4amyloidosis Texts

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General Pathology

AmyloidosisFibrinoid, Hyalin

Inst. Pathol. ,1st Med. Faculty, Charles Univ. Praguehttp://www1.lf1.cuni.cz/~jdusk/

aroslava !u"#ov$


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AmyloidosisDEF.:disorder o% &roteinmetabolismaccom&anied

'ith abnormal e(tracellularde&osition o% &roteinaceous

material ) amyloid


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Amyloid * starch li#e

+arl Freiherr von o#itans#y -1/0)12

udol% 3ud'ig +arl 4ircho' -151)16/52.

Amyloid ) history


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+arl Freiherr von o#itans#y -1/0)12

Austrian &athologist, born February 16, 1/0,

+7niggr8t9, :7hmen, Austrian ;m&ire-no'Hradec +r$lov, 1, ?ien.

Handbuch der &athologischen AnatomieIInd:and, ?ien 105

Amyloid ) history 5.


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Amyloidosis @ mor&hologyMacroscopy:

small amounts @ invisible

larger de&osits @ enlarged,%irm, 'a(y organs


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Ultrastructure :iochemistry o% Amyloid909!" non #ranch$d

fi#rils dia%. 101&n%

!10" pco%pon$nt 'lycoprot$in (fi#ron$ctin) la%inin) colla'$n *


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Amyloidosis

con%ormational disease-Carrell and 3omas, 3ancet, 1662

Barises 'hen a constituent &rotein

undergoes a change in si9e or

%luctuation in sha&e 'ith resultantsel% ) association and tissue

de&ositionDpl$at$d + , sh$$t structur$


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Con%ormational diseases

-Carrell and 3omas, 3ancet, 1662

Amyloidosis Prionoses ) transmissibles&ongi%orm ence&halo&athies -incl. m.

C!2

m. Al9heimeri

pl$at$d + , sh$$t structur$


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Amyloidosis

-lassification:

according to the source &rotein-more than 5/ di%%erent identi%ied2

according to the distribution

systemic -generalised2

localised


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Eystemic Amyloidosis ) I.

A3) imunocyte dyscrasia associated

li'ht chains ' %ostly B&rimaryD

!istribution tongue, heart, GI, liver, s&leen,#idney

Associated diseasesPlasma cell myeloma,: cell lym&homa,


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Eystemic Amyloidosis ) II.

AA) reactive systemic amyloidosis

22 3 $ru% 2%yloid 2ssociat$d

prot$in BsecondaryD!istribution liver, #idney, s&leen, GI, lym&h nodes,bo'el, adi&ose tissue

Associated diseases rheumatoid arthritis, chronic

in%ections -tb, le&rosy, bronchiectasiae,

osteomyelitis, I:!, neo&lasms M3H , CC


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Eystemic Amyloidosis ) III.

senile systemic EEA

&!" p$opl$ o4$r th$ a'$ of 50 y$ars 6

)nor%al transthyr$tin 778 pr$al#u%in

)mostly heart vessels invilvement


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Eystemic Amyloidosis ) I4.

A5) hemodialysis associated

&%icro'lo#ulin

Hereditary

AA ) Familial Mediterranean Fever

A ) Famil. &olyneuro&atiatransthyr$tin%utat$d for%


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Eystemic Amyloidosis ) com&lications

diminished %unctions o% some organs, es&.

+I!; FAI3U;

IIIrd stage Amyloid ne&hrosis


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3ocalised Amyloidosis ) I.

Eenile cardial

2778) transthyr$tin structurally nor%al

Eenile cerebral

2

) a%yloidprot$in


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Cardiac Amyloidosis @ clinical mani%estations

!ilated Cardiomyo&athy pr$do%inant systolicdysfunction

estrictive cardiomyo&athy pr$do%inant diastolicdysfunction

Congestive heart %ailure hytm abnormalities

Coronary insu%%iciency

4alvular dys%unction

Pericardial tam&onade

;nhance sensitivity to digitalis glycosides

Atrial thrombosis ) embolisation


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3ocalised Amyloidosis ) II.

