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8/16/2019 Lymphoma 28-1-15
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LYMPHOMALYMPHOMA
dr Putra Hendra SpPDdr Putra Hendra SpPD
UNIBAUNIBA
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ALL ALL MMMMCLLCLL LymphomasLymphomas
Hematopoietic
stem cell
Neutrophils
Eosinophils
Basophils
Monocytes
Platelets
Red cells
Myeloid
progenitor
Myeloproliferative disordersMyeloproliferative disorders AML AML
Lymphoid
progenitor Tlymphocytes
Plasmacells
Blymphocytes
nanaïïveve
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Bone Marrow Aspiration/BiopsyBone Marrow Aspiration/Biopsy
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B-cell developmentB-cell development
stem
cell
lymphoid
progenitor
progenitorB
preB
immature
Bcell
memory
Bcell
plasma cellplasma cell
DLBCL,
FL, HL
ALL
CLL
MM
germinalgerminal
center center
BcellBcell
mature
naive
Bcell
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Disease states correlate with stages in normal B-cell development
Diseases: AML ProBALL PreBALL BCLL!LBCL"LLBLMantle Cell lymphoma
Marginal #one lymphomaMALT$ALTHodg%ins &'(
BALL M$)*
Multiple MyeloPlasmacytoma
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Chronic lymphocytic
leu%emia
*mall lymphocytic lymphoma
+aldenstr,m-s macroglo.ulinemia
"ollicular lymphoma
Bur%itt-s lymphomaMantle /one
lymphoma
*0/ary syndrome
Mycosis fungoides
Peripheral T cell
lymphoma
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Lymphocytic Leuemia !Lymphocytic Leuemia !
LymphomaLymphoma
LeuemiaLeuemia" widespread in#o$#ement o% BM and" widespread in#o$#ement o% BM and
presence o% $ar&e num'er o% tumor ce$$s inpresence o% $ar&e num'er o% tumor ce$$s in
periphera$ '$oodperiphera$ '$ood
LymphomaLymphoma" $ymphoid pro$i%erations as discrete" $ymphoid pro$i%erations as discrete
tissue massestissue masses
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Lymphoma classifcationLymphoma classifcation(2001 WHO)(2001 WHO)
B(ce$$ neop$asmsB(ce$$ neop$asmsprecursorprecursor
maturemature
)(ce$$ ! N*(ce$$ neop$asms )(ce$$ ! N*(ce$$ neop$asmsprecursorprecursor
maturemature
Hod&in $ymphomaHod&in $ymphoma
Non
Hodg%inLymphomas
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Lymphomas
Hodg%insNon Hodg%ins
B Cells Nodular sclerosing 123
Mi4ed cellularity 562723
T Cells
813 913
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Hod&in DiseaseHod&in Disease
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Hodgkin DiseaseHodgkin Disease
DefnitionDefnitionneoplastic diso!de! "ith development o#neoplastic diso!de! "ith development o#
specifc inflt!ate containing pathologicspecifc inflt!ate containing pathologic
$eed-%te!n&e!g cells$eed-%te!n&e!g cells' t sally a!ises in' t sally a!ises in
lymph nodes and sp!eads to contigoslymph nodes and sp!eads to contigosg!ops' *+t!anodal p!esentation a!e !a!e'g!ops' *+t!anodal p!esentation a!e !a!e'
Disease is associated "ith de#ectiveDisease is associated "ith de#ective
cellla! immnity'cellla! immnity'
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REED-!ER"BER# $ R% CellREED-!ER"BER# $ R% Cell
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Hodgkin DiseaseHodgkin Disease
ncidencencidence
- 2-, cases pe! 100000 poplation yea!- 2-, cases pe! 100000 poplation yea!
