Lecture Neoplasma Sel Darah Putih

Post on 05-Mar-2016

295 Views

Category:

Documents

4 Downloads

Preview:

Click to see full reader

Transcript

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 1/76

NEOPLASMA SEL DARAH

PUTIH

Dr. Wien Wiratmoko. SpPA

Instalai PA - RSU.Dr. H. Ab!l Moeloek

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 2/76

"one marro# aala$ sat! or%an t!b!$

&an% terapat paa sel!r!$ trabe'!la

t!lan% ( me!la t!lan% pan)an%Tempat !tama proses $ematopoesis &an%

mempro!ksi sel ara$

"M a#al ber#arna mera$*komponeneritrosit+ % bertamba$ !sia ber!ba$

k!nin% *lemak+

 Akan kembali )i mera$ paa konisi

- perara$an $ebat

- $ipoksia

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 3/76

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 4/76

,e%anasan &an% berasal ari komponen

$ematopoetik #$ite bloo 'ells "M

inamakan LE,EMIA

Se'ara !m!m neoplasma W" 'ells iba%i

/. Neoplasma Lim0oi Ho%kin Lim0oma  an Non$o%kin Lim0oma

1. Neoplasma mieloi &an% men%enai

  'ells 2ran!losit3 Er&trosit3 Trombosit 4LMA3 2an%%!an mieloproli0erati0 kronik

  an Sinrom mieloisplastik

. Neoplasma Histiositik.

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 5/76

Proliliferative Disorders Neoplastik 

/. Le!kemia M&eloblastik Ak!t *AML+

T!mor itanai en%an proli0erasi sel mieloiimat!r *mieloblast+ &% men!mp!k lm "M

1. M&eloproli0erati5e DisorersT!mor itanai ole$ proli0erasi mat!r6e0erensiasi terminal3 tk terkenali3men!mp!k "M

. Sinrom M&eloisplasti'

2an%%!an i0erensiasi terminal &% tak terat!r 

Ditanai aan&a sel pre'!rsor "M ( sitopenia

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 6/76

Lekemia m&eloi mrp pen&akit ke%anasan

&an% itanai proli0erasi sel sistem$ematopoetik3 !tama i bone marro#

kem!ian t!mpa$ kealam sirk!lasi ara$.

Ta$!n 1778 USA aa /9.:17 kas!sPen&akit en%an spektr!m per)alanan

m!lai &an% !ntreate3 rapil&*0atal+ ; slo#l&

%ro#in%Se'ara traisional ibeakan A<UT (

<HRONI<

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 7/76

LATIHAN

Rema)a atan% % emam /7 $ari3 pemeriksaan 0isik t = <3perara$an ber!pa petekia i baan an ekstrimitas3

$epatome%ali3 splenome%ali3 lim0aenopati. Lab. Hb. /31

%6l3 Lekosit /=:76 mm 3 Hit!n% )enis se%men netro0il /

>3 Lim0osit ?= >3 Monosit / >. "iopsi s!ms!m t!lan%normal. Dia%nosa &an% ses!ai aala$

a. ALL b. AML '. Anemia aplastik . ITP e. In0eksi "akteri

Pemba$asan

"iopsi s!ms!m t!lan% normal3 apa e0inisi Le!kemia &ait!neoplasma s!ms!m t!lan%3 )ai $ar!sn&a ia $ipersell!ler kan.

"akteri &an% san%at 5ir!len bisa mn&ebabkan )!mla$ lekosit

t!r!n misaln&a in0eksi salmonela *t$&p$oi+

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 8/76

 A'!t M&eloi Le!kemia

In'iensi 396 /77.777 per t$.

Laki @ #anita 838

Etiolo%i Hereiter3 Raiasi *eBpos!re : ;C t$+3 ba$an kimia benena3 $erbisia3

pesti'ia

Obat kemoterapi *alkilatin% a%ent+Sem!a i$!b!n%kan en%an penin%katan

RESI,O

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 9/76

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 10/76

Tana an %e)ala klinis timb!l karena

/. UN2SI --- tiak aa sel mat!r2ran!losit m!a$ 4 In0eksi

Trombosit 4 Perara$an

1. HIPERFIS,OSITAS --- mieloblast i  sirk!lasi ara$3 in5asi ke or%an

,linis s!a$ m!n'!l selama G bln

Sinrom prelekemia i)!mpai p 1: >

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 11/76

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 12/76

UIS

Neoplasma mieloi aala$ pert!mb!$an&an% tiak terkenali men%enai sel-sel

iba#a$ ini ,E<UALI APA A

2ran!losit Er&trosit3

Trombosit

Histiosit

MonositHistiosit aala$ sel makro0a% &% aa i )arin%an mana sa)a3

 )ai ia b!kan pen!!k asli "M *s!ms!m t!lan%+.

Neoplasma mielo sinonim % le!kemia

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 13/76

/. Le!kemia M&eloblastik Ak!t *AML+

,lasi0ikasi A" *l!as ipakai klinisi+

M7 AML3 minimall& i00erentiate

M/ AML #it$o!t mat!ration

M1 AML #it$ mat!ration

M A'!te prom&elo'&ti' le!kemia

M8 A'!te m&elomono'&ti' le!kemia

M: A'!te monoblasti' le!kemiaM9 A'!te er&t$roi le!kemia

MC A'!te me%akar&oblasti' le!kemia

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 14/76

SOAL A

Men!r!k ,lasi0ikasi A"3 Ak!t Mielo%eno!sLe!kemia imana %ambaran $ap!san ara$

tepi tanpa aan&a bent!k mat!rasi keara$

ne!trop$ils3 sel "last iapatkan @ ?7 >3

mioloblast en%an n!'leoli prominent3sitoplasma tampak %ran!la a!ropilik aala$ M/

M:

M9

MC

M7

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 15/76

MI,IR SE"ENTAR O, AML mer!pakan neoplasma ara$ &an% itanai en%an

proli0erasi sel JJJJJ&% men!mp!k lm "M6S!ms!m T!lan%

Sel apaka$ it!

Mielosit

Monosit

Mieloblast Lim0oblast

"enar sem!a

Ingat yang jadi neoplasma adalah sel muda (blast) dengan

variasi adanya maturitas sel (prognosa lebih baik) maupun tanpamaturitas sama sekali (prognosa jelek) Gampang kan, itu maksud dari 

klasifikasi F!"

#imfoblast penduduk asli jaringan limfoid yang sekunder, seperti 

kelenjar getah bening"

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 16/76

,lasi0ikasi WHO 177/

 AML #it$ re'!rrent %eneti' abnormalities

 AML #it$ m!ltilinea%e &splasia AML an MDS3 t$erap&-relate

 AML3 not ot$er#ise spe'i0ie

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 17/76

 ADA SOAL DULU

Diba#a$ ini "U,AN mer!pakan klasi0ikasi

 Ak!t Mielo%eno!s Le!kemia men!r!t WHO3

aala$

 AML #it$ re'!rrent %eneti' abnormalities  AML #it$ m!ltilinea%e &splasia

 AML an MDS3 t$erap&-relate

 AML not ot$er#ise spe'i0ie

 AML #it$o!t mat!ration

Ha0al n%%ak K &a be%inila$ sekola$ ikeokteran

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 18/76

,elainan %enetik paa AML

 AML #it$ t*=1/+*1111+ * $#%&'+

 AML #it$ abnormal bone marro#eosinop$ils in5*/9+*p/11+ or t*/9/9+*p/11+ **!F+&$-%%+ A'!te prom&elo'&ti' le!kemia *AML #it$

t*/:/C+*11/1+*P$#&../ + an5ariants AML #it$ //1 *$##+ abnormalities

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 19/76

 AML #it$ t*=1/+*1111+ * $#%&'+

- Morp$olo%i'all& M1

 AML #it$ abnormal bone marro# eosinop$ils

in5*/9+*p/11+ or t*/9/9+*p/11+

**!F+&$-%%)

- Morp$olo%i'all& M8 A'!te prom&elo'&ti' le!kemia *AML #it$ t*/:/C+

*11/1+*P$#&../ + an 5ariants

- Morp$olo%i'all& M

 AML #it$ //1 *$##+ abnormalities

- Morp$olo%i'all& M:

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 20/76

,lasi0ikasi A"

M0. AML, minimally differentiated

Lekemia ak!t itanai tk aan&a

i0erensiasi sel m&eloi * li%$t mikroskop+

M&eloi linea%e iperiksa men%%!nakan

imm!nop$enot&pi' or !ltrastr!'t!ral st!&

Positi5e <D/3 <D3 <D//C3 <D8Ne%ati5e <D//3 <D/:

-6 MPO * onl& in 0e# blasts+

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 21/76

M1. AML without maturation

 A'!te le!kemia tanpa aan&a bent!k

mat!rasi keara$ ne!trop$ils"lasts iapatkan @ ?7 >

M2. AML with maturation A'!te le!kemia en%an mat!rasi bent!k

ne!tro0il lm berba%ai stai!m

"M bila i biopsiM&eloblasts 17 >

Ne!trop$ils /7 >

Mono'&tes G 17 >

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 22/76

M/. A'!te m&elo%eno!s le!kemia3 men!r!t A"

m&eloblast kromatin $al!s3 n!kleoli prominent % %ran!la a!ropilik i

sitoplasma

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 23/76

M3. Acute promyelocytic leukemia

,linis serin% iapatkan sitopenia ( DI<Mor0olo%i % "M aspirasi

 Abnormal $&per%ran!lar prom&elo'&tes

N!'le!s kine&-s$ape or bilobe

<&toplasm ab!nant3 3 bri%$t pink3 ensel&

pa'ke %ran!les3 b!nles o0 A!er ros *0a%%ot

'ell+

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 24/76

M A'!te prom&elo'&ti' le!kemia ; A"

bbrp sel inti bilobe3 m!ltiple neelelike a!er ros *'enter+3 %ran!la

a!ropilik

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 25/76

M4. Acute myelomonocytic leukemia

 A'!te le!kemia isertai proli0erasi

ne!trop$il an pre'!rsor mono'&te"M

Total blasts 17 >

Ne!trop$il an pre'!rsors 17 >

Mono'&te an pre'!rsors 17 >

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 26/76

M5. Acute monoblatic and monocyticleukemia 

 A'!te le!kemia &an% itanai pop!lasiseln&a =7 > aala$ mono'&ti' linea%e

M:A monoblasts *!s!all& @ =7 >+

M:" promono'&tes

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 27/76

M:b. A'!te mono'&ti' le!kemia ; A"

sat! monoblast an : promono'&te % membran inti &% melek!k

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 28/76

LATIHAN SOAL DULU

Laki-laki DEWASA kel!$an perara$an paa %!si m!l!t &an%s!a$ berlan%s!n% min%%!3 pemeriksaan 0isik iapatkanpenebalan paa %in%%i5a3 bila kena tekanan m!a$ berara$3$epatosplenome%ali3 lim0aenopati paa le$er3 lipat pa$a anketiak.Lab. Hb. //31 %6l3 Hmt. 3?>3 Trombosit ?:.7776mm3Lekosit 8:776mm3 Hit!n% )enis 1:> se%men3 /7> batan%3

::> lim0osit3 = > monosit. "iopsi s!ms!m t!lan% Hipersell!ler3ominant sel blast. Dia%nosa &an% palin% m!n%kin aala$  Ak!t Me%akar&ositik Le!kemia  Ak!t Lim0oblastik Le!kemia  Ak!t Monositik Le!kemia  Ak!t Er&troblastik Le!kemia

 Ak!t Promielositik Le!kemia

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 29/76

M!. Acute erythroid leukemia

Er&t$role!kemiaEr&t$roi pre'!rsors :7 > ari total n!'leate

'ells an

M&eloblasts 17 > mrp non-er&t$roi 'ells

P!re er&t$roi le!kemia

Er&t$roi pre'!rsors =7 > men!mp!k i "Mtanpa )!mla$ m&eloblast &% si%ni0ikan

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 30/76

M". Acute me#akaryoblatic

leukemia

 A'!te le!kemia imana :7 > ari

pop!lasi blasts aala$ me%akar&oblasts

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 31/76

0" $yeloproliferative Disorders

Menin%katn&a proli0erasi sel pro%enitor4 m&eloilinea%es i "M menyebar ke or#an ekunder

$%&  *$ati3 lien3 k%b+

Relati0 normal ok memperta$ankan i0erensiasi

terminal en%an $ematopoiesis &an% e0e'ti5e*eritrosit, platelet, granulosit, monosit,1 limfosit +

