Amyloidosis

24
Amyloidosis Amyloidosis Dr. Amitabha Basu Dr. Amitabha Basu

description

Amyloidosis. Dr. Amitabha Basu. What is amyloid (amylin : starch-like)?. Def : Homogeneous, Pink, extra cellular , pathologic material. Nature of Amyloid. Physical Nature : non branching protein fibrils 7.5 -10 nm in diameter. Beta pleated sheet. Chemical nature : 95% Protein Fibril . - PowerPoint PPT Presentation

Transcript of Amyloidosis

Page 1: Amyloidosis

AmyloidosisAmyloidosis

Dr. Amitabha BasuDr. Amitabha Basu

Page 2: Amyloidosis

What is amyloid (amylin : starch-What is amyloid (amylin : starch-like)?like)?

►Def : Homogeneous, Pink, extra Def : Homogeneous, Pink, extra cellular , pathologic material.cellular , pathologic material.

Page 3: Amyloidosis

Nature of AmyloidNature of AmyloidA.A. Physical NaturePhysical Nature : non branching : non branching

protein fibrils 7.5 -10 nm in diameter. protein fibrils 7.5 -10 nm in diameter. Beta pleated sheet.Beta pleated sheet.

B.B. Chemical natureChemical nature : :95% Protein Fibril 95% Protein Fibril

Page 4: Amyloidosis

Beta-Pleated Sheet Beta-Pleated Sheet

Page 5: Amyloidosis

Chemical natureChemical nature►Amyloid = Amyloid = 95% Protein Fibril95% Protein Fibril + 5% + 5%

Glycoprotein P component - pentagonal Glycoprotein P component - pentagonal moleculemolecule

►Many different chemical types of amyloidMany different chemical types of amyloid►Common typesCommon types

Amyloid associate ( AA)Amyloid associate ( AA)Amyloid light chain (AL)Amyloid light chain (AL)

Page 6: Amyloidosis

PathogenesisPathogenesis►PrecursorPrecursor of AA = of AA = SAASAA ( ( SSerum erum AAmyloid myloid

AAssociate protein from liver): chronic ssociate protein from liver): chronic inflammation.inflammation.

►PrecursorPrecursor of AL = Immunoglobulin from of AL = Immunoglobulin from plasma cells = ? Multiple Myeloma .plasma cells = ? Multiple Myeloma .

Page 7: Amyloidosis

AmyloidosisAmyloidosis►Def : Disease characterized by Def : Disease characterized by

deposition of amyloid in the tissue.deposition of amyloid in the tissue.

Page 8: Amyloidosis

Classifications of Classifications of AmyloidosisAmyloidosis

A.A. Systemic amyloidosisSystemic amyloidosis1.1. Primary amyloidosisPrimary amyloidosis2.2. Secondary amyloidosisSecondary amyloidosis

B.B. Localized amyloidosisLocalized amyloidosis1.1. Senile cerebralSenile cerebral2.2. Senile cardiacSenile cardiac3.3. Type 2 diabetes.Type 2 diabetes.

Page 9: Amyloidosis

Primary systemic Primary systemic amyloidosisamyloidosis

Disease Disease namename

Type of Type of amyloid in amyloid in the tissuethe tissue

PrecursorPrecursor

Multiple Multiple MyelomaMyeloma

ALAL Ig lambdaIg lambda(or kappa (or kappa chains)chains)

Page 10: Amyloidosis

Secondary systemic Secondary systemic amyloidosisamyloidosis

Disease nameDisease name Type of Type of amyloid in the amyloid in the tissuetissue

Chronic inflammatory Chronic inflammatory disease.disease.

AAAA

Hemodialysis associate Hemodialysis associate amyloidosis in chronic amyloidosis in chronic renal failure.renal failure.

A-beta 2 micro A-beta 2 micro globulin.globulin.(A(Aββ2- 2- micro micro globulinglobulin) )

Rheumatoid arthritisRheumatoid arthritis AAAA

Page 11: Amyloidosis

Localized amyloidosis : Localized amyloidosis : mainly due to agingmainly due to agingDisease nameDisease name Type of Type of

amyloid in the amyloid in the tissuetissue

Senile Senile cerebral cerebral amyloidosis:amyloidosis:Alzheimer's Alzheimer's disease disease

Amyloid beta Amyloid beta proteinprotein

(A(Aß)ß)

Type 2 Type 2 diabetesdiabetes

Islet amyloid Islet amyloid polypeptide polypeptide ( AIAPP) in ( AIAPP) in islet of islet of LangerhansLangerhans

