kuliah-anemia-dr-bastiana-sppk.pptx

8/9/2019 kuliah-anemia-dr-bastiana-sppk.pptx

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Gangguan Eritrosit:

Anemiadr. Bastiana SpPK



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www.themegallery.com Company Logo

Gangguan Eritrosit

Anemia

Polisitemia



LOGOANEMIA

Definisi Anemia: Sindroma klinis yang disebabkan penurunan m

assaeritrosit total dalam tubuh.

Keadaan dimana massa eritrosit dan atau massahemoglobin tidak dapat memenuhi ungsinya untuk

menyediakan oksigen bagi !aringan tubuh

Penurunan di ba"ah normal kadar #b$ hitungeritrosit$ dan hematokrit




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ANEMIA

Penurunan Hb dan Hct :

< batas bawah 95% interval referens

dari kelompok usia, jenis kelamin

dan lokasi geografis ketinggian!

Hb<"#$" g&dl ' Hct <()$"%!,

Hb<*g&dl s+mptom !

-kut: hipovolumia pucat,

ggn penglihatan, syncope, tachycardia! '

.ronis: tissue h+po/ia fati0ue, d+spnea,

Headache, angina!

Anemia



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5

ANEMIA % symptoms & syndrom

e

#b '

P() ' #ypo*ia % Otak $ Otot

+,( '

Kompensasi -

heart rate /% ta0hy0ardia % lo" rate / %0ardiomegaly % heart ailure % 1

blood lo" priority 2pallor3

+,( 4$56PG 0ontent /% O4 disso0.0ur7eshit to the right % O4 release to thetissues / .



LOGOKlasifiasi Anemia

,erdasarkan patoisiologi-

I. Kegagalan produksi sel darah merah-

A. Gangguan sel induk hematopoesis

Anemia Aplastik

,. Gangguan sintesis 6NA Anemia Megaloblastik

(. Gangguan sintesis #emoglobin 2#b3

Anemia 6eisiensi ,esi$ 8halasemia

6. Gangguan sintesis eritropoetin

Anemia karena GGK




LOGOLan!utan9..anemia berdasarkan patoisiologi

E. Gangguan karena mekanisme lain-

Anemia karena penyakit kronis$

anemia sideroblastik

Anemia karena iniltrasi sumsum tulang

II. Peningkatan destruksi sel darah merah-

Anemia #emolitik

III. Kehilangan darah 2,lood Loss3

Anemia karena perdarahan akut




LOGOAnemia

Anemia berdasarkan morologiAnemia se0. morologi eritrosit$ dilihat dari-

ukuran dan "arna di ba"ah mikroskop atau

indeks eritrosit 2M()$ M(#$ dan M(#(3

Kriteria :kuran 2si;e3- Normositik$ Mikrositik$

Makrositik Kriteria <arna 2pu0at3- Normokromik$

#ipokromik




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(ara Mengetahui :kuran eritrosit-

= membandingkan dengan inti sel limosit ke0il 2di

ba"ah mikroskop3 - % ukuran sama > normositik lebih ke0il > mikrositik

lebih besar > makrositik

= Menghitung M() 2Mean (ell )olume3 M()> P()&Ery ? @ 2L3 2@ L>@@4L> @Bm53

N - de"asa > C@ L $ di ba"ah @ thn > D C L

M() - normositik $ mikrositik$ makrositik = Eritrosit dengan 7ariasi ukuran yang abnormal

anisositosis



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,andingkan ukuran sel eritrosit dengan inti limosit



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Perhatikan <arna sel eritrosit -

,andingkan diameter 0entral pallor2(P3

dengan diameter sel eritrosit tersebut .

Normal$ bentuk sel eritrosit adalah seperti 0akrambikonka 2biconcave disk 3 %

pada hapusan darah tepi terlihat bulat$ F DC Bdengan area 0entral pallor di bagian tengah

(P @&5 F Eri > normokromik(PH F Eri > hipokromik



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Eritrosit dengan central palor (CP)

1andingkan diameter 2P dengan diameter sel eritrosit



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<arna$ dapat diketahui !uga dari M(# 2Mean (ell #b3

M(#> #b&+,( * @ 2pg36e"asa- M(#>4D54 pg$ Anakanak- M(#>455@ pg

2@pg>@@4g>@BBg3

M(# normal % normokromik M(# J normal % hipokromik

M(#( 2Mean (ell #b (on0entration3 -

M(#(>#b&P() * @ 2g&dL3Normal- M(#( > 545 g&dL



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Klasifiasi Anemia se!ara morfologi

"3 Anemia #ipokromikMikrositik3

#3Anemia NormokromikNormositik

(3Anemia Makrositik



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"

2ontoh:

$ -nemia

defisiensi 4e- halasemia- -nemia akibat

Pen+akit .ronik- -nemia

sideroblastik

#Contoh:- Anemia pasca perdarahan akt-

Anemia aplastik- Anemia hemolitik- Anemia aki!atpenyakit kronik

- Anemia pada""#

- Anemia pada mielo$i!rosis

- dll

(A. %egalo!lastik&

contoh: ' Anemia de$isiensi

(olat& ' Anemia de$isiensi)itamin *12

*. +onmegalo!lastik contoh: ' Anemia pd peny.

,ati kronis ' Anemia pd

hipotiroid& dll

627 <8 fl'

62H <#* pg

627 8 $95 fl

62H #*$( pg627 95 fl

-nemia

hipokromik$

mikrositik

-nemia

normokromik$

normositik

-nemia makrositik



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1-

"iporomi#Mirositi



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1

;ormokronik$normositik



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19

makrosito7al

2Anemia megaloblastik ditandai oleh makrosit o7al ini3

6akrositik



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Pendekatan diagnostik Anemia-

Anamnesis:onset $%leeding tenden!& $ routinemedi!inal $ o!!upation $ 'o%%& $ tra(el'istor& $ famil& $ diet $ GI s&mptoms $menstruation !&!le $ 'istor& of pre(iouspregnan!&#deli(er& $ al!o'ol !onsumption )et!

Pemerisaan fisi :!on*un!ti(a + lips ,pallor- $ mout'

,!'eilosis- $ tongue ,glossitis- $ gum $ nails,oilon&!'ia- ) 'air ,signa de %andera)alope!ia- ) *aundi!e ) pete!'iae ) li(er +spleen ) l&mp'enodes )re!tal $ (aginaltou!'er ) feet ,ul!er)art'ritis-



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Pemerisaan La%oratorium

(,( 20omplete blood 0ount 3% to 0onir manemia 2#b$ P()$ +,(3 the type o anemia

2M() M(# M(#(3$ +6<

+eti0ulo0yte 0ount % rele0ts marro"s responses .

P,S - to look or the +,(s shape and any abnormalities o

+,(s besides the other blood 0ell lines

Iron status 2 Serum Iron $8I,($ 8ranserrin

saturation $ Iron storage 3

,lood 0hemistry 2 dire0t&total bilirubin$L6#

and stool e*amination or o00ult blood test $ et03 .

PBS: Pheripheral blood smear



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+adiologi0al e*aminations 2 (hest ?ray$

:SG $ M+I 3 (ardiologi0al e*aminations 2EKG$8readmill$

E0ho0ardiography3

Notes -

irst 0onirm Anemia 2 #b $ P() $ +,( 3

(lassiy the anemia 2M()$ M(#$ M(#(3

(auses o anemia

anjutan=3 Pendekatan >oagnostik=



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Anemia #ipokromikMikrositik

Setiap kondisi yang menimbulkan gangguan

sintesis #b gambaran hipokromikmikrositik

Anemia 6eisiensi ,esi penyebab tersering

dari anemia #ipokromikMikrositik Perhatikan penyebab lain 266>di diagnosis3

sebelum mendiagnosis Anemia de. besi$ spt-

anemia akibat penyakit kronis

8halasemia

anemia Sideroblastik$ dll



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2/



LOGOANEMIA 6EISIENSI ,ESI

6einisi-

Anemia yang timbul akibat kosongnya 0adangan besitubuh besi utk eritropoeisis pembentukan #b

Anemia de. e$ ditandai dgn-

anemia hipokromik mikrositik

besi serum

8I,( 28otal Iron ,inding (apa0ity3

Saturasi transerin

eritin serum

Penge0atan ,esi sumsum tulang negati

+espon terhadap pengobatan dengan preparat e




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t P % % ,Eti l i-



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29

ator Pen&e%a% ,Etiologi-

I. Keseimbangan negati e 2Negati7e Ironbalan0e3- Asupan e '

inade0uate diet , impaired absorption!

e loss /2GI bleeding$ e*0essi7e menstrual lo"$

bleeding diathesis3

/ demands 2inan0y$ pregnan0y$ la0tation3



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II. InadeQuate presentation to erythroidpre0ursors- atranserrinemia Anti 8r+ Ab

III. Abnormal e balan0e - A0eruloplasminemia Autosomal dominant hemo0hromatosis 2 mutations in erroportin 3

anjutan=34aktor Pen+ebab



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Patogenesis desifisiensi e

/ pat'ogeneti! fa!tors:

Impaired #b synthesis 20onseQuen0e o

redu0ed e supply3

8ranserin saturationJ @inadeQuate esupply tomarro" % #b 0ontents o +,( ' % hypo0hromi0

mi0ro0ytosis

# Generali;ed dee0t in 0ellular prolieration

edei0ient % o*idati7e damage to the red

0ells membrane % +,( deormability ' % +,(7iability '% +,( destru0tion / espe0ially in spleen% redu0ed +,( sur7i7al



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Status %esi tu%u':

Serum Iron > SI

8otal Iron ,inding (apa0ity 28I,(3 8ranserrin Saturation > SI&8I,(*@ Simpanan besi 2Iron storage3-

#emosiderin %produk degradasi eritin yang tidaklarut dalam air % mayoritas tdd aggregat kristal

erri0 o*yhydro*ide$ eOO# 2di #epar danSutul%dideteksi dengan biopsi&aspirasi dan penge0atanbesi 2prosedur in7asi3

erritin % kompleks garam e5Rdan apoeritin

yang larut dalam air$ dengan !umlah yang sangatke0il di serum.2dideteksi dengan metode imunoasai3



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Kandungan besi tubuh > 5 mg&kg,,-

TC e ungsional$ sebagai heme-Iron

2 #b$ myoglobin$ en;im

heme - 0yto0hrom($A$A5$,$

0atalase $ pero*idase3

Nonhemee 2sebagian ke0il3

4 simpanan besi & Iron storage 2erritin$

hemosiderin3

hanya T @ pada "anita .4 circulating 2terikat pada8ranserrin3



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Iron (y0le in the body -

ediet % as hemee 2#b$ myoglobin$

en;ymee3$ 5 adsorbedrom animal&meat sour0es $

adsorbed easily . % as nonhemee 27egetables $

legumes3$ U o diete butonly 44 o it absorbed %depends on the ironstatus and

the ratio o Enhan0er-Inhibitor



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Enhancers 2;at yang menstimulasi

penyerapan 2absorbsi3 - As0orbate$ (ytrate$ organi0 a0ids & otheramino a0ids $ by redu0ing e5R to e4R.

Inhibitors 2;at yang menghambat absorbsi3 -

(arbonate$ Phytate$ 8annins$ Phosphate$O*alat 0helate Nonhemee %

unabsorbable



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,ahan makanan yang menghambat absorbsibesi non heme 2Non-heme Iron3 -

Phytate 2dari legumes$ sayuran3

8annin Polyphenol 2dari teh$ kopi$ "ine$0oklat 3

Phosphate&phosphoprotein dari kuningtelur

Minerals 2(a$ Vn$ (d3

8etra0y0line yang bereaksi dengan e %

menghambat absorbsi



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3-

Silus e dalam tu%u' :

6iets Iron % duodenum & pro*imal !e!unum .

Iron rom gut % released into 0ir0ulation $

bound to transerin % distributed to bodysorgan & tissues2 to bone marro" as a part oheme & #b 3 % 0ir0ulate inside red blood 0ells"ith blood lo"

0' d l t f IDA



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3

0'e de(elopment of IDA

? Stage@ 2prelatent edei0ient3-

progressi7e loss o storagee bodys e reser7e is still sui0ient to

maintain both the transport and un0tional0ompartment $ so +,( de7elopment isstill normal .

peripheral blood pi0ture is normal $ nosymptoms o anemia $ but erritin is ' .

=I6A> Iron 6ei0ien0y Anemia



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= Stage4 2latent edei0ient3

E*haustion o storagee $ +,(produ0tion is still normal $ erritin ''

(ir0ulatinge 2SI3 begin ' $ 8rans +e0eptor / .

= Stage5 2e6ei0ien0y Anemia3

Stadium o Iron 6ei0ien0y Anemia



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Stage@

2prelatent3

Stage4

2latent3

Stage5

2I6A3

6arrow

4erritin

ransf$@atsrfA

Aetic Hbcontent

Hb627

@+mptoms

B

B

;;

;

;;

fatigue

$ !

<"#ug&

<")%C

B

;;

fatigue

$ !

<"#ug&

<")%C

B

<<

pallor



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Symptoms Morphology SI 8I,( erritin

D > - -nemia H+po E6icro

@DB $D12 C

BB

-32 > -nemia H+po E6icro

@D '↓

*C

+↓

;& C

Pendekatan 6iagnostik Anemia 6eisiensi

e



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Pendekatan 6iagnostik Anemia 6eisiensi e

@. Anamnesis W pola menstruasi$ kehamilan &persalinan$ tendensi perdarahan$penyakit kronis$ diet$ peker!aan$

ri"ayat bepergian

4. Pemeriksaan isik W sistematik dari seluruhpermukaan tubuh sampai ke organ dalam 2 hati$limpa$ kelen!ar getah bening 2lmphnodes3



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5. Laboratorium #ema 26L$ LE6$ #apusan

darah tepi$ +etikulosit3

Serum 2SI$8I,($erritin$ ,ilirubin3

,MA 2,one Marro" Aspiration3

Pemeriksaan :rine dan tin!a

X. Penun!ang +adiology 2EKG$ :SG3

Endos0opy



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S I 8I,(

Normal ; "&( mol3rsf!

;

I 6 A ↓ C

An.o (hroni06isease

↓ ; & ↓

e O7erload CC ; & ↑

Pemeriksaan Lab Anemia de e



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Pemeriksaan Lab. Anemia de. e

@. (,( W 0onirm Anemia ind hypo0hromi0mi0ro0yti0 pi0ture rom ,SE and +ed(ells Indi0es 2 #b$ P() $M() $ M(# $M(#(3

4. SI W e4R

released rom 8ranserrin R erro;ine20hromagen3 % measured 0olored0omple*

8I,( W serum R e*0ess e(l4 % to ill all 8ranserrin binding sites % the e*0ess e is i*ed by Mg

0arbonate % esaturated 8ranserrin ismeasured "ith erro;ine 2> 8I,(3



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Saturasi 8ranserrin > SI&8I,( ? @

Erythropoeisis impaired "hen 8.Sat J @

5. erritin Serum -

Serum erritin le7el Y estorage

erritin J@ ug&L % 6einiti7e e6ei0ient N&/ erritin in I6A $ i -

impaired li7er un0tion 2 damagedhepato0yte3$

hemolysis$ inlammation & ine0tion &malignan0y 2 erritin > acute-phase protein 3

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4-

X. 8ranserrin Serum -

measured by immunodiusion methode

Normal 7alue - 4X g&L

. ,one Marro"s Aspirate e7aluation -2 using Perls or Prussian ,lue stain 3

LOGOAnemia o (hroni0 Infe!tion



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4

Anemia o (hroni0 Infe!tion

Ge*ala linis miripdengan anemia def.e

Gam%aran la%. 'ematologi 1 Anemia def. e,An."&po#Mi!ro) M23 ) M2" ) SI - ) tapi↓ ↓ ↓

0IB2 N$ and erritin N$ -↓ ↑

Pat'ogenesis :

e storage $$ 0ransferrin→

0issues $ 4ES

LOGOPenyebab menurunnya Z0ir0ulating



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@. Impairment o e release romma0rophage in 0ompeting "ithla0toerrin$ phago0ytes produ0t $ e7enstoragee is still enough .

4. InadeQuate EPO +espons to"ardsanemia 2ee0ts o 0ytokine produ0tion by

ma0rophage3 .

Penyebab menurunnya 0ir0ulatinge :

LOGO6i i A i kib t kit k

i



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50

6iagnosis Anemia akibat penyakit kr onis-

lab hematologi-

Anemia hipokromik mikrositik SI ' $ 8I,( '&N $ erritin N&/ 2 !ika erritin '$An. 6e.e 3 Inlamasi & ineksi 2R3 -

(+P and LE6 /

Problem- I6A "ith inlammation % erritin /2alsely diagnosed as A(63 it 0an be

dierentiated by s8+ e*am 2serumtranserrin re0eptor3 that / in I6A but normalin A(6 .

LOGOAnemia Sideroblastik



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51

Anemia Sideroblastik

6eek pada sintesis #eme % akumulasi e dimito0hondria % degenerasi e % granula edi sekitar inti normoblast$ membentukstruktur spt 0in0in [paling !elas terlihatdengan penge0atan Perl 2Perls stain3 \ %

!inged Sideroblast 2karakteristik anemiaSideroblastik3

Sideroblast bisa di!umpai se0ara normal disutul

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52

Sideroblast and +inged Sideroblast 2 in

Sideroblasti0 Anemia 3

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53

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54

2lassifi!ation of Sidero%lasti!

Anemia

@. #ereditary : F$linked, defect in heme$

s+nthesis enG+me pathwa+

4e absorption C % of ransferrin

saturation and 4erritin level C

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55

4. A0Quired -

Primary -

Stem 0ell 0lonal mutations2M6S >

"elo#splastic Sndromes $ !%-!S 3Normo0hromi0ma0ro0yti0 anemia .

Marro" - erythroid hyperplasia "ith

dysplasti0 or megaloblasti0 appearan0e

ringed sideroblast in normoblast .

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Se0ondaryAbnormal metabolism o )it., 2al0oholism$malabsorption3 $ impairment o hemesynthesis 2 Pb into*i0ation3 $ +hematoid

Arthritis $ or An.megaloblastik .

:sually related to myeloprolierati7ediseases 2 AML$ Myeloibrosis$ Poly0ythemia

or another types o M6S 3

LOGOMa0ro0yti0 Anemia



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5-

Ma0ro0yti0 Anemia

NonMegaloblasti0 Ma0ro0yti0 Anemia -

+eti0ulo0ytosis

Li7er disease & Al0oholism

Myelodysplasti0 Syndrome

Erythroleukemia 2A,M3

Megaloblasti0 Ma0ro0yti0 Anemia

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5

ma0ro0yte > erythro0yte "ith M() H normal . ma0ro0yte&mi0ro0yte depend on the balan0e

bet"een nu0lei 0ytoplasmi0 maturation .

2nu0lear di7iding stopped "hen intra0ellular #bprodu0tion rea0h a proper le7el 3 .

I nu0lear maturation delayed 2 in 6NAsynthesiss dee0t 3 or 0ytoplasmi0 maturation /2 in0rease o EPOs a0ti7ities 3 % 0riti0al le7el o

#b a0hie7ed earlier % Ma0ro0yte


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59

Megaloblast > bigger than normal

normoblast .Megaloblasti0 0hanges > in0reased si;e ohemopoieti0 pre0ursor 0ells in bone marro"2 not only in normoblast 3

Primary dee0t - 6ee0t o 6NA synthesis2 altered almost all a0ti7e 0ells & organs i.e -hemopoieti0 tissue$ epithelial 0ells $ mu0ous0ells$ et0 3

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Etiology o 6NA synthesis dee0t -

dei0ien0y o 7it.,@4 and oli0 a0id %maturation dysharmony bet"een nu0lei

0ytoplasm 2delayed nu0lei maturation3 %in0reased 0els 2megaloblasti0 0hanges3 %marro"s inee0ti7e erythropoiesis %intramedullary hemolysis % total&indire0t

,ili and L6# /.

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6ei0ien0y o oli0 a0id-

InadeQuate diet2intake J & demand / in pregnan0y

la0tation $ 0hilds gro"th & malabsorption

in tropi0al sprue & bo"el rese0tion & smallintestine inlammation 3

6rugs ee0t 2antiepilepsi3

A loss / 2dialysis3

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6ei0ien0y o oli0 a0id-

InadeQuate diet2intake J & demand / in pregnan0y

la0tation $ 0hilds gro"th & malabsorption

in tropi0al sprue & bo"el rese0tion & smallintestine inlammation 3

6rugs ee0t 2antiepilepsi3

A loss / 2dialysis3

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6ei0ien0y o )it.,@4-

InadeQuate diet -Intake J in 7egetarians $ demand / $impaired absorption 0aused by

de0reased Intrinsi0 a0tor2 gastre0tomy $ perni0ious anemia 3

Malabsorption 2bo"el ine0tion $ "orms & blind loop syndr 3

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)I8AMIN ,@4 ASAM OLA8



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/4

$4ood from animal products$Heat stabile

$@torage : enough for ( +rs$Aelativel+ low needs onl+"% of folate re0uirements!

$imited sources vegetable ,

fruits!$Heat labile$@torage enough onl+ for (mths$Higher folate needs

(A:SE O 6EI(IEN(] (A:SE O 6EI(IEN(]

$7egetarian seldom!$Dmpaired Dntrinsic 4actor

pernicious anemia!$Iastrectom+$-tropic Iastritis$-nticonvulsant, alcoholism

$;utrition alcoholism, goatJsmilk diet!

$Prematurit+$Hemod+alisis$1owel resection$Pregnanc+$-nticonvulsant , 6F

LOGOPathogenesis o Megaloblasti0 Anem

ia -



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g g

Megaloblasti0 0hanges

atrophy o tongue papilla mu0osal GI %glossitis $ gastritis$ nausea $ 0onstipation.

,@4 dei0 % demyelinisation o spinal 0ord

peripheral ner7e % loss o oots balan0e &sensory 2Neuropatia3

A dei0 % hyperhomo0ysteinemia %thrombosis and 7as0ular o00lusion .

LOGOB56 Meta%olism



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//

B56 Meta%olism

)it.,@4 % purine pyrimidin synthesis %

synthesis 6NA +NA % mitosis andmaturation

)it.,@4 made rom mi0robiologi0al sour0e

be0ause plants do not produ0e ,@4 2 meat $li7er$ eggs and milk are ri0h o )it ,@4 3.

)it.,@4 0ontent in the daily diet is 5ug $daily reQuirement o ,@4 is @5 ug$ and ,@4bodys storage is 4 mg 2enough or 5 yrs3

LOGO)it ,@4 absorption



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/-

)it.,@4 absorption

,@4 diet % in gaster bind by I 2Intrinsi0 a0tor 3

produ0ed by parietal 0ells % I,@4 0omple* %ileum - ,@4 absorbed $ I reed into the lumen

impaired I - gastre0tomy&gastritis& AutoAbantiI orAutoAbantiparietal3 % no absorption o ,@4 %

impaired 6NA synthesis % 2Perni0ious Anemia"ith A0hlorhydria3

Perni0ious Anemia > autoimmune disease % auto

Ab to parietal 0ells 2AntiI or AntiParietal3

LOGO#ematologi0al pi0tures o Megaloblasti0 An

emia



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#ematologi0al pi0tures o Megaloblasti0 Anemia

,one Marro" -

megaloblastosis

inee0ti7e erythropoiesis

Peripheral blood - O7al ma0ro0ytosis

#ypersegmented neutrophil 2 i7e lobed

0ells or one lobed 0ell3 or the mean lobeso @ neutrophils is H 5.X

LOGOMegaloblasti0 Anemia



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g

find oval$6acroc+te cell and h+persegmenteneutrophil 3

LOGO6iagnosis o Megaloblasti0 Anemia



-0

g g

S0reening -

(,( $ Neutrophils lobe 0ount Serum Indire0t ,ilirubin $ L6# 2la0tate

dehydrogenase3

Spesii0 tests -# ,one Marro" Aspiration- megaloblastosis megaloblasti0 0hanges$ erythropoieti0 a0ti7itiy / 2 inee0ti7eerythropoiesis3

olate )it.,@4 assay Gastri0 !ui0e analysis S0hilling 8ests Antibody Assay

LOGOAnemia #emolitik



Anemia #emolitik

Anemia hemolitik- anemia yang disebabkan

oleh proses hemolitik. #emolisis- peme0ahan eritrosit sebelum

"aktunya 2sebelum masa hidup rerata eritrosit$yaitu @4 hari3.

2Proses peme0ahan eri karena sdh "aktunya

senes0en0e>penuaan3

#emolisis dapat ter!adi di dalam pembuluh

darah 2hemolisis intra7askular3 dan di luarpembuluh darah 2hemolisis ekstra7askular3.


LOGO#EMOL]8I( ANEMIA



-2

#EMOL]8I( ANEMIA

Normal red 0ells sur7i7al > @@@4 days %

destru0ted by ma0rophage in marro" andspleen .<hen the sur7i7al are shortened % EPOprodu0tion is stimulated 20ompensated3 %

no #b 0hanges % anemia 2W3 .I the destru0tion is a0ute or 0hroni0 "ith

7ery shortened lie o red 0ells $ there "ill no0ompensation % anemia 2R3 .

LOGO6einition o #emolyti0 Anemia :



-3

6einition o #emolyti0 Anemia :

anemia 0aused by shortened red 0ells

sur7i7al as a result o e*0essi7eun0ompensated destru0tion o red 0ells .

#emolyti0 pro0ess > e7ery pro0ess o red0ells destru0tion "ith still & "ithout0ompensated by bone marro" % anemia isnot al"ays present .

LOGO# (ompensation ability o bone marr

o" -



-4

(ompensation ability o bone marr o" -

Ability to / red 0ells produ0tion 2 C * normal

3 - sur7i7al shorten % produ0tion / 4* sur7i7al shorten ^ % produ0tion / X* sur7i7al shorten @& % produ0tion / *

sur7i7al shorten @&C % produ0tion / C*

/ o produ0tion C * is maksimum .I red 0ells li7e only 4 days % anemia 2R3.

LOGO6iagnosti0 approa0h in #emolyti0



-5

nemia -@. (onirm anemia 2#b&P()&+,(3

an a0ute 0ase usually a0Quired $ and0hroni0 0ase is mostly hereditary .

4. 8o ind the signs o hemolyti0 pro0ess .

5. E*tra or Intra7as0ular _

X. #ereditary or a0Quired _

. 8he 0ause o hemolysis episodes .

LOGO8he signs o #emolyti0 pro0ess -



-/

8he signs o #emolyti0 pro0ess -

@. In0reased o red 0ells destru0tion

:n0on!ug.bilirubin serum / % !aundi0e :robilinogenuria #buria % sign o intra7as0ular hemolysis Abdom.pain % splenomegaly$ spleen inar0tion

Legs :l0er % intrinsi0 dee0t o erythro0yte #aptoglobin serum ''&neg % intra7as0ular

hemolisys .

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--

4.6estruksi eritrosit -

Mi0rosphero0yte$ ragmento0yte$ Poikilo0yte Erythro0yte Osmoti0 ragility /

Positi7e Autohemolysis test

Shortened o red 0ells sur7i7al

5. 8anda Peningkatan Eritropoisis-

+eti0ulo0ytosis

Normoblastosis

Erythropoieti0 #yperplasia in bone marro"

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-

LOGO



-9

LOGO



0

LOGO



1

LOGO

Hemolisis Kkstra vaskular



#emolisis ekstra7askular lebih sering di!umpaidibandingkan hemolisis intra7askular

#emolisis ter!adi di sel makroag dari sistemretikuloendothelial 2+ES3 terutama pada Lien$ hepardan sutul karena sel ini mengandung en;im hemeoksigenase

Lisis ter!adi karena kerusakan membran eritrosit2misal Akibat reaksi AgAb presipitasi hb disitoplasma$ menurunnya leksibilitas eri$dll3

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3

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4

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5

LOGOKlasiikasi Anemia #emolitik



6ibagi atas 4 golongan besar$ yaitu-

@. Anemia hemolitik karena aktor di dalameritrosit sendiri 2gangguan intra korpuskuler3

4. Anemia hemolitik karena aktor di luar

eritrosit 2gangguan ekstra korpuskular 3


LOGO lan!utan9.Klasiikasi anemia hemol

itik -



-

!

@. Gangguan intra korpuskular 2#ereditary#emolyti0 Anemia 3

Membrane abnormality 2hereditarysphero0ytosis $ hereditary o7alo0ytosis 3

dee0t o globin 0hain 28halassemia$ #bpathia3

en;yme dee0t 2 GP6 dei0ien0y $ PK dei0ien0y3

LOGO"ereditar& Sp'ero!&tosis :



LOGO"ereditar& O(alo!&tosis :



9

LOGO

anjutan==klasifikasi anemia hemolitik



90

4. Gangguan ekstrakorpuskular

2A0Quired #emolyti0 Anemia3-

physi0al & 0hemi0al substan0es

ine0tions 2ba0teria$ parasites$ 7iruses$ungi3 me0hani0al trauma 2prosteti0 heart 7al7es3 Immune me0hanism 2Alloimmune &

Autoimmune & 6rugIndu0ed #A3

j

LOGO# #ereditary Sphero0ytosis -



91

autosomal dominant

Sphero0ytosis$ de0reased membrane sura0e arearelati7e to 0ell 7olume % osmoti0 ragility test 2O83/among the amily member .

8he primary lesion is 0aused by membrane protein

dee0ts 2'o spe0trin3 % 0ytoskeleton instability . 0hroni0 anemia $ !aundi0e$ splenomegaly$ 4

"ithout hemolysis & splenomegaly .,ilirubin e*0retion / $0ausing bilestone in :SG.

LOGO8halassemia -



92

6ee0t o @ or more globin0hain synthesis 2the

amount > Quantitati7ely3 -

dei0ien0y o ` globin0hain % `thalassemia dei0ien0y o globin0hain % thalassemia dei0ien0y o globin0hain % thalassemia

the primary dee0ts in #bpathia is in the globinamino a0ids stru0ture 2Qualitati7ely3

LOGO



93

LOGO



94

LOGO`8halassemia



95

`8halassemia > is 0aused by the impairment

o `globin 0hain produ0tion&synthesis .

`globin 0hain synthesis is dire0ted by 4

pairs o `gene 2X lo0us `gen3 % dependingo the number o dee0ted lo0us % 5 types o`8halassemia 2`thal trait $ #b# 6isease$and #b,arts #ydrops etalis3

LOGO(lini0al 0onseQuen0es in `8halassemi

a



9/

6ei0ien0y o `globin 0hain % e*0ess o $ c

0hain sin0e etal lie to orm Xtetramers2#b#3 or cXtetramers 2#b,art3 .

6ee0t o @4 `Gen > `trait 20lini0ally good3

6ee0t o 5 `Gen > #b# disease 2 #b @@@g&dl3 % e*0ess o 0hain % to orm Xtetramers 2#b#3 as intra0ellular in0lusion %

dete0ted by ,(,stain .

LOGO"%"#in!lusion ,78- in "%" Disease as s'o

9nin B2B staining ,!ompare 9it' reti!ulo!&te-



9-

LOGO



9

6ee0t o X `gene 2#b,artshydrops etalis3% 0lini0ally se7ere $ stillborn baby "ith

hydrops etalis 2 se7ere hypo*ia 3 .#b,arts > cXtetramers 2e*0ess o c0hainsthat unable to orm #b 3 .

#b,arts and #b# in0lusions pre0ipitated inred 0ells membrane % me0hani0al trappingin spleen % ma0rophagi0 phago0ytosis %

hemolysis .

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99

LOGO



100

LOGO# 7#0'alassemia



101

(lini0ally 0onseQuen0es in 8halassemia -

No problems during etal lie be0ause #bsynthesis is normally produ0ed2normal ` and c 0hains3

<hen #bA is dominantly needed $ the0lini0ally

problems e*ist as in0apability to synthesi;e#bA 2`443 % e*0ess o `0hain %0ompensated / o and c produ0tion %

#bA4/ 2in 8halassemia minor3 and #b / 2in8halassemia mayor3

LOGO

L$halassemia ma+or :



102

se7ere anemia % repeated transusion isotenly needed % e// % hemo0hromatosis

0hroni0 inee0ti7e erythropoiesis %

medullary hypertrophy in 0hildhood % a0ialmalormation-

= rontal bossing= Ma*illary hypertrophy

= #ypertelorism 2mongoloids eye3

LOGO 0hain deletion orms -



103

7#0'alassemia : no 7#!'ain

produ!tion.7;0'alassemia : 7#!'ain produ!tion

<<in 'etero=&gous !ase : medium se(erein 'omo=&gous : se(ere ,Cooley’sanemia-

LOGO



104

LOGO Laboratory 6iagnosis in 8halassemia



105

@. (,($ Peripheral ,lood Smear

4. #bEle0trophoresis - in (eluloseA0etat 2p#C.X3 or thalassemia and #bpathias0reening:sing hemolysate % ormed bands odierent types o #b 2 normal - bands A$ $and A4 $ measured densitometri0ally3

LOGO



10/

LOGO

anjutan=33ab diagnosis in thalasemia



10-

5. #bA4 mesurement to diagnose 8halassemia trait

using anion-e&change resin column chromatograph

in both #bELP and 0hromatography $ #b($ #bE and#bO 0an interrupt the 0on0lusion be0ause o the

same band lo0ation "ith #bA4 .

X. #b determination - Alkali 6enaturation 8est

A0idelution 2Kleihauer3 test +I6 or ELISA methods

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10

>. #b# In0lusion dete0tion -

Supra7ital staining using ,rilliant(resyl ,lue 2,(,3 or Ne"Methylene,lue 2NM,3

#b# in0lusion seen as dispersed blue green granules in red 0ells

20ompare "ith reti0ulo0yte as a ilament3 in #b# disease - #b# in0lusion RRR

in 8halassemia`trait - #b# in0lusion Rin @- @ eritrosit .

LOGO

>efisiensi I$)P>



109

# O*idant % produ0e #4O4 % o*idi;ing#bs ree sulhydryl % to orm Sul#b %aggregates that pre0ipitated as 'ein

Bodies destru0ted in spleen .

O*idant & Sul#b are 0ontrolled by+edu0ed Glutathione 2GS#-

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110

LOGO



111

?linked$ T 5 7ariants .

normal GP6 genes - type , 2Gd,3 type A 2GdA3 Abnormal en;yme types -

@. GdAW 2type AW3

4. GdMediterranean 2GdMed35. Gd(anton - many in Asia

GP6 dei0ient red 0ells are resistent

to Plasmodium al0iparum .

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Substan0es 0ausing lysis in G P6



112

# Substan0es 0ausing lysis in GP6dei0ien0y -

@. Antimalaria . a7a beans

4. Sulonamides D. Naphtalene5. )it.K$ )it.( C. :remia

X. Lung Ine0tion U. Antibioti0s 27irus$ba0teria3 2Peni0illine $

. Antipyreti0um streptomy0ine

LOGO

8h hi h t G P6 ti it i i



113

8he highest GP6 a0ti7ity is inreti0ulo0yte .

GP6 s0reening test -

8ests prin0iple -

GP6GP R NA6P PG R NA6P#

:)

2luores0en0e3

LOGOA0Quired #emolyti0 Anemia -



114

Se0ondary #emolyti0 Anemia !aused %&

infe!tion $ s&stemi! disorders :

Malignan0y W Autoimmunerea0ted hemolysis

$ mi0roangiopathy or hypersplenisme $appearing Anemia o 0hroni0 disease$bleeding tenden0ies$ and marro"ssuppression

LOGO



115

6isseminated Intra7as0ular (oagulation

26I(3-Systemi0 intra7as0ular 0oagulation % ibrindeposit intra7as0ularly & endothelial damage2mi0roangiopathyi3 0aused by sepsis % red0ells destru0tion .

(hroni0 Li7er 6isease - hemolysis 0aused byhypersplenism .

(hroni0 +enal 6isease- hemolysis 0aused bymi0roangiopathy

LOGOA0Quired #emolyti0 Anemia 2e*tra0or

pus0.3



11/

+ed 0ell membranebound Ab hemolysis .

8he speed hemolysis lo0ation depend onIgG or IgM$ and the ability to a0ti7ate

0omplement .Optimal temperature to bind Ab -

5D( W <armIgG8ype

J5

( W (oldIgG8ype

Immune #emolyti0 Anemia

LOGO

anjutan=3ac0uired hemol+tic anemia



11-

(ellRIgG % destru0ted by spleen(ellRIgM % enhan0e the a0ti7ation o0omplements 0as0ade % intra7as0ularhemolysis

Immune destru0tion oten 0ause minimallymembrane damage % shape 0hange into

sphero0yte .

LOGO



11

Immune #emolyti0 Anemia 0lassii0ation -

@. Alloimmune - 8ransusion +* $ 'emoltic

#isease o* the Ne+born ('#N)

4. Autoimmune - <arm&(old AI#A$Paro&smal Cold 'b-uria 2P(#3

5. #rug-induced '% - peni0illine type$

aldomet$ and stibophen type .

LOGO#emolyti0 6isease o the Ne"born 2#6N3 W+hneg mother $ "ith +hPos etus $ during I and se0ondpregnan0y



119

LOGOAntiglobulin 8ests 2(oombs3 -



120

6ire0t (oombs 8est 26ire0t Antiglobulin

8est&6A83 > Ab dete0tion test 2IgG and or (5d &0omplementbound red 0ells3 .

Indire0t (oombs 8est > test or serum ree Ab

.

6A8 usually positi7e in AI#A 2.

LOGO6rugIndu0ed hemolyti0 anemia :



121

Peni0illine type - drug as hapten binds red 0ell

membrane % antigeni0 % stimulate Ab produ0tionagainst 6rug in drugred 0ell 0omple*

Phena0etin&uinidin type - 6rug 2hapten3 adsorbed

protein % stimulatedAb binds drugprotein 0omple*% a0ti7ate 0omplement % red 0ell lysis.

Aldomet type - drug 0hange red 0ell membranesstru0ture % dete0ted as oreign 0ell % Autoantibodyprodu0tion .

LOGO



122

LOGOAplasti0 2#ypoplasti0_3 Anemia



123

Se7ere atal Anemia be0ause o ' red

0ells&leu0o0ytes&platelet produ0tion2pan0ytopenia3 0aused by Stem (ellsimpairment 2radiation$ 0hemi0als$ drugs$ orgeneti0 matters3

Marro" aplasia & hypoplasia0ausingsubstan0es radiation $ ben;ene$0ytostati0s 2MP$

busulan3$ arsen$ 0hlorampheni0ol$anti0on7ulsant 2phenytoin3$ analgeti02phenylbuta;one3 $ 668$ et0

LOGOSymptoms Lab.appearan0e o Aplasti0 An

emia



124

atigue$ palpitation$ ine0tions$ bleeding tenden0y

Lab - pan0ytopenia normo0hromi0 normo0yti0 Zdrytap marro" $ hypo0ellularity

Prognosis - bad espe0ially or J X yrs old patients %

marro" transplantation .

LOGO# 8reatment or Aplasti0 Anemia -



125

@. A7oid e7ery to*i0 material

4. A7oid ine0tions & bleeding tenden0y5. :se <ashedErythro0yte i transusion is

needed or Plat.(on0entrate 2P(3 or anyprouse bleeding 2 gi7e 0orti0osteroid ibleeding is minimal3

X. Marro" stimulants 2androgeni0 hormon 3

. Marro" 8ransplantation

LOGOPOLISITEMIA(ERITROSITOSIS)



Peningkatan patologis massa eritrosit massa eritrosit normal : (sea level )

- o : 26 - 32 ml kg !!

- o : 23 - 2" ml kg !! eritrositosis : massa eritrosit # normal

( P$% : o #&' o #*+ )

LOGO

• ,lasiikasi :



• ,lasiikasi :

I. Primer (Otonomik)A. Polisitemia %era

!. Eritrositosis M/rni (Eritremia)

II. Sek/n0erA. 1isiologis (Oksigenasi aringan

)

!. on-isiologis (Oksigenasiaringan )

III. Eritrositosis Relati

LOGO

E!,'!.C,.SIS - #I%/N.SIC ESS



• Complete Blood Count

• Bone "arro+ e&amination

• %rterial Blood /as analsis

• 0eukocte %lkaline Phosphatase

• P 1.

• I2P or renal ultrasound

• 0iver ultrasound or C scan

• Erthropoietin level

• Erthroid progenitor assa • Sleep apnea evaluation

LOGO

POLISI8EMIA )E+A

P li i kl l l tik l



• Prolierasi klonal neoplastik selprogenitor hematopoitik pluripoten

• Kriteria diagnosis P.). -

Kategori A

@.Massa eritrosit-

Lk H 5 ml & kg,, 2P() H X3

Pr H 54 ml & kg ,, 2P() H @3 4. Saturasi oksigen H U4

5. Splenomegali

LOGO

.ategori 1



.ategori 1

"3 rombositosis 3 & µl!

#3 ekositosis "#3 & µl!

(3 @kor -P ↑

3 1"# serum 9 pg&ml

• >iagnosis P7 bila :

-" -# -( atau -" -# dan # dari kategori 1

LOGOP!I"%!, 3P4!E5 E!,'!.C,.

SIS

( E!,'!E"I% 3



( E!,'!E"I% 3

• peningkatan massa eritrosit murni

• tidak ada pen+ebab eritrositosis sekunder

•

kadar eritropoitin normal atau rendah• mungkin akibat mutasi gene reseptor

eritropoitin → progenitor eritroid jadi lebih

sensitif terhadap eritropoitin3

LOGOII. E+I8+OSI8OSIS SEK:N6E+



• 6erupakan respons terhadap keadaan lain

+ang bersifat :

$ fisiologis : akibat oksigenasi jaringan +ang ↓

$ non fisiologis : tanpa penurunan oksigenasi

jaringan

LOGO

III.E+I8+OSI8OSIS +ELA8I



• @indroma Iaisbock• @tress er+throc+tosis

• Pseudo er+throc+tosis

- 6assa eritrosit tinggi normal

- 7olume plasma rendah

LOGO



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1 Nyonya Ana usia 40 tahun MRS (Masuk Rumah Sa

kit)

@M- -DH-; :




1. Nyonya Ana, usia 40 tahun, MRS (Masuk Rumah Sakit)

dengan keluhan pusing, dan badan terasa lemah. emeriksaan

!isik" #$ lemah, %ensi" 100&'0 mmg, Nadi"0 *&menit, RR" +0*&menit, suhu"-/. #epala&eher" anemia (), tidak di2umpai

ikterus, dyspnea dan sianosis, %horak&/or dan Abdomen "dalam

batas normal (dbn). 3*tremitas" dbn. asil laboratorium" b

g&dl, R5/ ,+0 * 101+&, ematokrit +4 6, M/7 -8 !l, M/ +8

pg, M// g&dl. 9ika anda adalah dokter 2aga di RS tersebut,

dari data yang ada, kemungkinan diagnosis pasien tersebut adalah"

A. Anemia normokromik:normositik

5. Anemia hipokromik:mikrositik /. Anemia makrositik

;. Anemia makrositik:megaloblastik

3. Anemia makrositik:non megaloblastik

LOGO

anjutan =333soal latihan




+. ;ari kasus ny. Ana, 40 tahun tersebut, diagnosis di!erensial untuk

penyebab anemianya adalah"

A. Anemia de!isiensi !olat, anemia de!isiensi 7itamin 51+,

5. Anemia karena perdarahan akut, anemia aplastik

/. Anemia de!isiensi besi, thalasemia, anemia sideroblastik

;. Anemia hemolitik, anemia pada penyakit mielo!ibrosis3. Anemia pada penyakit li<er, anemia pada penyakit hipotiroid

LOGO

anjutan =333soal latihan




. ;ari soal kasus Ny. Ana, 40 tahun tersebut, langkah

pemeriksaan laboratorium selan2utnya yang perludilakukan untuk kon!irmasi diagnosis adalah"

A. pemeriksaan bilirubin, haptoglobin, hitung

retikulosit

5. Serum =ron, %=5/ dan >eritin

/. emeriksaan 51+ dan asam !olat dalam darah

;. emeriksaan %, %4 dan %S

3. emeriksaan Aspirasi sumsum tulang

LOGO

- (5$+ear$old man complains of chronic ph+sical fatigue whic

h

2D;D2- 2-@K



- (5 +ear old man complains of chronic ph+sical fatigue, which

began ($ weeks ago3 He said he felt tired all of the time even

through his occupation as a software developer was mentall+ but

not ph+sicall+ demanding3 He breathed comfortabl+ at rest but,

when he e/erted himself, he e/perienced difficult+ in breathing and

had hard time catching his breath3 He also complained of Nmore

than usualO mental fatigue, confessing an increasing inabilit+ to

concentrate and focus his attention on tasks at hands3 2olleagues

noticed his pallor and his inattentiveness at brainstorming sessionsand suggested he reschedule his annual ph+sical e/amination for

an earlier date3 He complained of vague abdominal pain and sense

of abdominal fullness3 His appetite was depressed, and he thought

perhaps his ph+sical and mental s+mptoms were caused b+ poor

diet3 However, attempts to increase eating resulted in nausea3 Hisstools, he said, were sometimes loose and tarr+3 Kventuall+,

increased heart palpitations and chest pain made him seek medical

advice

LOGOLaboratory findings revealed thefollowing:L b P i N

l



following:Laboratory test Patient Normal

RBC (red blood cell count) 3.5 T/L 4.5-.!T/L

"CT (#ematocrit ratio) $%& 4!-5$&

"b (#emo'lobin) %.!'/dL 3-'/dL*C+ (mean cor,uscular

olume)

!L %-5L

*C" (mean cor,uscular#emo'lobin)

$$.%,' $,'

*C"C (mean cor,uscular

#emo'lobin concentration)

$%& 34&

LOGO

C hi t ti :

QK@DM;@



Case history questions:@. <hat general medi0al 0ondition is suggested by

the persons symptoms_4. <hat undamental 0hange in un0tion o blood

related to the red blood 0ells 0ouldsimultaneously ae0t the un0tion o se7eral

systems 20ardio7as0ular$ respiratory$gastrointestinal$ and others3_

5. <hat spe0ii0 diagnosis is supported by thelaboratory indings_

X. #o" 0ould the stool be related to the laboratoryindings?

LOGO

Answers:

-;@RKA



Answers:@. Anemia

4. A redu0tion in o*ygen0arrying 0apa0ity o theblood and thus a redu0tion in the deli7ery oo*ygen to 7arious body tissues

5. An iron dei0ien0y anemia

X. Most 0ases o irondei0ien0y anemia resultrom internal blood loss.

6ark$ tarry loose stools suggest bleeding

rom the gastrointestinal tra0t and "arranturther tests to determine the e*a0t 0ause

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