Tk $eran bila tana !taman&a aala$

Hepatome%ali an t!. splenome%ali sebab

$emopoesis ekstrame!la3 lim0aenopat$i rin%an

an elemen neoplastik i ara$ peri0er 

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 32/76

M&eloproli0erati5e isorers *MPDs+

 WHO Classification 2001

/. Le!kemia Mielo%enosa ,ronik *LM,+

a. <$roni' m&elo%eno!s le!kemia

b. <$roni' ne!trop$ili' le!kemia'. <$roni' eosinop$ili' le!kemia

1. Pol&'&t$emia 5era

. Primar& m&elo0ibrosis *metaplasia mieloi en%an

mielo0ibrosis+8. T$rombo'&t$emia Essential

:.<$roni' m&eloproli0erati5e isease3 !n'lassi0iable

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 33/76

1. Leukemia Mielo#enoa 'ronik (LM')

 A!lt !sia 1:-97 t$ % insiensi p!n'akekae 8 ata! :

/: ; 17> ai sem!a le!kemia

Dia%nosisData klinis

Laboratori!m

"one marro# biops&

2eneti's st!& 0or t*?11+

O''asional imm!nop$enot&pi' st!&

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 34/76

 AWAS Seoran% Laki e#asa atan% keokter en%an

kel!$an ben)olan paa re%io 'olli3 aBilla m!ltipleiameter 1 ; 9 'm3 soli3 l!nak. Paa pemeriksaan0isik T /C76/77 mmH%3 t C 7<3 lien terabamembesar3 Lab. Hb. /731 %6L3 Hmt 73: >3 M<F ?7 !m. Trombosit 1.7776mm3 Lekosit

/.776mm3 biopsi s!ms!m t!lan% normal. Dia%nosa&an% ses!ai keaaan iatas aala$ In0eksi monon!'leosis In0eksi m&'oba'teri!m T"  Anemia Hipoplastika

,ronik Lim0ositik Le!kemia  Ak!t Lim0oblastik Le!kemia

In%at sekali la%i "M normal3 ,ronik lim0ositik le!kemia aala$neoplasma sel lim0oi i )arin%an lim0oi sek!ner *,2"+ nantikala! s!a$ pen!$ t!mpa$ )!%a ke sirk!lasi ara$ tepi. Qeniskronik p oran% e#asa3 ak!t paa anak-anak.

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 35/76

LM,

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 36/76

Pato%enesa

 ?:> LM, 4 kromosom P$3 t *?311+men&ebabkan terbent!kn&a %en 0!si "<R-

 A"L men%koe protein 0!si ba%ian "<R

an omain tirosin kinase &% akti5itasn&atk tekenali

 Aan&a prek!rsor %ran!lositik *palin%ominan+3 eritroi3 me%akariositik3 lim0osit" seba%ian T 4 mrp b!kti LM, berasalari sel pl!ripoten

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 37/76

LATIHAN U,

Le!kemia Mielo%enosa kronik ?:> item!kan kromosom

P$ilaelpia3 ter)ai kelainan apa paa kromosomn&a nin% K

Translokasi ,romosom */?3 11+

!si %en "<R ; A"L

M!tasi titik

M!tasi S!presor %en

M!tasi Protoonko%en

Ter)ai translokasi kromosom ?3 11

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 38/76

Chr 22

BCR

e/JJ e// e12 e13 e14 e15 e16 e17 e18 e19 e20

b1 b2 b3 b4 b5

majorC1 c2 c3 c4

muminor

Chr 9

a1 a2 a3 a4 a5 a6 a7 a8 a9 a10 a11

ABL

b1 b2 b3

ba1 0!sion---p1/7 protein

p1/7 "<R-A"L

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 39/76

b2a2 or b3a2 fusions

of the BCR-ABL genes

*ncreain# cell proliferation

+ecreain# cell death

*nducin# independence from #rowth

factor

$educin# adheion of affected cell to

troma

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 40/76

IN2AT LO

,romosom P$ilaelpia paa LM, memi'! ter)ain&a

kelainan-kelainan iba#a$ ini3 ,E<UALI

Menin%katn&a proli0erasi sel

Men!r!nn&a kematian sel

Menin%katn&a se'ara bebas t$ 2ro#t$ a'tors

Menin%katn&a ikatan *a$esion+ antara sel anstroma

Men!r!n&a apoptosis sel

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 41/76

,linis

2e)ala a#al tak spesi0ik3 pro%resi0 lambat

Splenome%ali &% ekstrem3 r!pt!r3 in0arkLekosit @ /77.7776!L ominan netro0il an

mielosit tapi baso0il3 eosino0il )!%a tampak

Trombositosis

"M 4 $ipersel!ler % t!r!nan %ran!lositik an

me%akariositik

Tana k$as LM, kromosom P$ an

menin%katn&a baso0ilDapat men%alami trans0ormasi

Seikit komplikasi m&elo0ibrosis

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 42/76

Terapi

Da$!l! terapi paliati0 an kemoterapi3 tkmen'e%a$ ke krisis blast

De0initi0 Transplantasi "M 4 C7> semb!$bila onor 'o'ok

Terapi onkoprotein STI- :C/ *2lee5e'+ 4analo% aenosin tri0os0at men%$ambatker)a tirosin kinase termas!k protein 0!si

"<R-A"R

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 43/76

Soal !l!

Paa saat ini terapi &an% palin% !m!m

ilak!kan ter$aap neoplasma sel ara$

p!ti$ aala$

Pembea$an ( Raioterapi

Raioterapi ( ,emoterapi

,emoterapi ( Transplantasi

Transplantasi ( Im!noterapi Sem!a benar 

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 44/76

SOAL MANIN2 ,AN2

Hal &an% palin% s!lit ikenalikan alamta$ap-ta$ap terapi Le!kemia aala$ In0eksi oport!nistik

Perara$an Serebri Trombosis Mikrosirk!lasi

Sem!a bisa ikenalikan

Qa#aban A " < benar 

Qai pasien menin%%al b!kan karenale!kemian&a3 s!lit sekali &a. Qai perl!monitorin% ketat

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 45/76

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 46/76

LATIHAN SOAL LA2I A

an% imaks! en%an respon komplit *<omplete

Response+ pasien le!kemia &an% menapatkan terapi

se'ara meis aala$

Tiak iapatkankann&a Sel Neoplasma Lekosit

Q!mla$ lekosit abnormal G :7776!L

Q!mla$ Lekosit G /7.7776!L3 mor0olo%i normal

,aar Hb normlal3 Trombosit G /7.7776!L

Sem!a ,omponen ara$ G /7.7776!L

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 47/76

1. Pol&'&t$emia 5era *PF+

Dibeakan

/. PF primer6 Er&tositosis Absol!t1. PF se'!ner 

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 48/76

Category I : 1. Increased erythrocyte volume

(>36 ml/kg in male, >32 ml/kg in female)

  2. Arterial oxygen saturation > 92%

  3. Slenomegaly

Category II : 1. !latelet count > "##$### ul

  2. &eucocytosis > 12$###ul

  3. Increased &A!&eucocyr Al'ali !hosatese  scorewithout fever or infection

  ". Serum (it 12 > 9## gml

  or unboundvit b12-binding capacity > 22pg/ml

Polcite!ia "era #riteria $% &

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 49/76

Pol&'&t$emia 5era *PF+

,riteria Dia%nosti'

2emua *ategory I

or 

% 3 0 in *ategory I   an 0 *ategory II 

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 50/76

Pol&'&t$emia 5era WHO 'riteria

A1: Elevated RC ma!! "#25 $ above mean%& 'b # 18(5 in men and #16(5 in )omen%

A2: *o cau!e o+ !econdar, er,t-roc,to!i!

A3: ./lenomeal,A4: Clonal enetic abnormalit, ot-er t-an CRA in

marro) cell!

A5: Endoenou! er,t-roid colon, +ormation in vitro

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 51/76

1: -romboc,to!i! # 400&000

2: C # 12&000

3: anm,elo!i! in bone marro) bio/!, )it-/rominent er,t-roid and meaar,oc,tic

/roli+eration

4: o) !erum er,t-ro/oietin level!

