special stain and amyloid

• Lugals iodine Brown in gross specimen of heart.

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Special stain and amyloid
Congo red ( normal light) Brick red
Congo red ( polarized light) Apple green birefringence
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Morphology

• Gross of any organ with amyloidosis Waxy in
  nature.

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Amyloidosis of kidney amyloid deposit in the
mesengeum of the glomerular tuft
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Amyloid in heart name the stain and viewing
light
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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
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Laboratory findings

1. Biopsy from gingiva and rectum and special stain.
2. Diagnosis of Multiple Myeloma associated amyloid
   (AL).
3. Presence of M-band in electrophoresis.
4. Bens-Jones protein in the urine.
5. Increased light chain and monoclonal Ig.

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Presence of M-band in electrophoresis
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Disease and Amyloid

• Primary amyloidosis AL,
• Secondary amyloidosis AA,
• Hereditary (familial) amyloidosis ATTR

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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 Aß2 micro globulin
Alzheimer's disease Amyloid beta
Medullary CA thyroid calcitonin
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Thank you