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Pathology of Amyloidosis

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Amyloidosis


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all evil comes from people whose idea

“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…!

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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.

 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.

Glomerulus - Congo Red Stain

Polarized Microscopy

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Amyloidosis: Types & their Pathogenesis:

Pathogenesis

Primary

Secondary

Senile / Hereditary

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.

all evil comes from people whose idea 1

“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

Normal

 Previously known as Senile Cardiac Amyloidosis.

 Restrictive Cardiomyopathy & Arrhythmias.

 American Black population 4%, Transthyretin (ATTR)

 Familial amyloid polyneuropathy (FAP): Sweden, Japan, common.

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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.

Normal

Kidney

Liver

all evil comes from people whose idea 2

“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.

no doubt knowledge is valuable but above

“No doubt knowledge is valuable,

but above it is Character”

Knowledge without character is dangerous!