- &imodal age dist!i&tion - &imodal age dist!i&tion
1.-/. yea!s and a&ove .0 yea!s1.-/. yea!s and a&ove .0 yea!s
- male p!edominance 1341- male p!edominance 1341
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+pidemio$o&y , Hod&in-s+pidemio$o&y , Hod&in-s
Bimoda$Bimoda$
Postu$atedPostu$ated
to ha#eto ha#ein%ectiousin%ectious
associationassociation
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Classical Hodg&in L'mphoma
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5linical 6!esentation5linical 6!esentation 7ontende! lymph nodes enla!gement ( localised )7ontende! lymph nodes enla!gement ( localised )
neck and sp!aclavicla! a!eaneck and sp!aclavicla! a!ea 80-90:80-90: mediastinal adenopathymediastinal adenopathy .0:.0:
othe! ( a&dominal3 e+t!anodal disease )othe! ( a&dominal3 e+t!anodal disease ) systemic symptoms (B symptoms)systemic symptoms (B symptoms) /0:/0:
#eve!#eve! night s"eatsnight s"eats ne+plained "eight loss (10: pe! 8 months)ne+plained "eight loss (10: pe! 8 months)
6el-*&stein #eve!6el-*&stein #eve!''
!elapsing3 high-g!ade #eve! that can !each 10.-108;3!elapsing3 high-g!ade #eve! that can !each 10.-108;3pe!iodicity o# 4-10 days' eve! spikes a&!pt in onsetpe!iodicity o# 4-10 days' eve! spikes a&!pt in onset
and !esoltionand !esoltion othe! symptomsothe! symptoms
#atige3 "eakness3 p!!its#atige3 "eakness3 p!!its cogh 3 chest pain3 sho!tness o# &!eath3 vena cava synd!omecogh 3 chest pain3 sho!tness o# &!eath3 vena cava synd!ome a&dominal pain3 &o"el dist!&ances3 ascitesa&dominal pain3 &o"el dist!&ances3 ascites &one pain&one pain
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(el-E)stein Fever
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In#esti&ationsCBPCBP ""
Anemia . normochromic / normocytic0 eosinophi$Anemia . normochromic / normocytic0 eosinophi$
neutrophi$ia0 $ymphopenianeutrophi$ia0 $ymphopenia +S1+S1 22 L3)( .$i#er in4$ / o's at porta hepatisL3)( .$i#er in4$ / o's at porta hepatis13)( prior to treatment13)( prior to treatmentUrate 0 5a0Urate 0 5a0 LDH ( ad#erse pro&nosisLDH ( ad#erse pro&nosisCXR- mediastinal massCXR- mediastinal massCT thorax / abdomen / pelvis-for stagingCT thorax / abdomen / pelvis-for stagingOther: Gallium san! P"T!Other: Gallium san! P"T! Lymphan&io&raphy 0Lymphan&io&raphy 0
LaporotomyLaporotomy +6cisiona$ 'iopsy o% a $ymph node+6cisiona$ 'iopsy o% a $ymph node
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*tage * *tage ** *tage *** *tage *+
%taging o# lymphoma%taging o# lymphoma
A: a.sence of B symptoms
B: fever; night s
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A practica$ way to thin o% $ymphomaA practica$ way to thin o% $ymphoma
Categor' rvival o
ntreated
patients
Cra)ilit' !o treat or
not to treat
"on-Hodg&in
l'mphoma
=ndolent >ears $enerally not
cura.le
Generally defer
Rx if
asymptomatic
Aggressive Months Cura.le in
some
Treat
?ery
aggressive
+ee%s Cura.le in
some
Treat
Hodg&inl'mphoma
All types ?aria.le 5months to
years
Cura.le inmost
Treat
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Non Hod&in LymphomaNon Hod&in Lymphoma
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"ormer "irst Lady
@acueline ennedy nassis
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ing Hussein of @ordan
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*mall lymphocytic =mmuno.lastic
Mantle cell Large Cell
Types of NonHodg%in-s Lymphoma
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+tio$o&y o% NHL+tio$o&y o% NHL
Immune suppressionImmune suppressioncon&enita$ .7isott(A$drichcon&enita$ .7isott(A$drich
or&an transp$ant .cyc$osporineor&an transp$ant .cyc$osporine
AIDSAIDS
increasin& a&eincreasin& a&e
DNA repair de%ectsDNA repair de%ectsata6ia te$an&iectasiaata6ia te$an&iectasia
6eroderma pi&mentosum6eroderma pi&mentosum
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+tio$o&y o% NHL+tio$o&y o% NHL
5hronic in8ammation and anti&enic stimu$ation5hronic in8ammation and anti&enic stimu$ation#eliobater p$lori#eliobater p$lori in8ammation0 stomachin8ammation0 stomachChlam$dia psittaiChlam$dia psittai in8ammation0 ocu$ar adne6a$in8ammation0 ocu$ar adne6a$
tissuestissues
S9:&ren-s syndromeS9:&ren-s syndrome;ira$ causes;ira$ causes+B; and Buritt-s $ymphoma+B; and Buritt-s $ymphomaH)L;(I and ) ce$$ $euemia($ymphomaH)L;(I and ) ce$$ $euemia($ymphoma
H)L;(; and cutaneous ) ce$$ $ymphomaH)L;(; and cutaneous ) ce$$ $ymphomaHepatitis 5Hepatitis 5
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Crrent p ront treatment regimens or aggressive l'mphomas
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Regimen Drgs
CH.( Cyclophosphamide; !o4oru.icin; ?incristine; Prednisone
BAC.( Bleomycin; !o4oru.icin; Cyclophosphamide; ?incristineDPrednisone
M-BAC.D Methotre4ate; Leucovorin; Bleomycin; Cyclophosphamide;
?incristine; !e4amethasone
(roMACE/M.(( Prednisone; Methotre4ate; Leucovin; !o4oru.icin;Cyclophosphamide; Etoposide
MAC.(-B Methotre4ate; Leucovorin; !o4oru.icin; Cyclophosphamide;?incristine; Bleomycin; Prednisone; Trimethoprimsulfametho4a/ole
Crrent p-ront treatment regimens or aggressive l'mphomas
&)sed at various doses;
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)reatment Options in )reatment Options in
Ad#anced Indo$ent LymphomasAd#anced Indo$ent Lymphomas
O'ser#ation on$y" 1itu6an0 Be66ar0
?e#a$in
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Age Average age is FGDG
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Mu$tip$e Mye$omaMu$tip$e Mye$oma
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Mu$tip$e Mye$omaMu$tip$e Mye$oma
More than @ p$asma ce$$s in the 'oneMore than @ p$asma ce$$s in the 'one
marrow
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5$inica$ 3eatures5$inica$ 3eatures
Bone marrow %ai$ureBone marrow %ai$ure
( Anemia0 throm'ocytopenia0( Anemia0 throm'ocytopenia0
neutropenianeutropenia
1ena$ %ai$ure1ena$ %ai$ure
Bone disease with se$eta$ destructionBone disease with se$eta$ destruction
$ytic $esions$ytic $esions
&enera$iFed decrease in 'one density&enera$iFed decrease in 'one density
Hyperca$cemiaHyperca$cemia
Di i d St iDi i d St i
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Dia&nosis and Sta&in&Dia&nosis and Sta&in&
7orup7orupBone marrow 'iopsy and aspirateBone marrow 'iopsy and aspirate
SerumSerum protein e$ectrophoresis andprotein e$ectrophoresis and
immuno46ationimmuno46ation
Se$eta$ sur#eySe$eta$ sur#eyP$ain 6(rays are 'etter than 'one scan
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*erum Protein Electrophoresis
Total protein GDF αF glo.ulin 2D1
al.umin 7D1 β glo.ulins 2DG α8 glo.ulin 2D81 γ glo.ulin 8D7
Total protein GDJ αF glo.ulin 2DI
al.umin 6DJ β glo.ulins 2DGα8 glo.ulin 2D8J γ glo.ulin FD7
Normal Monoclonal *pi%e
Al)0 1 2 β γAl)0 1 2 β γ
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Monoc$ona$ Immuno&$o'u$inMonoc$ona$ Immuno&$o'u$in SpieSpie
on Serum Protein +$ectrophoresison Serum Protein +$ectrophoresis
.SP+P.SP+P Mu$tip$e mye$omaMu$tip$e mye$omaNon(Hod&in-s $ymphomaNon(Hod&in-s $ymphoma
Monoc$ona$ &ammopathy o%Monoc$ona$ &ammopathy o%
undetermined si&ni4cance .MUS
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http:872DF18D1D82FPathologyO=mageshttp: