Pathology of Amyloidosis

1. “All evil comes from people whose idea
of wellbeing only includes themselves,
their own family, community, race,
religion or nation.”
-- Sadhguru
Selfishness is the cause of evil…!

2. 2
CLINICAL PATHOLOGY
The foundation of Clinical Medicine.
Shashidhar Venkatesh Murthy
A/Prof & Head of Pathology
College of Medicine & Dentistry
Amyloidosis

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Amyloidosis: Introduction
 Group of disorders with extracellular
deposition of fibrillary non digestible
abnormal (misfolded) proteins waxy material
(> 20 different proteins).
 “Amyloid” (starch like - Virchow) due to
charged sugar molecules in the protein.
 Routine Microscopy shows as uniform pink
deposits.
Glomerulus - Congo Red Stain
Polarized Microscopy
 Congo red stain – specifically stains
amyloid – red which apear apple green
under polarized microscopy.
 Primary, Secondary/Inflammatory*,
Systemic / Local & Hereditary types.
 Hepatosplenomegaly, restrictive
cardiomyopathy & organ failure.

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Amyloidosis: Types & their Pathogenesis:
Primary Secondary
Senile / Hereditary
Pathogenesis
β pleated

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Amyloidosis: Clinical Disorders
 Systemic Amyloidosis: (multiorgan)
 Primary: Plasma cell / Immunocyte disorders: Multiple myeloma. - AL
 Secondary: Reactive, Chronic Inflam. TB, Auto.Imm dis. – AA
 Hemodialysis Associated: Chronic Renal Failure – β2 microglob.
 Systemic Senile Amyloidosis: Ageing Transthyretin, cardiomyopathy ATTR
 Hereditary Amyloidosis: Many subtypes, familial: ATTR
ATTR: Amyloid of TTR transporter of Thyroxin & Retinol (VitaminA)
 Localized Amyloidosis: (Specific tissue / organ)
 Senile cerebral: Alzheimer disease Aβ
 Endocrine: Diabetes DM2 – AIAPP Proinsulin.
 Cardiac Amyloidosis: atrial natriuretic factor. AANF
 Medullary carcinoma thyroid – ACal – Calcitonin.

6. “All evil comes from
people whose idea of
wellbeing only -- Sadhguru
Think before thinking negative..…!

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Senile Systemic Amyloidosis – Heart, Restrictive CMP
 Previously known as Senile Cardiac Amyloidosis.
 Restrictive Cardiomyopathy & Arrhythmias.
 American Black population 4%, Transthyretin (ATTR)
 Familial amyloid polyneuropathy (FAP): Sweden, Japan, common.
Normal

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Amyloidosis: Morphology
 Gross: Organ enlargement, pale, rubbery firm (waxy deposits)
 Microscopy: extracellular Pink deposits. Congo Red stain +ve.
 Effects: Pressure atrophy of surrounding cells, Narrowing of BV.
Ischemia, infarctions, dysfunction / hypofunction.
Kidney
Liver
Normal

9. “All evil comes from people whose
idea of wellbeing only -- Sadhguru

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Amyloidosis: Summary
 Extracellular deposition of waxy, misfolded, non digestible proteins
with β pleated fibrillary structure.
 Leading to cellular damage & organ failure.
 Primary, Secondary, Systemic, Localized & Hereditary types.
 Secondary Amyloidosis, Commonest, chronic inflammation. SAA.
 Clinical: Hepatosplenomegaly, brain, kidney & Heart failure
 Gross: Firm, pale, waxy enlargement of organs.
 Microscopy: Extracellular, pink protein.
Congo red stain positive, apple green under polarized microscopy.
 Late complication in chronic infections(TB), Inflammations,
Autoimmune disorders, Ageing and B cell neoplasms like multiple
myeloma. Rarely hereditary disorders.

11. “No doubt knowledge is valuable,
but above it is Character”
Knowledge without character is dangerous!