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: