Amyloidosis

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. Amyloidosis

3. 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 .

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

5. 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 – β 2 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 A β 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.. …!

7. 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

8. 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

10. 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.

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