Page 12: Amyloidosis

Disease and amyloidDisease and amyloidMultiple Myeloma Multiple Myeloma ( Primary)( Primary)

ALAL

Rheumatoid arthritis/ TB Rheumatoid arthritis/ TB ( secondary, reactive)( secondary, reactive)

AAAA

Senile systemic Senile systemic amyloidosis, amyloidosis, familial familial polyneuropathiespolyneuropathies

TTRTTR

Acute Familial Acute Familial Mediterranean FeverMediterranean Fever

AA AA

Hemodialysis associatedHemodialysis associated AAββ2 micro 2 micro globulinglobulin

Alzheimer's disease Alzheimer's disease Amyloid betaAmyloid betaMedullary CA thyroidMedullary CA thyroid calcitonincalcitonin

Page 13: Amyloidosis

Special stain and amyloidSpecial stain and amyloid►Lugals iodine: Used only on gross Lugals iodine: Used only on gross

specimenspecimen►PAS: Stain PAS: Stain PP component ( in Biopsy component ( in Biopsy

specimen)specimen)►Congored : Most important (in Biopsy Congored : Most important (in Biopsy

specimen)specimen)

Page 14: Amyloidosis

Special stain and amyloidSpecial stain and amyloid►Lugals iodine : Brown in gross Lugals iodine : Brown in gross

specimen of heart.specimen of heart.

Page 15: Amyloidosis

Special stain and amyloidSpecial stain and amyloidCongo red ( normal Congo red ( normal light)light)

Brick red Brick red

Congo red Congo red ( polarized light)( polarized light)

Apple green Apple green birefringencebirefringence

Page 16: Amyloidosis

MorphologyMorphology►Gross of any organ with amyloidosis :

Waxy in nature.

Page 17: Amyloidosis

Amyloidosis of kidney ; amyloid Amyloidosis of kidney ; amyloid deposit in the mesengeum of the deposit in the mesengeum of the

glomerular tuftglomerular tuft

Page 18: Amyloidosis

Amyloid in heart : name the Amyloid in heart : name the stain and viewing light stain and viewing light

Page 19: Amyloidosis

Clinical manifestationClinical manifestationKidney Kidney Nephrotic syndrome, Nephrotic syndrome,

protenuria, renal protenuria, renal failurefailure

Tongue , GITTongue , GIT Macroglossia, Macroglossia, malabsorptonmalabsorpton

Heart Heart Cardiomegaly, heart Cardiomegaly, heart failurefailure

Bone marrow Bone marrow involvement in involvement in Multiple Multiple Myeloma.Myeloma.

Fracture/ Multiple Fracture/ Multiple myeloma myeloma

Page 20: Amyloidosis

Laboratory findingsLaboratory findings1.1. Biopsy from gingiva and rectum and Biopsy from gingiva and rectum and

special stain.special stain.2.2. Diagnosis of Multiple Myeloma Diagnosis of Multiple Myeloma

associated amyloid (AL).associated amyloid (AL).1.1. Presence of M-band in electrophoresis.Presence of M-band in electrophoresis.2.2. Bens-Jones protein in the urine.Bens-Jones protein in the urine.3.3. Increased light chain and monoclonal Ig.Increased light chain and monoclonal Ig.

Page 21: Amyloidosis

Presence ofPresence of M-bandM-band in in

electrophoresielectrophoresiss

Page 22: Amyloidosis

Disease and AmyloidDisease and Amyloid►Primary amyloidosis: Primary amyloidosis: AL,AL, ►Secondary amyloidosis : Secondary amyloidosis : AA,AA, ►Hereditary (familial) amyloidosis: Hereditary (familial) amyloidosis: ATTRATTR

Page 23: Amyloidosis

Disease and amyloidDisease and amyloidMultiple Myeloma Multiple Myeloma ( Primary)( Primary)

ALAL

Rheumatoid arthritis/ TB Rheumatoid arthritis/ TB ( secondary, reactive)( secondary, reactive)

AAAA

Senile systemic Senile systemic amyloidosis, amyloidosis, familial familial polyneuropathiespolyneuropathies

TTRTTR

Acute Familial Acute Familial Mediterranean FeverMediterranean Fever

AA AA

Hemodialysis associatedHemodialysis associated AAββ2 micro 2 micro globulinglobulin

Alzheimer's disease Alzheimer's disease Amyloid betaAmyloid betaMedullary CA thyroidMedullary CA thyroid calcitonincalcitonin

Page 24: Amyloidosis

Thank youThank you