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 52/76

Pol&'&t$emia 5era WHO 'riteria

Dia%nosis PF

  A/ A1 an& A

or 

  A/ A1 an& 1 o0 "

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 53/76

MAU TANA APA S 2 TANA

,riteria minimal &% ipakai mene%akkania%nosa Polisitemia Fera ADALAHen%an men%%!nakan stanart WHO Sem!a A "/ ata! "1

 A/ A1 sala$ sat! A lain  A1 sem!a "

 A/ sem!a "

Sem!a "

2ampan% &a kala! $a0al3nanti kala! )ai okter 

l!pa tin%%al b!ka b!k! n%%ak aa &% melaran%

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 54/76

Splenome%ali Masi0 717 %ram *N /:7-177 %ram+

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 55/76

Pol&'&t$emia 5era

Pat$olo%& i "MH&per'ell!lar trilinea%e marro#3 re5erse

ME or ME G 1/

In'rease er&t$roi 'ells3 all kins o0normoblasts

In'rease me%akar&o'&tes3 mil to

moeratePleomorp$i' 0orm o0 me%akar&o'&tes

an mil 0ibrosis

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 56/76

Per)alanan pen&akit

Insiensi @ 87 t$ ; 97 t$

Menin%katn&a 5ol!me an kekentalan ara$

akibat Er&topoesis

Serin% timb!l $ipertensi3 sakit kepala3 sal!ran

'erna3 $ematemesis3 melena

"ila tiak menapat terapi berta$an bbrp b!lan

setela$ DB,omplikasi 4 trombosis ( in0ark *'or3ren3lien+

M&elo0ibrosis ter)ai G /7> kas!s

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 57/76

Terapi

Ter%ant!n% Qenis

P$lebotomi 'ara seer$ana bisa berta$an/7 t$

Mielos!presi0 TB '$emot$erap& ( 0os0or

raioakti0 4 e0ek m!ta%enik

PR l

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 58/76

PR !l! Laki e#asa en%an kel!$an sakit kepala an p!sin%

se)ak b!lan &an% lal!3 kaan% m!n'!l %atal-%atalsel!r!$ t!b!$ an "A" #arna ke$itaman. P t 93:7 <3 T /976?: mmH%3 $epar ( lien tak teraba. Sample0eses positi0 aan&a eritrosit3 Hb 113 %6l3 $ematokrit 9C3/ >3 M<F ?8 !m3 trombosit 8:.7776 mm3Lekosit C=776mm. Apa ia%nosa ses!ai

Sinrom Mieloisplastik

Er&trole!kemia

<ML

Polisitemia Fera

Disseminate Intra5as'!ler <oa%!lation *DI<+

P$lebotom& serin% )!str! meran%san% pro!ksi er&trosit makin

menin%kat3 ok 'ari pen&ebab&a. "ila Smoker polisitemia &a

istop rokokn&a an biasan&a tiak isertai % splenome%ali

- i Mi l fib i

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 59/76

. -rimary Mielofibroi

Mrp %an%%!an mieloproli0erati0 kronis spent p$ase ter)ai paa a#al per)alanan

pen&akit 4 ibrosis "M

Hematopoesis intrame!la ber%eserekstrame!la *tk berat!ran ( ine0isien+

men&ebabkan anemia sean% ; berat (

trombositopenia

ibrosis isebabkan ole$ kelainan

$ematopoetik t! Me%akariosit

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 60/76

Pen'et!sn&a aala$ stim!lasi !tk

berproli0erasi ter!s mener!s ole$ P2 (

Trans0ormin% %ro#t$ 0a'tor  &%

iprooksi ole$ me%akar&osit neoplastikPaa !m!mn&a pasien atan% s!a$

iapatkan ibrosis "M ( Hematopoesis

ekstrame!la

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 61/76

Mor0olo%i

Hepatoslenome%ali men'apai 877 %r *$ati (

,2" rin%an+ 4 ekstrame!la $ematopoesis

Histolo%is ter)ai proli0erasi ari normoblast3

pre'!rsor %ran!losit an me%akariosit

"M tampak $iposel!lar an 0ibrosis l!as

Me%akariosit tampak palin% menon)ol ba$kan

men%alami isplastik

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 62/76

'hro!bocte!ia Pri!ar (ielofibrosis

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 63/76

<. Sinrom m&eloisplasti'

Mrp %an%%!an mat!rasi s$% $ematopoesistiak e0ekti0 an memp!n&ai resikoter)ain&a trans0ormasi LMA

"M i%anti ole$ sel pro%enitor m!ltipoten&% m!tan &% memperta$ankan

kemamp!an beri0erensiasi men)ai SDM3%ran!losit an trombosit % 'ara &% tiakterat!r ( ine0ekti0 4 Hipersel!ler ata!normosel!ler tepi ara$ tepi Pansitopenia

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 64/76

8. T$rombo'&temia *san%at )aran%+

Sito%enetik ter)ai elesi kromosom : ata!

C

Insiensi ekae : ; C t! laki-laki

/7 -87> tran0ormasi LMA sisan&a

menerita in0eksi3 anemia ok tak aan&a selm&eloi i "M

Ptro%nosis berta$an antara ? ; 1? b!lan

Respon kemoterapi )elek

N l Hi ti itik

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 65/76

Neoplasma Histiositik*Lan%er$ans <ell Histio'&tosis+

Immat!re enriti' 'ell *Lan%er$ans 'ell+

EBpress S-/773 <D /a

N!'lei linear %roo5e or 0ol

<&toplasm "irbe'k %ran!le *EM+

L $ ll $i ti t i

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 66/76

Lan%er$ans 'ell $istio'&tosis 

 An ele'tron mi'ro%rap$ s$o#s rolike "irbe'k %ran!les #it$ '$ara'teristi' perioi'it&

an ilate terminal en.

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 67/76

PERTANAAN DULU Pasien anak3 kel!$an !tama lesi er!psi k!lit an

emam. Sat! ta$!n &an% lal! perna$ menerita Otitismeia an s!a$ iterapi. Pemeriksaan 0isikiapatkan lim0aenopati3 $epatosplenome%ali3 $asilbiops& tampak "irbe'k %ran!la paa sitoplasma sel.Dia%nosa &an% ses!ai aala$

 Ak!t Lim0oblastik Le!kemia

M!ltiple M&eloma

Lan%er$ans <ell Histiositosis

Ho%kin isease

NonHokin isease

Tin%%al i$a0alkan sa)a nin% ; akan%3 mas - mbak

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 68/76

Letterer ;Si#e Disease

M!lti0o'al3 m!ltis&stem L<H

G 1 &rs

Skin ∼ seborr$ei' er!ption on tr!nk (

s'alp

$epatosplenome%al&3 l&mp$aenopat$&3

p!lmonar& lesion osteol&ti' bone lesion

<$emo tB → :7> pB s!r5i5e : &ears

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 69/76

Eosinop$ili' %ran!loma

Uni0o'al 6 m!lti0o'al3 !nis&stem L<H

Histio'&te3 Eosinop$il 3 l&mp$o'&te3

plasma 'ell3 ne!trop$il

Uni0o'al skeletal s&stem in oler'$ilren or a!lts

M!lti0o'al m!ltiple erosi5e bone mass

in &o!n% '$ilrenHans-)chuller-Christian tria 'al5arial

bone e0e't iabetes in'ipi!s

eBop$t$alm!s

Pemeriksaan "M

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 70/76

Pemeriksaan "M

 Dibeakan Aspirasi an "iopsi masin%-masin%memp!n&ai ke!n%%!lann&a

Inikasi aala$ kelainan ara$ tepi3

pen&akit ke%anasan3sta%in% paa t!mor paat3

In0eksi UO6 0e5er o0 !nkno#n ori%in3

pen&akit metabolik

Lokasi paa anak sem!a ekstrimitas sean%

e#asa proksimal ekstremitas3 krista iliaka anteriorata! posterior

Man!bri!m sterni ok ketebalan /'m 4 Aspirasi

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 71/76

Hemopoiesis

2ran!lopoiesis

Er&t$ropoiesis

Me%akar&o'&tes

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 72/76

B*+ - $,)PO)ABL* A()H,$,-'.P*

BO+*-(ARROW B,OP). +**$L* 

a!shi/i bone !arro asiration an/

bios nee/le

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 73/76

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 74/76

"IOPSI

 ASPIRASI

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 75/76

7/21/2019 Lecture Neoplasma Sel Darah Putih

http://slidepdf.com/reader/full/lecture-neoplasma-sel-darah-putih 76/76

top related