Retikulo-End. FK UMS 2010U

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PPatologiatologi SSistemistemRRetikulo-etikulo-EEndhotelialndhotelial

Kuliah FK. UMS SurakartaKuliah FK. UMS Surakarta

oleh : dr. Oyong, SpP.oleh : dr. Oyong, SpP.

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meliputi !meliputi !- sel darah putih "leukopenia,- sel darah putih "leukopenia,

neutropenia#, leukemia,neutropenia#, leukemia,- in$lamasi sel darah putih %- in$lamasi sel darah putih %

kel.getah &ening,kel.getah &ening,- neopl. sel darah putih% 'M,- neopl. sel darah putih% 'M,- leukemia "oleh PK#,- leukemia "oleh PK#,

- mieloma multiple,- mieloma multiple,- histiositosis sel 'angerhans,- histiositosis sel 'angerhans,

splenomegali.splenomegali.

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Enlargement of Lymph NodesEnlargement of Lymph Nodes

Anatomy & TopographyAnatomy & Topography

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'nn. :'nn. :

(. tdk tera&a)sangat ke*il,(. tdk tera&a)sangat ke*il,+. tera&a: reakti$,+. tera&a: reakti$,in$eksi"spesi$ik)tdk#, neoplasmain$eksi"spesi$ik)tdk#, neoplasma

primer, neoplasma sekunder.primer, neoplasma sekunder.

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Physical Characteristics of enlarged nodesPhysical Characteristics of enlarged nodes

Nodes with metastatic CaNodes with metastatic Ca  : Stony hard, nontender, ell- : Stony hard, nontender, ell-lo*alied, &ound to surrounding tissue, nonmoa&le.lo*alied, &ound to surrounding tissue, nonmoa&le.

Acute infectionAcute infection : $irm, tender, asymetri*, matted, oerlying: $irm, tender, asymetri*, matted, oerlying

skin might &e red % edematous.skin might &e red % edematous.Chronic infectionChronic infection : nontender, nonedematous.: nontender, nonedematous.

Acute leukemiaAcute leukemia : tender.: tender.

Nodes in lymphomaNodes in lymphoma : large,symetri*, ru&&ery, $irm, moa&le,: large,symetri*, ru&&ery, $irm, moa&le,

dis*rete, nontender.dis*rete, nontender.

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Location of enlarged nodesLocation of enlarged nodes

Generalized adenopathy ! " separate nodes#Generalized adenopathy ! " separate nodes#

-- Non$%odgkin LymphomaNon$%odgkin Lymphoma

- /hroni* 'ympho*yti* leukemia- /hroni* 'ympho*yti* leukemia

- immuno&lasti* lymphadenopathy- immuno&lasti* lymphadenopathy

Generalized adenopathyGeneralized adenopathy

- Un*ommon in adult ith in$e*tion, e0*ept- Un*ommon in adult ith in$e*tion, e0*ept

- mononu*leosis, 12/, hepatitis, se*ondary syphilis,- mononu*leosis, 12/, hepatitis, se*ondary syphilis,to0oplasmosis, histoplasmosis.to0oplasmosis, histoplasmosis.

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'pecific Location'pecific Location

Post auricular adenopathyPost auricular adenopathy : Ru&ella: Ru&ella

(nilateral anterior auricular adenopathy(nilateral anterior auricular adenopathy : 1ra*homa,: 1ra*homa,*on3un*tiitis, 12/, syphilis.*on3un*tiitis, 12/, syphilis.

)ilateral cer*ical adenopathy)ilateral cer*ical adenopathy : 12/, mononu*leosis,: 12/, mononu*leosis,

to0oplasmosis,to0oplasmosis, lymphomalymphoma, leukemia., leukemia.(nilateral cer*ical mass(nilateral cer*ical mass : Metastati* $rom 4P/.: Metastati* $rom 4P/.

Palpa+le supracla*icular nodesPalpa+le supracla*icular nodes : 4eoplasti* in: 4eoplasti* ina&domen)*hest.a&domen)*hest.

A,illary nodesA,illary nodes : drain part o$ &reast.: drain part o$ &reast.

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'pecific location-'pecific location-

.nguinal adenopathy.nguinal adenopathy : 'ymphogranuloma enereum,: 'ymphogranuloma enereum,*han*roid,syphilis.*han*roid,syphilis.

Enlarged mediastinal/hilar Enlarged mediastinal/hilar  nodes "5-ray# may&e asymptomati*nodes "5-ray# may&e asymptomati*or tra*heo&ron*hial *ompression.or tra*heo&ron*hial *ompression.

)ilateral mediastinal adenopathy)ilateral mediastinal adenopathy : *ommon in: *ommon in N%LN%L%ilar adenopathy%ilar adenopathy : 12/ "unilateral#, *o**idioidmy*osis %: 12/ "unilateral#, *o**idioidmy*osis %sar*oidosis "&ilateral#.sar*oidosis "&ilateral#.

A+dominal nodesA+dominal nodes might enlarge in any disorderith *ausemight enlarge in any disorderith *ausegeneral adenopathy.general adenopathy.

0etroperitoneal adenopathy0etroperitoneal adenopathy : 4eoplasti*, espe*ially: 4eoplasti*, espe*iallylymphomatuslymphomatus

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'ymph 4ode'ymph 4ode

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'ymph 4ode'ymph 4ode1 A22 L34P% 5E'

" CAP'(LE

6 PE0. P%E0 '.N('

7 PA0A 28L.C(LA0

C80TE9/ T CELL

:8NE

; L34P%8.< 28L.CLE/

) CELL :8NE

= E22 L34P% 5E'

> A0TE0. & 5E.N

? 4E<(LLA

@ 4E<(LLA03

'.N('E'

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'6MP7 4O8E'6MP7 4O8E R/791E/1URE R/791E/1URE

/orte0/orte0

Para*orte0Para*orte0

MedullaMedulla

/apsule and $i&rous tra&e*ulae/apsule and $i&rous tra&e*ulae

SinusesSinuses

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1he *orte0: 2-one o$ lymph1he *orte0: 2-one o$ lymph

nodenodePrimary lymphoid $olli*lePrimary lymphoid $olli*le irgin 2 *ells and mature 2 *ellsirgin 2 *ells and mature 2 *ells Folli*ular dendriti* *ellsFolli*ular dendriti* *ells

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Organiation o$ Organiation o$ se*ondary lymphoid $olli*lese*ondary lymphoid $olli*le

Marginal one *ells "9gM#

Mantle one *ells "9gM;8#

Folli*le *enter *ells "9gM,9g<, 9g or 9gE, not 9g8#

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RE/19ERE/19E SS '6MP7OM'6MP7OM

E4SR7-PFKU9=>=?@?A, /79'8, ( 6RS, 28OM94' MSS ; MU'19P'E '6MP78E4OP176

KPP 'M28 2/'+

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 /U1E 4O4 SP '6MP78E4919S /U1E 4O4 SP '6MP78E4919S

0EACT.5E0EACT.5E

C%ANGE'C%ANGE' :: 4icro organism4icro organism

Cell de+risCell de+ris 2oreign material2oreign material

*eri*al*eri*ala0illariesa0illaries

inguinalinguinal

Ma*rosMa*ros : Enlarged,: Enlarged,sollen, grey-red,sollen, grey-red,pain.pain.

Mi*rosMi*ros : 'ymphoid $oll: 'ymphoid $ollprominent, largeprominent, largegerminalgerminal*enter,histiosit,*enter,histiosit,

neutrophil.neutrophil.

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massa papa&el, *olli :massa papa&el, *olli :lim$onodi mem&esar :lim$onodi mem&esar :

radang dan neoplasma malignaradang dan neoplasma maligna"primer, sekuder#"primer, sekuder#

sitologi)sitopatologi ! diagnostiksitologi)sitopatologi ! diagnostikpendahuluan, &kn $inalpendahuluan, &kn $inal

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17E 1E/749BUES OF F417E 1E/749BUES OF F4

4eedle moed &a*k4eedle moed &a*kand $orth ithinand $orth ithintarget tissuetarget tissue

4egatie pressure4egatie pressure

released hilereleased hileneedle remains inneedle remains intarget tissuetarget tissue

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Acute Non specific Lymp$itisAcute Non specific Lymp$itis

Rea*tie *hangesRea*tie *hanges

/eri*al, a0illaries, inguinal, mesenteri*/eri*al, a0illaries, inguinal, mesenteri*

4orphology4orphology : enlarged, sollen, grey-red,: enlarged, sollen, grey-red,distended *apsdistended *aps  tender.tender.

%istopathology%istopathology : 'ymph. Folli*les CC,: 'ymph. Folli*les CC,

'arge germ / ith mitoti* a*tiity,'arge germ / ith mitoti* a*tiity,neutrophil in$iltration. Sinuses *ellneutrophil in$iltration. Sinuses *ell  hyperhypertrophi ) plasia.trophi ) plasia.

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Chronic Non 'pecific LympitisChronic Non 'pecific Lympitis

9nguinal, a0illaries, not tender.9nguinal, a0illaries, not tender.

%istopathology%istopathology::

$olli*ular hyperplasia-2 *ell$olli*ular hyperplasia-2 *ell

dark one, light one, plasma *ell,dark one, light one, plasma *ell,histiosit, PM4, Eosinophyl.histiosit, PM4, Eosinophyl.

2ollicular Lymphoma B2ollicular Lymphoma B

(. r*hi. 9nter $oll. 1 % Sinus(. r*hi. 9nter $oll. 1 % Sinus

+. ariation shape % sie nod+. ariation shape % sie nod

D. 8ark-'ight, Phago*yti*-ma*.D. 8ark-'ight, Phago*yti*-ma*.

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9.9. Folli*ular hyperplasiaFolli*ular hyperplasia

a*tiated 2 *ell, larged, round germinal *enter.a*tiated 2 *ell, larged, round germinal *enter.

99.99. Para*orti*al lymphoid hyperplasiaPara*orti*al lymphoid hyperplasiaRea*tie *hages 1 *ellRea*tie *hages 1 *ell

iral 9n$e*tion.iral 9n$e*tion.

999.999. Sinus histio*ytosis.Sinus histio*ytosis.

8istention-prominent sinusoid8istention-prominent sinusoid

'ymph node draining *an*er 'ymph node draining *an*er 

Chronic Non 'pecificChronic Non 'pecific

LymphadenitisLymphadenitis

S O S'9MF8E4919S KRO49S

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'9MF8E4919S KRO49S'9MF8E4919S KRO49S4O4 SPES9F9K4O4 SPES9F9K

1.1. Gambaran jaringan kelenjar limfe denganGambaran jaringan kelenjar limfe dengansentrum germinativum membesar dansentrum germinativum membesar danaktif mengandung limfosit-limfosit mudaaktif mengandung limfosit-limfosit muda yang menunjukkan mitosis atau proliferasi yang menunjukkan mitosis atau proliferasi

sel-sel retikulum yang sering mengandungsel-sel retikulum yang sering mengandungkuman atau debris seluler yang telahkuman atau debris seluler yang telahdifagositosis.difagositosis.

2.2. Penambahan sel retikulum dan limfositPenambahan sel retikulum dan limfosit

dalam sinus disebut sinus catarrh.dalam sinus disebut sinus catarrh.3.3. Fibrosis diantara jaringan limfoid.Fibrosis diantara jaringan limfoid.

.. !apsul dari nodus limfatikus bisa!apsul dari nodus limfatikus bisamengalami periadenitis akan tampak tebalmengalami periadenitis akan tampak tebal

dengan infiltrasi sel-sel radang kronisdengan infiltrasi sel-sel radang kronis

'9MF8E4919S KRO49S

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'9MF8E4919S KRO49S'9MF8E4919S KRO49S4O4 SPES9F9K4O4 SPES9F9K

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Chronic specific LymphadenitisChronic specific Lymphadenitis

T()E0C(L8'AT()E0C(L8'A

4AC08'4AC08' BB

&ergerom&ol, pa*ked, *entral *aseous.&ergerom&ol, pa*ked, *entral *aseous.

4.C08'4.C08' BBtu&er*le, *heese, ne*roti*, langhans *ell,epitheloid *ell.tu&er*le, *heese, ne*roti*, langhans *ell,epitheloid *ell.

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MorphologyMorphology : &esar, multiple,: &esar, multiple,

&ergerom&ol, tdk nyeri, liide, $istel&ergerom&ol, tdk nyeri, liide, $istel7istopatology :tu&er*, epiteloid, *aseosa,7istopatology :tu&er*, epiteloid, *aseosa,langhans.langhans.

'okasi'okasi : 'eher, Supra*la, a0illa: 'eher, Supra*la, a0illa

Chronic specific LymphadenitisChronic specific Lymphadenitis 

L34P%8.< NE8PLA'4AL34P%8.< NE8PLA'4A

'im$oma - 'eukemia'im$oma - 'eukemia

%< N%L

'9MF8E4919S KRO49S SPES9F9K'9MF8E4919S KRO49S SPES9F9K

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'9MF8E4919S KRO49S SPES9F9K'9MF8E4919S KRO49S SPES9F9K"12/#"12/#

1.1. "ampak struktur jaringan limfoid dengan"ampak struktur jaringan limfoid dengansarang-sarang tuberkel yang khas# berupasarang-sarang tuberkel yang khas# berupanekrosis perkejuan di tengah dikelilingi olehnekrosis perkejuan di tengah dikelilingi olehsel-sel yang tersusun epiteloid dan tampaksel-sel yang tersusun epiteloid dan tampakbeberapa sel datya $anghansbeberapa sel datya $anghans

2.2. Fibrosis disekitarnya dengan infiltrasiFibrosis disekitarnya dengan infiltrasi

limfosit yang padat.limfosit yang padat.

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.ntroduction.ntroduction

Lymphoproliferati*e malignancies originating from the )$Lymphoproliferati*e malignancies originating from the )$

cell )$N%L# or the T$cell lineage TN%L#cell )$N%L# or the T$cell lineage TN%L#

8epending on the *lini*al *ourse, *lassi$i*ation as high-grade8epending on the *lini*al *ourse, *lassi$i*ation as high-grade"aggressie# or lo-grade lymphoma is 3usti$ied."aggressie# or lo-grade lymphoma is 3usti$ied.

There is a worldwide epidemic of N%LsThere is a worldwide epidemic of N%Ls ith more than @=,===ith more than @=,===*ases diagnosed in the United States ea*h year.*ases diagnosed in the United States ea*h year.

<istri+ution B 4ale ! 2emale<istri+ution B 4ale ! 2emale

.C<$1 B C?"$C?? C@11.C<$1 B C?"$C?? C@11

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9ntrodu*tion9ntrodu*tion

'ymphoma re$ers to a *lonal tumor arising'ymphoma re$ers to a *lonal tumor arising$rom the malignant trans$ormation o$$rom the malignant trans$ormation o$mature or immature lympho*ytes "2 *ells,mature or immature lympho*ytes "2 *ells,

1 *ells, or 4K *ells#1 *ells, or 4K *ells#

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No 4alignancy 2reDuency(+DD?

@A>(=(((+

Lymphoma<astrointestinal<yne*ology2reastUrinary tra*t

7ead and ne*k1hyroidSkin /a'ung /aOsteosar*oma/4SSo$t tissue tumor 1otal

A "+=G# "(>G#+ "(G#+A "(+G#+ "(=G#

( "A.AG#(= ".DG#  A"DG#  @ "+.?G#

"(.AG#  + "=.G#  ( "=.G#+D+

Solid 1umor in+=((H

H 8ata Pathology anatomy 8epart RSM7Palem&ang

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No 4alignancy 2reDuency

(+D?

@A>(=((

<yne*ology2reastLymphoma7ead and ne*k /a<astrointestinal

So$t tissue tumor Urinary tra*tSkin /aOsteosar*oma'ung /a/4S1otal

 (D= "++G# (( "(>G#  ?= 1;#  A "(DG#  @?"((G#

  D@ "@G#  +? "G#  +( "DG#  ("+G#  (("(.G#  "=.AG# ?

Solid 1umor in +=(+H

H 8ata $rom Pathology natomy 8ept RSM7 Palem&ang

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/hara*teristi*H/hara*teristi*H

Age y# 4ale 2emale Total

I+=+= J +>D= J D>= J >

; F ;@@= J @>  A= 

@(=A

11D+

-@

7(-

"@G#(= "(@G#( "++G#(D "+=G#

1; "7# "@G#+ "DG#

D +? @+

H Population : 78 and 47' pts at 9nternal Med 8epart, in +=(( - +=(+

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/lini*al Features/lini*al Features

Su&3e*ties :Su&3e*ties :

Chief complain 2reDuency

Masses or nodal enlargement in *eri*al D+

Masses or nodal enlargement in a&domen >

8ysphagia or dyspnea

Masses or nodal enlargement in *eri*al,supra *lai*ula, a0illa and inguinal

+

Masses in shoulder (Masses in rm (

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/lini*al Features "*ontL.#/lini*al Features "*ontL.#

ide ariety o$ presentationside ariety o$ presentations

2 symptoms "$eers, night seats, t loss#2 symptoms "$eers, night seats, t loss#

Painless adenopathy, mostly in *eri*al regionPainless adenopathy, mostly in *eri*al region

9ntraa&dominal masses9ntraa&dominal masses8ysphagia8ysphagia

8yspnea8yspnea

O&stipationO&stipation

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Etiological & 0isk 2actorsEtiological & 0isk 2actors

9mmune de$i*ien*y syndromes9mmune de$i*ien*y syndromes

RE' /l i$i i $

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RE' /lassi$i*ation o$ RE' /lassi$i*ation o$ non-7odgkin lymphomasnon-7odgkin lymphomas

Pre*ursor lympho&lasti* lymphoma)leukemia

"2 or 1 *ell type#

Peripheral 2-*ell neoplasms

Peripheral 1-*ell % postulated 4K-*ell lymphoma

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9ntrodu*tion "*ontL#9ntrodu*tion "*ontL#

Non$%odgkins lymphoma N%L# BNon$%odgkins lymphoma N%L# B

is a heterogeneous group o$ lymphoproli$eratieis a heterogeneous group o$ lymphoproli$eratiemalignan*ies.malignan*ies.

47' results $rom *lonal e0pansion o$ tumor *ells47' results $rom *lonal e0pansion o$ tumor *ells

deried $rom 2, 1, or natural killer "4K# *ells.deried $rom 2, 1, or natural killer "4K# *ells.Most 47's, a&out ?G to >=G in the United States,Most 47's, a&out ?G to >=G in the United States,

are deried $rom 2 *ells.are deried $rom 2 *ells.47's are *hara*teried &y aria&le natural histories47's are *hara*teried &y aria&le natural histories

and therapeuti* responses.and therapeuti* responses.

1he *lini*al *ourses : ary greatly, ranging $rom1he *lini*al *ourses : ary greatly, ranging $romindolent to rapidly $atal.indolent to rapidly $atal.

47' is a disease predominantly o$ the elderly47' is a disease predominantly o$ the elderly

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PER9P7ER' 2 /E''PER9P7ER' 2 /E''

4EOP'SMS4EOP'SMS2-*ell S'')/'')P''2-*ell S'')/'')P''

'ymphoplasma*ytoid'ymphoplasma*ytoidlymphomalymphoma

Mantle *ell lymphomaMantle *ell lymphomaFolli*le *enterFolli*le *enterlymphomalymphoma

Marginal one 2 *ellMarginal one 2 *ell

lymphomalymphoma E0tranodal "M'1#E0tranodal "M'1# 4odalH4odalH

Spleni* marginal oneSpleni* marginal one2 *ell lymphomaH2 *ell lymphomaH

7airy *ell leukemia7airy *ell leukemia

Plasma*ytomaPlasma*ytoma8i$$use large 2 *ell8i$$use large 2 *elllymphomalymphoma

2urkitt lymphoma2urkitt lymphoma

7igh grade 2 *ell7igh grade 2 *elllymphoma, 2urkitt-lymphoma, 2urkitt-likeHlikeH

* Provisional entities

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>>

PendahuluanPendahuluan %1&%1&

'orfologi $' sel " ( tidak jelas'orfologi $' sel " ( tidak jelas

'orfologi $' sel ) ( sebagian bisa dikenal'orfologi $' sel ) ( sebagian bisa dikenal

*el " di korteks thymus ( "dt# +,1a# +,3#*el " di korteks thymus ( "dt# +,1a# +,3#+,# +,+,# +,

*el " mature ( +, atau +,/*el " mature ( +, atau +,/*el 0! ( ! %!iller nhibitory eceptor&*el 0! ( ! %!iller nhibitory eceptor&

PER9P7ER' 1 /E'' %PER9P7ER' 1 /E'' %

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PER9P7ER' 1 /E'' %PER9P7ER' 1 /E'' %POS1U'1E8 4K /E''POS1U'1E8 4K /E''

4EOP'SMS4EOP'SMS1-*ell /'')P''1-*ell /'')P''

'arge granular'arge granular

lympho*yte leukemialympho*yte leukemia 1 *ell or 4K *ell type1 *ell or 4K *ell type

My*osis $ungoidesMy*osis $ungoides

Peripheral 1 *ellPeripheral 1 *ell

lymphoma unspe*i$iedlymphoma unspe*i$ied

 ngioimmuno&lasti* 1 ngioimmuno&lasti* 1*ell lymphoma*ell lymphoma

 ngio*entri* lymphoma ngio*entri* lymphoma

9ntestinal 1 *ell9ntestinal 1 *ell

lymphomalymphoma dult 1 *ell lymphoma- dult 1 *ell lymphoma-leukemia, 71'(;leukemia, 71'(;

 naplasti* large *ell naplasti* large *ell

lymphoma, 1 % null-*elllymphoma, 1 % null-*ell

 naplasti* large *ell naplasti* large *elllymphoma, 7odgkin-likeHlymphoma, 7odgkin-likeH

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?(?(

$imfoma sel " mature$imfoma sel " mature %2&%2&

4tranodal 0! 5 " cell lymphoma nasal4tranodal 0! 5 " cell lymphoma nasaltypetype

nteropathy associated "-cell lymphomanteropathy associated "-cell lymphoma

6epatosplenic "-cell lymphoma6epatosplenic "-cell lymphoma

*ubcutaneous penniculitis like "-cell*ubcutaneous penniculitis like "-celllymphomalymphoma

'ycosis fungoides'ycosis fungoides

*e7ary syndrome*e7ary syndrome 

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?+?+

$imfoma sel " mature$imfoma sel " mature %3&%3&

Primary cutaneous peripheral "-cellPrimary cutaneous peripheral "-celllymphomalymphoma

Peripheral "-cell lymphoma 08*Peripheral "-cell lymphoma 08*

9ngio immunoblastic "-cell lymphoma9ngio immunoblastic "-cell lymphoma

9naplastic large cell lymphoma# 9$!9naplastic large cell lymphoma# 9$!

positivepositive9naplastic large cell lymphoma# 9$!9naplastic large cell lymphoma# 9$!negativenegative

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?D?D

$imfoma sel " mature$imfoma sel " mature %3&%3&

Primary cutaneous peripheral "-cellPrimary cutaneous peripheral "-celllymphomalymphoma

Peripheral "-cell lymphoma 08*Peripheral "-cell lymphoma 08*

9ngio immunoblastic "-cell lymphoma9ngio immunoblastic "-cell lymphoma

9naplastic large cell lymphoma# 9$!9naplastic large cell lymphoma# 9$!

positivepositive9naplastic large cell lymphoma# 9$!9naplastic large cell lymphoma# 9$!negativenegative

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E0tranodal 4K)1-*ellE0tranodal 4K)1-*elllymphomalymphoma

(.(./lini*al $inding/lini*al $inding

+.+.MorphologyMorphology

D.D.9mmunophenotyping9mmunophenotyping

..<eneti* alteration<eneti* alteration?.?.1reatment1reatment

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E0tranodal 4K)1-*ellE0tranodal 4K)1-*elllymphomalymphoma

(.(./lini*al $inding/lini*al $inding

+.+.MorphologyMorphology

D.D.9mmunophenotyping9mmunophenotyping

..PrognosisPrognosis?.?.1reatment1reatment

Lethal midline granuloma

Location BNPrototype B Nose

  $$$$$ENHTCL nasal typ

  palate paranasal sinu

  nasopharyn,N E,tra$nasal B

  $$$$$ skin soft tissue G

  testis

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E0tranodal 4K)1-*ellE0tranodal 4K)1-*elllymphomalymphoma4orphology B4orphology B 

(.(.Ma3ority : polymorphousMa3ority : polymorphous  H small, medium, large atypi*al lympho*ytesH small, medium, large atypi*al lympho*ytes

  H in$lammatory *ells : lympho*ytes, plasma *ells,H in$lammatory *ells : lympho*ytes, plasma *ells,ma*rophages, neutrophilsma*rophages, neutrophils polymorphic reticulosispolymorphic reticulosis

  malignant histiocytosismalignant histiocytosis

  malignant reticulosismalignant reticulosis

  'teward granuloma'teward granuloma

+. Prominent ne*rosis+. Prominent ne*rosis lethal midline granulomalethal midline granuloma

D.D. ngio*entri*ity ngio*entri*ity angiocentric T$cell lymphomaangiocentric T$cell lymphoma

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E0tranodal 4K)1-*ellE0tranodal 4K)1-*elllymphomalymphoma

4orphology B4orphology B (.(.Ma3ority : polymorphousMa3ority : polymorphous

  H small, medium, large atypi*al lympho*ytesH small, medium, large atypi*al lympho*ytes

  H lympho*ytes, plasma *ells, ma*rophages,H lympho*ytes, plasma *ells, ma*rophages,

neutrophilsneutrophils ----------+e careful of nasal+e careful of nasal

predilectionIIpredilectionII

+. Prominent ne*rosis -----sear*h $or+. Prominent ne*rosis -----sear*h $or atypicalatypical

cellscells

D.D. n io*entri*it n io*entri*it

TAHE %84E

4E''AGE 1#

1> yo female mass in nasal ca*ity

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;7%1"

y y

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;7%1"

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@(@(

8ther e4tra nodal lymphoma8ther e4tra nodal lymphoma

4tranodal 0!5"-cell lymphoma nasal4tranodal 0!5"-cell lymphoma nasaltypetype

nteropathy associated "-cellnteropathy associated "-celllymphomalymphoma

6epatosplenic "-cell lymphoma6epatosplenic "-cell lymphoma

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9ntrodu*tion "*ontL#9ntrodu*tion "*ontL#

%odgkins <isease B%odgkins <isease B

/hara*teried &y Reed-Stern&erg /ells/hara*teried &y Reed-Stern&erg /ells

1ends to o**ur in young adults1ends to o**ur in young adults

Mediastinal disease *ommonMediastinal disease *ommon

Spreads to *ontiguous nodesSpreads to *ontiguous nodes

/ommon to hae a lo*aliedQ/ommon to hae a lo*aliedQpresentationpresentation

7ighly *ura&le ith *urrent treatments7ighly *ura&le ith *urrent treatments

7 d ki 8i7odgkin 8isease

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7odgkin 8isease7odgkin 8isease

/hain nodes-spread *hara*teristi*/hain nodes-spread *hara*teristi*

<iant *ells,<iant *ells, Reed-Sten&erg *ellReed-Sten&erg *ell indu*edindu*ed

R5 lymph, histio*yt, granulo*yt.R5 lymph, histio*yt, granulo*yt.

4odular s*lerosis4odular s*lerosis

mi0ed *ellularitymi0ed *ellularity

lymph predominantlymph predominant

lympho*yte-depletedlympho*yte-depleted

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Reed-Sternberg cells and variantsReed-Sternberg cells and variants

Cytopathologic diversity of H-RS cellsCytopathologic diversity of H-RS cells

Diagnostic Reed-Sternberg cellsDiagnostic Reed-Sternberg cells

Mononuclear Hodgkin cellMononuclear Hodgkin cell

Lacunar cellLacunar cell

L&H cellL&H cell

Mummified cell apoptosis cells!Mummified cell apoptosis cells!

"leomorphic Reed-Sternberg cell"leomorphic Reed-Sternberg cell

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#-L"HL

L&H cell

#S-HL

Lacunar cell

MC-HL

Diagnostic RS cell

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$ppropriate cellular environment$ppropriate cellular environment

Depending on the specific subtype of HodgkinDepending on the specific subtype of Hodgkin

lymphoma% presence or absence of the folloing'lymphoma% presence or absence of the folloing' Small lymphocytes% ith fe or no immunoblastsSmall lymphocytes% ith fe or no immunoblasts

HistiocytesHistiocytes

"lasma cells"lasma cells

(osinophils and neutrophils(osinophils and neutrophils

)ibroblasts)ibroblasts

Hodgkin lymphoma'Hodgkin lymphoma'

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Hodgkin lymphoma'Hodgkin lymphoma'

*f the term of +Hodgkin, be replaced out%*f the term of +Hodgkin, be replaced out%

#odular lymphocyte predominant Hodgkin lymphoma#odular lymphocyte predominant Hodgkin lymphoma

  #odular lymphocyte predominant large .-cell#odular lymphocyte predominant large .-cell

lymphomalymphoma

#odular sclerosis classical Hodgkin lymphoma#odular sclerosis classical Hodgkin lymphoma

  Lacunar cell lymphoma ith sclerosisLacunar cell lymphoma ith sclerosis

Mi/ed cellularity classical Hodgkin lymphomaMi/ed cellularity classical Hodgkin lymphoma (.0-associated polymorphous large-cell(.0-associated polymorphous large-cell

lymphoproliferative disorderlymphoproliferative disorder

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Sel 8oroty Reed-/arl Sten&ergSel 8oroty Reed-/arl Sten&erg

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Sel 8oroty Reed /arl Sten&ergSel 8oroty Reed /arl Sten&erg

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7odgkin 8isease7odgkin 8isease

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7odgkin disease7odgkin disease

(==G kel lim$e(==G kel lim$e

1unggal1unggal  menye&ar menye&ar 

+=-= th"D=-D? th#+=-= th"D=-D? th#

enisenis 4odular s*ler.4odular s*ler. : @?G: @?G

Mi0ed *ellularityMi0ed *ellularity

: +?G: +?G

'im$ositik predominan'im$ositik predominan (=G(=G 'im$ositik depletion'im$ositik depletion

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'ympho*yte predominan*e

Mi0ed *ellularity

4odular s*lerosis

'ympho*yte depletion

4odular lympho*yte predominant

/lassi*al 7odgkin lymphoma

'ympho*yte-ri*h

Mi0ed *ellularity

4odular s*lerosis

'ympho*yte depletion

03E CLA''.2.CAT.8N J%8 CLA''.2.CAT.8N

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/lini*al staging o$ 78/lini*al staging o$ 78

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78 % 47'78 % 47'

7878 47'47'

'o*al lymph ./ER-'o*al lymph ./ER-ME.S1-P.ME.S1-P.

MU'19P7ER9P7ER'MU'19P7ER9P7ER'

/O419BU916/O419BU916 4O4O

E51R 4O8'E51R 4O8'

U4/OMMO4U4/OMMO4

/OMMO4/OMMO4

'8E6ER %'8E6ER %MESE41ER9/MESE41ER9/94O'E8 RRE94O'E8 RRE

/OMMO4/OMMO4

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4O8U'R S/'EROS9S4O8U'R S/'EROS9S

 48'T C8448N =;$>;48'T C8448N =;$>; K 38(NGK 38(NG

PRO<4OS 29K, 'E7ERPRO<4OS 29K, 'E7ERSUPR/'9/U', ME89S194UM.SUPR/'9/U', ME89S194UM.

4O8U'R, R9414O8U'R, R941 R.SR.S.,., 'a*unar *ell'a*unar *ell,,*ollagen &and*ollagen &and,, diide lymphositis-nodule.diide lymphositis-nodule.

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Reed-Sten&erg *ellReed-Sten&erg *ell

4icroskopis B

0eed$sten+erg cell: &esar, &inukleus,&erhadap-ka*a,kadang-kadang multinu*, single lo&,sekitar halo area

Lacunar cell B 

nodular sel

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4 7 d ki ' h4 7 d ki ' h

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4on 7odgkin 'ymphomas4on 7odgkin 'ymphomas

MU'19P'E M6E'OMMU'19P'E M6E'OM

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MU'19P'E M6E'OMMU'19P'E M6E'OM

P'SM /E''P'SM /E''  &one, skin '4.&one, skin '4.

C T , 9g C , prot 2en*e ones, Midle age "?=#.C T , 9g C , prot 2en*e ones, Midle age "?=#.

/lini* :/lini* :

 Multiple &one destru*tMultiple &one destru*t ert, Ri&s, Skull, pelis, $emur, Rert, Ri&s, Skull, pelis, $emur, R &ulat oal,&ulat oal,

&atas&atas $ra*t. Patol.$ra*t. Patol.

Mi*ros : plasma *ell CC pleo morphi*, e0entri* nu*leusMi*ros : plasma *ell CC pleo morphi*, e0entri* nu*leus

plasma &lastplasma &last spleen, lier, kidney, lungsspleen, lier, kidney, lungs

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F li l l hF li l l h

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47' : paling sering"?G#47' : paling sering"?G#TVTV! deasa! deasa

Mi*ros : nodular struktur, small *ell irreg.Mi*ros : nodular struktur, small *ell irreg.

inti &igger *ell *hrom CC, nu*leoli Cinti &igger *ell *hrom CC, nu*leoli C/lini*al : Painless lim$adenopati general/lini*al : Painless lim$adenopati generale0tra nodul rare, median surial A-> the0tra nodul rare, median surial A-> th

10 : /hemoteraphy, radiasi, palliati 1010 : /hemoteraphy, radiasi, palliati 10

Foli*ular lymphomaFoli*ular lymphoma

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8i$$ ' 2 ll ' h8i$$us 'arge 2 *ell 'ymphoma

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8i$$us 'arge 2-*ell 'ymphoma8i$$us 'arge 2-*ell 'ymphoma

+=G 47'+=G 47'TTI, @= thI, @= th

Mi* :Mi* :  8i$$us patern8i$$us patern

'arge *ell : -?0 lim$ ke*il'arge *ell : -?0 lim$ ke*il 4u*lei : &ulat-oal, *leaed-lo&ulasi4u*lei : &ulat-oal, *leaed-lo&ulasi  nak inti : +-D atau ( di tengah nak inti : +-D atau ( di tengah

/lin/lin ::

pem&esaran kel. lim$., kadang-kadang e0tra nodal : <it, &one,pem&esaran kel. lim$., kadang-kadang e0tra nodal : <it, &one,

&rain, skin.&rain, skin.P0 :P0 : elekelek

10 :10 : 9ntensie mi0 *hemo 109ntensie mi0 *hemo 10*omplete remission @=-=G, ?=G $or*omplete remission @=-=G, ?=G $orseeral years.seeral years.

2 *ell l mphoma2 *ell lymphoma

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2-*ell lymphoma2-*ell lymphoma

/hroni* lympho*iti* lymphoma/hroni* lympho*iti* lymphoma

Foli*ular lymphomaFoli*ular lymphoma

8i$$us large 2 *ell lymphoma8i$$us large 2 *ell lymphoma

2urkitt lymphoma2urkitt lymphoma

Plasma *ell neoplasm, et*.Plasma *ell neoplasm, et*.

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1 *ell lymphoma % natural killer *ell 41 *ell lymphoma % natural killer *ell 4

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1 *ell lymphoma % natural killer *ell 41 *ell lymphoma % natural killer *ell 4

(.(. Peri 1 *ell ' U4SPPeri 1 *ell ' U4SP

+.+.  dult 1 *ell 'ymphoma dult 1 *ell 'ymphoma

D.D. My*osis $ungoidesMy*osis $ungoides

.. 7odgkin disease7odgkin disease

Pre*ursor 2 /ell % 1 *ell 4Pre*ursor 2 /ell % 1 *ell 4

 *ut 'ympho&lasti* ' *ut 'ympho&lasti* '

Klasi$ikasi &erariasiKlasi$ikasi &erariasi

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mnrt : sal :sel 2,sel 1.mnrt : sal :sel 2,sel 1.

Struktur : FO',89F.Struktur : FO',89F.

ol.sel : 'R<E,SM.ol.sel : 'R<E,SM.

89FERE4S9S9 : ORK94< FROM.,RE'89FERE4S9S9 : ORK94< FROM.,RE'/'S.,RPPPOR1./'S.,RPPPOR1.

 /U1 '9MFO2'S19/ '9MFOM)'EUKEM9 /U1 '9MFO2'S19/ '9MFOM)'EUKEM9

di S : +?== KSUS 2RU)17di S : +?== KSUS 2RU)17

I (? th, kulit putih +5I (? th, kulit putih +5

C atau W T, &alita, I (? th.C atau W T, &alita, I (? th.Mi* : Str 4orm kel li$ rusak, sel lim$o&las dominan : 2esar.Mi* : Str 4orm kel li$ rusak, sel lim$o&las dominan : 2esar.9nti 'o&ulated, Mitosis CC9nti 'o&ulated, Mitosis CC

/linn : nemia, 'emah, Panas/linn : nemia, 'emah, Panas  a*ut. 9n$eksi, 2one pain,a*ut. 9n$eksi, 2one pain,

Spleen % lier enlargement, <eneral lim$adenopati, dllSpleen % lier enlargement, <eneral lim$adenopati, dll

Chronic lymphocitic / small cell limfomaChronic lymphocitic / small cell limfoma

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Chronic lymphocitic / small cell limfomaChronic lymphocitic / small cell limfoma

Sering pada deasaSering pada deasa

Small 2 *ell pd sirkulasi "leukemia#Small 2 *ell pd sirkulasi "leukemia#

Mi*ros : str 4' rusak, Small lim$osit predominan,Mi*ros : str 4' rusak, Small lim$osit predominan,

inti ke*il, kom&inasi dgan sel C &esarinti ke*il, kom&inasi dgan sel C &esar"prolim$ositi*#"prolim$ositi*#

/lin :/lin :  C ?= tahunC ?= tahun TTI atau W , sering asimptom, 22X, lemah, anoreksi,I atau W , sering asimptom, 22X, lemah, anoreksi,

'ekosit CC, 'im$adenopatia, spleen; 'ier 'ekosit CC, 'im$adenopatia, spleen; 'ier  

2urkitt lim$oma2urkitt lim$oma

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2urkitt lim$oma2urkitt lim$oma

Endemi*-sporadi*-agresi$ "$rika#.Endemi*-sporadi*-agresi$ "$rika#.Mi* : inasi intermed sie sel lim$osit, intiMi* : inasi intermed sie sel lim$osit, intioal-&ulat, nu*leoliC( kromatin kasar,oal-&ulat, nu*leoliC( kromatin kasar,

mitosisCC starry skymitosisCC starry skypatern" ma*rophages, ingest nu* de&ris#patern" ma*rophages, ingest nu* de&ris#

/lin :/lin :

e0tra nodal mani$es, mandi&ula, organ intrae0tra nodal mani$es, mandi&ula, organ intra &domen. &domen. /hildren, muda./hildren, muda.

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SP'EE4SP'EE4

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SP'EE4SP'EE4

SP'EE4SP'EE4

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SP'EE4SP'EE4

(?= <R : (+ 0 A 5 D /M(?= <R : (+ 0 A 5 D /M

M/ROS : KPSU' 19P9S, 2U-2UM/ROS : KPSU' 19P9S, 2U-2U 1R2EKU' P8. PRE4K9M MER71R2EKU' P8. PRE4K9M MER7

8<4 2ER/K PU197-791E PU'P. RE88<4 2ER/K PU197-791E PU'P. RE8PU'P. S94USSO98 8<4 KP9'ER 88PU'P. S94USSO98 8<4 KP9'ER 8819P9S19P9S

FU4<S9FU4<S9

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FU4<S9FU4<S9

(.(. Pem&entukan sel darah "diluar sumsumPem&entukan sel darah "diluar sumsumtulang#.tulang#.

+.+. Proses hemolisisProses hemolisis

D.D. Reseroir darah, mo&ilisasi &ila perluReseroir darah, mo&ilisasi &ila perlu

.. Reaksi pertahanan-keke&alan tu&uh,Reaksi pertahanan-keke&alan tu&uh,produksi lim$osit dan at antiproduksi lim$osit dan at anti

NeoplasiaMyeloproli$eratie and myelodysplasti*disorders, leukemia,

d l h

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and lymphoma%emolytic anemias

7ereditary sphero*ytosis, ellipto*ytosis

1halassemias, si*kle *ell anemiaAutoimmune disorders

 utoimmune hemolyti* anemia1hrom&o*ytopenia, essential neutropeniaSystemi* lupus erythematosusRheumatoid arthritis

Sar*oidosis.nfections

9n$e*tious mononu*leosis, &ru*ellosis, et*.Malaria, to0oplasmosis, et*.'epsis

Congestion

/irrhosis o$ the lier 2udd-/hiari syndrome/hroni* *ongestie heart disease'torage diseases

<au*her disease, 4iemann-Pi*k diseaseAmyloidosis

@@(AD=

<angguan sirkulasi<angguan sirkulasi

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<angguan sirkulasi<angguan sirkulasi

(.(. 2endungan akut2endungan akut+.+. 2endungan menahun2endungan menahun

D.D. Sindroma &anti : splenomegali kongesti$Sindroma &anti : splenomegali kongesti$

CC, lekopeni, anemi.CC, lekopeni, anemi... 9n$ark : O&str. Em&oli *or single) multiple-9n$ark : O&str. Em&oli *or single) multiple-

2a3i.2a3i.

 <amna-<andy &odies : &er*ak a&u+-*oklat<amna-<andy &odies : &er*ak a&u+-*oklat"$i&rosis; endapan pigmen# pd 2anti"$i&rosis; endapan pigmen# pd 2antisindromsindrom

SplenomegaliSplenomegali

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p gg

(.(. 9n$eksi : 1yp. 12/, Mal syph9n$eksi : 1yp. 12/, Mal syph

+.+. /ongesti : /irh, de*omp, portalthrom/ongesti : /irh, de*omp, portalthrom

D.D. 'im$o-hematogen dis.'im$o-hematogen dis.

.. 9mmunogeni*9mmunogeni*?.?. Storage dis : gau*her, niemann-Pi*kStorage dis : gau*her, niemann-Pi*k

@.@. 'ain : amyloid, neopl'ain : amyloid, neopl

4eoplasm : arang4eoplasm : arang

2enign : 7emangioma, 'im$angioma, $i&roma2enign : 7emangioma, 'im$angioma, $i&roma

histiositosis sel 'angerhanshistiositosis sel 'angerhans

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histiositosis sel 'angerhanshistiositosis sel 'angerhans

"histiositosis 5# !"histiositosis 5# !

- prol. sel '/, normal di- prol. sel '/, normal di

epidermis,epidermis,- &erakuola, sitoplasma- &erakuola, sitoplasma

&anyak, inti esikular, lon3ong&anyak, inti esikular, lon3ongatau &erlekuk,atau &erlekuk,

D klinikopatologik !D klinikopatologik !

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p g !p g !

- histiositosis '/ akut terse&ar- histiositosis '/ akut terse&ar"sindr. 'etterer-Sie#,"sindr. 'etterer-Sie#,

- histiositosis '/ uni$okal "gra.- histiositosis '/ uni$okal "gra.eosino$ilik#,eosino$ilik#,

- multi$okal "peny. 7ands*huller- multi$okal "peny. 7ands*huller

/hristian#/hristian#

8endriti* "antigen presenting#8endriti* "antigen presenting#

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8endriti* "antigen presenting#8endriti* "antigen presenting#*ells*ells

'angerhans *ell "skin#'angerhans *ell "skin#9nterdigitating dendriti*9nterdigitating dendriti**ell "1-one#*ell "1-one#

9ndeterminate *ell "skin#9ndeterminate *ell "skin#

eiled *ell "lymphati*#eiled *ell "lymphati*#

Folli*ular dendriti* *ellFolli*ular dendriti* *ell"2 *ell $olli*le#"2 *ell $olli*le#

Fi&ro&lasti* dendriti*Fi&ro&lasti* dendriti**ell "Y#*ell "Y#