Logy Course 2

Cutsul

II

Cellular 8 extracelular lesions

Ifltracellul^r ecculrrhiions Thercare3typesdsubdanesidldedn ompoisG (ipids pd6'G aid (elnoi, s)

and endosenous p

9d6ic

6fte,Aosenous (n ieG sobdaicet or endoseno6 (Pdd lcad

bY abi om3L

lnrrlcellul.rr acct,mulations r

Factorciavodng intracellu af accum ulations:

!

endogsnous substane ls p.oduced n a normalamount butlhe rate of removalofce Ls is redlced (ial

r

endogenous subslance accumulales becauso lican nol be melabolized (defective eizyme) > storage disease D erogenous abnomalsubstance s deposled in lhe c€l becaue lhe cellhas no the capaciiy lo caffy it (accumulation ol cadon a.d siica)

iret rlrlrccllular rcr.urnullnorrs (dr.turbrnces in f^t metrbo|sm)

,

Liplds ln ihe body are otlhree categores 'ats /s'eaioss. obes'v. elc) choesteroland cholesterol este6 (ATS, etc) D phosphollpids-PL (sloragediseaset

a rgylendes o' leLUa

r

Steatosi; (frrttr ch.rnge) Slealos s means excessrve

hglycer des in parenchymal

.

!. ,

conlarn this metabolile Stealos s occure frequenly nlhe iver whch is lhe major organ invo v€d in lipid metaboism but aso n kidney. heart and mls.le

srs

i.( |irn.gdlls

!-.

I{epatic latty change

/

hepatic steatosis

i.l '..,'".-:..

.. se r r!

anrusGs

srttrB dlr

f

Cholesterol and cholestelol esters

" "

Manv cells are uslrg the cho eslerclior synihesis ofcell menibranes without prcducifg an intracellular accumllalion of cholesierol (CT) and cholesiefol esle6. lnkacellular accumu aiion ofCTls presentrn some

p. 'ooqrca, pro.esses, rs ma.r'esleo b) nslologc?l Intracyioplasmic vacuoles (foamy or €.thic

-l

Cholcsrcroi and cholcsrerol esrcr

r.cLn rlrtron

Accumulat on of CT n phagocyl c ce s - madophages are load€d wrh tui .raitri c.ers lcelswlh E

g

r

slrawberry !a lbLadder sk'n: &nlelasma (at ntema

in hypei pidem c states - Hypenlpldaemic heredtary of acq! red syndrornes

.nse

eye)

t,11,

5rr.s\berry \csrcLe or L

n-meor ,"ioic; t !:o r'" {. b:*Eoundoilhe onqet ra bi:D m

Strawbctq, gallbaddcr

congesied nucosaL s!dace Cholesterolosis resu ls lrcm excesslve cholesierol lrom

focal co lecllons ofroamy

Xanthonras

connective lissue at the

F.rmrhal Irr pcrlrplde mi.r

l.iirr1r Neph.olic

./:, li,r,,.11. '' , . - i-; I sd..*

lhrough

.eabsoblion at lhe evel

(6os nop-hilc)

-

iito cell

olasma @ls. wiich rema n in RER with fomation of

;: i.{ 'r!,.i.Jlt,.; '. "ir l'.i

Glycosen smorerydenl nthe

;

metabol sm of alucose glycosen a@umu alon

i' nuces

o*scer).;id

i

i'r'; t:

'^.-:-t'i !iGLti."lin'l s

. .

endoseioG (s,nihenzed inhe bodn or a ooenoB (brclshLf'dm od'id?orhe bodri

*

Exogenolrs prgmenls

Anthracosrs

macropnages !n neavy

Gfossly, in smoking the lungs wiLl g€t b acker and Anlhracosls is the lerm ls€d to describe b ack

r

Endogenous pigments

Car-boo

'l'etoo Profess ona taltooeE use

ox'de or even heratoiyL

i)

'

Lipofuscin Lipotuscin r€Pre*nts the Pesence of ry6Gom$ that h4eadcumukted indiqesrrbre pds appeadns as

yelld

l\licrcspida lY, it appeac as a perinucd6ne broM (ru$us) piqment. Tris is a prciem nenl iniEcylop asm o pismenl nto hepalooyt€s and erd dyoo$es r^ro @r s at lhe e deiy !.d swere

Hemosidcrin pigrnent Hem osided n is an endoqenoug

result ns irom the deo€dat on

i.