;ndocrine

ACal ) ca medullare gl. thyreoideae AIAPP ) islets o% 3angerhans associated

AAF ) isolated atrial amyloidosis atrial natriur$tic polyp$ptid$

odular tumori%orm amyloid de&osits ton'u$) lun')laryn) skin) urinary #ladd$r) or#ita


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Clinical !iagnosis o% Amyloid

Ecintigra&hy-in vivo2

using human serum amyloid com&onentmar#ed 'ith 15>

;chocardiogra&hy-atrial amyloid2


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Clinical !iagnosis o% Amyloid

:iochemistry

seJuening !A )hered. %orms

e(traction o% %ibrils fro% a #iopsy sp$ci%$n

s&ectrometry

seJuening o% the amyloid &rotein


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Amyloidosis @ mor&hology

Macroscopy:

small amounts @ invisible

larger de&osits @ enlarged,%irm, 'a(y organs


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Mor&hological

!iagnosis o% Amyloid

Macrosco&y

@reaction 4ircho' I sol. u'olli

4ircho' II ;&


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Mor&hological

!iagnosis o% AmyloidMicrosco&y

@ +KGK red

-LPK3AIEAIK2 L +MnK0

@ thio%lavine E,

@ crystal. violet %$tachro%asia@ IMMUKHIEKCH;MIE

$l$ctron %icroscopy


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Mor&hological !iagnosis o% Amyloid

Materials

@ GI -stomach, duodenum rectum, gingiva2

bio&sy@ #idney

@ sural nerve muscle

@ %at as&irationbio&sy @ needle 'ith an internal

diam. /,)1,5mm

Rcken Ch. Sletten K.: Amyloid in Surgical Pathology

Virchows Arch., 2!, "#2$


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CKGK ed synth$siz$d #y youn' ch$%ist at =ay$r co%p. 155> as

th$ first of $cono%ically lucrati4$ dir$ct nod n$$din' a%ordant t$til$ dy$s

pat$nt$d#y 2?F2 155!

2kti$n'$s$lschaft f@r2nilinfar#$nfa#rikation

> w$$ks aft$r th$ conclusion of th$A$st 2frica -onf$r$nc$

to Europ$ans in 155!) th$ word -on'o $4ok$d $otici%a'$s of faroff c$ntral 2frica known as 7h$ Dark

-ontin$nt th$ -on'o r$d stain was na%$d B-on'oC for %ark$tin'

purpos$s #y a ?$r%an t$til$ dy$stuff co%pany in 155!

Steensma %P: &Congo' Red. (ut o) A)rica* Arch. Pathol.+a.-ed.,2", "2, 2#2


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eversibility o% Amyloid

he de&osits are K irreversible.$.'. ;rncic 8. $t al: 2nti#ody %$diat$d r$solution ofli'ht chain , associat$d a%yloid d$posits. 2%..

athol.) &000) 1!)1&>G9*G Progression o% generalised amyloidosis

can be delayed or sto&&ed by treatmento% the underlying disease.8Hck$n -h. hak$sp$ar$ 2nn: atholo'y) dia'nosisand patho'$n$sis of 22 a%yloidosis. Iirchws 2rch. )&00&) **0) 111&&


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Prevention hera&y o% Amyloid

Prevention treatment o% theunderlying diseases

4accination against N am. &rotein in mice

diminished senile &laJue %ormation andim&roved memory. Jatur$ M$dicin$) &001)) 15th an.

A N @based e(&erimental thera&ies basedon degrading en9ymes. Kloko4ic $t al.:J$uro4ascular athways and 2lzh$i%$r 2%yloid +p$ptid$. =rain athol. ) &00!) 1!) 55>


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Fibrinoid Hyalin

disorders o% &rotein metabolism


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Fibrinoid Change o%

Collagen vessels and connective tissue damage

&lasmorrhagia -lea#age o% &lasma2

de&osits o% Ag)A: com&le(es

staining characteristics %ibrin ) li#e


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Hyaline change

!e%inition historical) d$scripti4$

intra) or e(tracellular change

o% homogenous rose B glassyD

a&&earancein the H; stained histological

sections


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Hyaline change

;(tracellular

cor&us albicans, scars, hyalinoses o%serous membranes

Intracellular

Croo#e cells, MalloryO hyaline,ussell bodies


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Ultrastructure Fibrinoid) collagen %ibres

surrounded by &lasma

&roteins may be reversible Hyalin@ collagen %ibres

increased in thic#ness,changed architecture rather

stable


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Hyaline change

;(tracellular

cor&us albicans, scars, hyalinoses o%serous membranes

Intracellular

Croo#e cells, MalloryO hyaline,ussell bodies


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Eigni%icance o% Fibrinoid

Change diminished Juality o% the collagen

- %irmness, &ermeability2

tendency to thrombosis in the

vessels, aneurysms %ormation


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Eigni%icance o% Hyalin

Change diminished Juality o% the

collagen - elasticity2

ischemia in organs 'ith

thic#ened arterial 'allsintracellular ) %unction, death

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