Hemcidedn is a fom ol ircn McrcsoprcaLy, in FlE staining

Flemositletosrs excessive accumulatron

Local Hemosidetosrs .onditonsol chonicstasis e s

r

aveoa, ma(ootuges conra n nq a

!

b6M pgme nE

al lhe Pe(Phery or beed nsi e q

blueq€en pigm€nr Pers

Hemosidenn The brown granu ar matera in a veolar macrophages is accumLlated asa resu I or

In hepalocytes hemosdern accumlLation s slaLftd m blue using a speclalslaiiLnq

Biluubir pigmcnr B lnubln is an

endog€nous

Esulting from degBdatLon

ex@ss ve accumulat'on ol

clinica ly manifesled bY an ye low colorofsclerae and

Xlorphol o-tl-

j.ru

rr.Lcc

In cterus skin scerae and o€ans are geen sh-yelow Ye low

coloriums in green

Th€re are rema n unsbLned

lr,lacroscop ca Ly the

llveris

,\cc,.roruLatioo

of L, i:1. pigL,rcrrL:

M'os@p'.a|iy'he|eare2

drahepalocyt-A granura'

\ll

lhslypeof]:lndcalhedomjmnt

EliTRACLl.l,aILrl}.. Aricil

{

::l,ij:.**'"-

illl".li;il ":,"^-'"" .;;.,;""

e

LelU LrL'1

i1]l 'l3

Hyalinosis

.

Hvalinosis represenls a

abromar acc*.r . a,io- o' nyaln

Amyloidosis Amvooocisischaracie-zed by an e(racellular accumulatjon of amyLoid (nbrillar abnormal proieins) n iissues and organs in a vad,Aiy

ofconditions

TCi'Itc

It

I \t

Fyaln is a descriplve le.m thal describes an a tered -Anracellular prote n. which has an u.iform appea €nce

ICH-Hyalnoss represenls accumulalion oi hya in. ECH Hya nos s repres€nts a ussu ar accumllation oi

Protein accumulation (hyalinosis)

* \.'i

Ar\{\'LOID

r

O SIS

ssles arro oqans

rn

a

reacuon wrih starah. hen@

AIII\LOIDOSIS [rlcrcsftpi€lry by

v rchotr reaclion

n HE sra n ng

amyroiddeposits 3€ 3tained n ihe sameuY as hya n i Pink (bY

b.own red,lhatlums ln

I'hyslcal structure or amylorq .

i;

EM ihe afryLo d E iep€sded by lrr ck unb.ancfred I amedts

onla- rro.'s "spo-
' ! .

Amylodosis diaqnoss s made moDholosica v bv dentryns

amyod biopsy specmens !c \ed od ^ opa lr.os.oor.-519.c'odoqdl'gs ous $snoph Lc nva tr si,6shrc€ that noroorc$veaEumrralon sprc,lrinsan

the

atroplry orcompression 0n adla@ni ce s To d fteGoiiare the amyi oid lmh olher rrya n .jepog ts (e q

...d a..iF/or' .i0.. 1 !e b " o-e lhemo'l used be nq lrtconqo Re r slains lhe amyloid n bdckied in l!1o !ecl!! gltyEqsqeu q,{!.ngq9q?! polari49qlslt

"

9

Chcmical !irucRIre of amlloid Chemical structure of an Yloid: , 95% of the mateial consisis of amyloid librillar

,

1.

lhe remaining 5% is represented bv the P component (glycoprotein - seic amyloid)

Amyloid fbrillar Ptotetn s oi amyloid P@teins

r'"xix".?L:il*",i"":1il?:","

5de,

d

romp6ma.e slmmu^(kl

)n 3

'. ,';rT

'

I :.."*r'f:;1""". i": o

""'*td"

3rbrllar

!irqf pdei

nofrE

*-' * *r:: "5";' Jir

; 'v;l:x':11.'"""'i':..""'3 " "

flfii::t;;:"?.ii i:!is,

"il

'{+J'*$}:i'+li"ii:i{r

$m"i"s},ffi *jf:,1iil1'.r;r.f o z.Lmd,r a^'.a *ru. Fde"

c. .!o- ). I ,rlgTra o o:

"

rc_n

2. P Component P componeni is a glycoprctein

. .

disilncl€d byiibdLs of amio d.

lscoselyassoqaGdwithflbnsof anilodinallfomsor

ha6 a similar sltucture wilh C-Eacli@ p@ie n.

serum componeni (PAS +) has affnity ior amioid riber and may

be r€quiredtor

is an lselllprcrein ln IMH deiect n9 orthe amyloid in

amyloldosis

.

isro4nary heamybd

s

' I.1

r...;: it.r..t--\i .;

,.tl$,:;ir:ilJ';

Renal amyloidosis

t_tlcpaIc

--

lm)1o1dosl\

C.rr.lirc arlvloiJo:rs Emoe.onirci

n

"10tie-d nk,.-o /ed!eaf! I aso_rcv'o

eBiemtr se^'lafllLodosis

Lhe

i'{o.o, /.. :in b0 omosc 5r"l 6 deoosd oaee"i ui dndudLno neiem cadf;mvodcs Gn mer*r4 .au5e

condlcr

s.oA oo

at

t'd in'"ri.

ondisl!brn

oai, €d4df/'om\na € ed€sr"4ryo€ofcvP oa" 'mpai€d

..\i,,n: .:r!r,L h\ .il.,Nnr ir((l,,,llri: ,ls({!rLr.rr Paihological crlcincadon Cacilicationdiseasemeansabnormalslo€georcacum There are 2 types of pathological calcification

r r

DystroPhiccalcificatio.

e

occure ld€ny in nonllabLe ordead lssues

r

in ihe abse^ce of d

studan€ in ca metabo sh

Metastatic calcificalion

! ! .

@cu6 in loca v ab e lissues in €se ol nc€ased *rum concentalonsorca nihe pr*ence of ab^o rmaL melabolism or ca

:

Dr sttophic calcifi cation

.

Dysirophic calcincaton rnay occuf in dead tissues n case of normal calcaemia

r ! r J

Dyslroph c calcfication 6 s€en in areasofnecrosis ol d fterenl types:coagulal on nec Ca I

dtcal on

asooccu6

n card ac

atered valves

whatever lhe p ace of slorage ca cufr salts appear as white linegEnules ofien ooklnq cretareols

--

:-

s a so present n alheromas of advanced

-.

.

lYletllstatlc ctlcrlrcatron -

' .

N4elastat c calcification may occur in normal tissues in case of hypercalcaern a.

There are 4 major causes of hypercaLcaem a: ! incrcased secEtionolparalhyrcd homone wlha consequenl bone €sorbton. as n parathyroid tumors

!

r

bone tssue damagelhatoccurs in pdmarytumors bone o dfuqF .Let.rat a"rr.td.", b ede.

.9,

ol

"1("-

dsorders realed iovllamn D which ncludes poisoninq

d re.alfailure,

causing €tention ofphosphate which leads to secondary hypepaGthyro dism

Nletastatic ca1cifi cation - NIorPholog,Y E Locallons metaslatccalclicaton occurs anvlvhere nthe body but affecls mai.ly irtersutialt ssues oflhe gaski. mucosa kidney, pllmonary syslemc aneres and

. A lhese iissleswilh difiereni localation lost acldsand

thus became an aLkali.e afea predlsposing to metaslat c

Morphologically

' e

nd

llese rocl oa)

srmilar to thoF descfbed In dysboph c.a clfication Thus, they aPpear as arnorphous non_cryslalline deposits or in the form ofcrystallline deposils GeneralLy, mineral salt deposils are nol causlng clin ca dvsf .1c-or o'roadq:olallr ri,la)s re opoo" ls il ri,/ ca,.- espratof the kidnev herroca c Fos slmay cause renal dysi!6c1 on-

o-c..r'l.

r,1L"d'r. r '.. ,r^dnl!1 ."1'r'n<..- r'r'r

l ( Moickeberq :derosceros

t

C.rlcitlc.l r rh-cs

PethoLogical rissue calciEcations

Prrh,,loe,crl u :ue c-rl.itrc ro ls

PrthologLcrl rissuc t'icrfi c:titurs

calc!!m

sa ts stain dark

blue on H&E.

specialstains lke the Von Kossa ls applied

i