SMD 2014
description
Transcript of SMD 2014
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SINDROAME MIELODISPLAZICE
Dr Mariana Patiu
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Displazie/mielodisplazieDisplazie/mielodisplazie– DiseritropoiezaDiseritropoieza– DisgranulocitopoiezaDisgranulocitopoieza– DismegacariopoiezaDismegacariopoieza– Displazie secundara/primaraDisplazie secundara/primara
CitopeniiCitopenii
CitogeneticaCitogenetica
Genetica molecularaGenetica moleculara
EpigeneticaEpigenetica
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DEFINITIE:
Sindromul mielodisplazic reprezinta un grup heterogen de:
afectiuni hematologice
maligne,
dobandite,
clonale,
ale celulei stem pluripotente,
caracterizate prin:
-hematopoieza ineficienta de diverse grade - citopenii progresive si persistente
-modificari displazice ale liniilor mieloide,
-potential major de evolutie spre leucemie acuta
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ISTORICISTORIC
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ISTORIC
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Sindroamele mielodisplazice: sinonimeSindroamele mielodisplazice: sinonime
Preleucemia sau anemia preleucemica;Preleucemia sau anemia preleucemica;
Hemopatii mieloide indolente;Hemopatii mieloide indolente;
Leucemii latente acute;Leucemii latente acute;
Leucemie subacuta sau leucemie mieloida latenta;Leucemie subacuta sau leucemie mieloida latenta;
Oligoleucemia;Oligoleucemia;
Anemia pseudoaplasticaAnemia pseudoaplastica
MielodisplazieMielodisplazie
Displazie hematopoieticaDisplazie hematopoietica
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Criterii pentru citopenii*Criterii pentru citopenii*
Hemoglobina < 10 g/dlHemoglobina < 10 g/dl
Neutrofile (val. abs) < 1,8 x10Neutrofile (val. abs) < 1,8 x1099/L/L
Trombocite < 100 x 10Trombocite < 100 x 1099/L/L
* International Working Group for the Prognosis of MDS (IWG-PM) * International Working Group for the Prognosis of MDS (IWG-PM)
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Modificari morfologice in SMD Modificari morfologice in SMD Seria eritrocitara Tablou sanguin Frotiu medular
Anemie*
* Cea mai frecventa citopenie
MacrocitozaNormocitozaMicrocitoza*Normocromie,DimorfismPoikilociteCorpi PappenheimerPunctatii bazofileEritroblasti circulanti(displazici± inelari)ReticulocitopenieReticulocitoza-rar•Hgpatie H sau •α sau ß talasemie dobandita
HiperplazicaHipoplazica/aplazica (f. rar)SE normo-macro- megaloblasticaDefecte de hemoglobinizare Vacuole citoplasmaticeBi/multinuclearitatePunti internucleareNuclee lobulate/inmuguritePunctatii bazofileEritroblasti inelari
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diseritropoieza
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diseritropoieza
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diseritropoieza
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diseritropoieza
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Modificari morfologice in SMD Modificari morfologice in SMD
Seria granulocitara
Tablou sanguin Frotiu medular
Neutropenie
An. pseudo Pelger HuetCondensare anormalaProiectii nucleare multipleFragmente de nucleu detasateDegranulare partiala/totalaGranule giganteCitoplasma bazofila in Sg Corpi DohleEozinopenie,BazofilopenieEozinofilie, bazofilie-rarMonocitoza > 1x 109/L (displazie)Blasti ± corpi AuerHipersegmentare
HiperplazicaDefecte de granulare -degranulare -granule aberanteDefecte de condensare comatinianaAnomalie pseudoPelger HuetMMC gigante -rarBlasti cu corpi AuerPMC hipo/hipergranulareMonocite/promonociteFrecvente mastocite
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disgranulopoieza
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disgranulopoieza
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disgranulopoieza
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disgranulopoieza
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disgranulopoieza
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Modificari morfologice in SMD Modificari morfologice in SMD
Seria megacariocitara
Tablou sanguin Frotiu medular
TrombocitopenieTrombocitoza, Nr normal
AnizotrombocitozaMegalotrombociteTrombocite degranulateMicomegacariocite
SMkc:normo/hiperplazica, rar hipoplazicaMicromegacariociteNuclee nelobulate (5q-)Nuclee bizare-botrioideMono/binucleereMultiple fragmente nelegateAnomalii de granulare
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dismegacariopoieza
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dismegacariopoieza
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Criterii de diagnostic pentru SMDCriterii de diagnostic pentru SMD
CITOPENIE*CITOPENIE* – Hemoglobina < 10 g/dlHemoglobina < 10 g/dl– Neutrofile (val. abs) < 1,8 x10Neutrofile (val. abs) < 1,8 x1099/L/L– Trombocite < 100 x 10Trombocite < 100 x 1099/L/L
++ DISPLAZIE DISPLAZIE
>10% din celule displazice in linia citopenica >10% din celule displazice in linia citopenica * persistente 6 luni daca nu exista o anomalie genetica* persistente 6 luni daca nu exista o anomalie genetica
persitente 2 luni daca este bicitopeniepersitente 2 luni daca este bicitopenie2525
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Teste diagnosticeTeste diagnostice
A. HemogramaA. Hemograma
B. Frotiu sanguinB. Frotiu sanguin
C. Frotiu medular-MedulogramaC. Frotiu medular-Medulograma
D. Biopsie osteomedularaD. Biopsie osteomedulara
E. Citochimie E. Citochimie (peroxidaza, r Perls’)(peroxidaza, r Perls’)
F. ImunofenotipareF. Imunofenotipare
G. GeneticaG. Genetica
H. BiochimieH. Biochimie
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A HemogramaA Hemograma CitopeniiCitopenii
AnemieAnemie
Neutropenie absolutaNeutropenie absoluta
TrombocitopenieTrombocitopenie
Monocitoza absoluta ( >1000/microL)Monocitoza absoluta ( >1000/microL)
TrombocitozaTrombocitoza
MacrocitozaMacrocitoza
Anizocitoza eritrocitaraAnizocitoza eritrocitara
AnizocromieAnizocromie
AnizotrombocitozaAnizotrombocitoza
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B , C frotiu sanguin/aspirat medular
vor fi examinate pentru:evidentierea modificarilor displaziceaprecierea procentului de blasti;
Se considera blasti: - mieloblastii
- monoblastii - megacarioblastii
- promonocitele sunt “ echivalenti blastici” - NU eritroblastii ( exceptie LAM6)
- NU se recomanda considerarea celulelor CD34+ evidentiate la citometrie drept blasti !!
monocitesideroblasti inelari – coloratia Perls’
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D. Biopsia osteo-medulara (BOM)
BOM de obicei nu este necesara BOM poate exclude afectiuni confundabile cu SMD:
HCL, metastaze, LNH etcBOM ofera material pentru IHC si biologie molecularaBOM poate aduce date despre
-celularitatea MO - SMD hipoplazic -fibroza: SMD - F-displazia megacariocitelor-afectarea arhitecturii medulare - ALIP
(abnormal localization of immature precursors)
-% blasti
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Precursorii imaturi localizati anormal intertrabecularNormal paratrabecular si perivascular
Smkc displazica si plasmocite CD 138 +
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F. IMUNOFENOTIPIZAREA
Informatii putine– apartenenta blastilor – Identificarea Mkc foarte displazice
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In SMD cu displazie neconvingatoare citometria in flux demonstreaza– Defecte de granulare ale neutrofilelor– Densitate antigenica anormala– Desincronizarea expresiei antigenica– Expresie anormala antigenica*
Reducerea expresiei CD45
Lipsa de expresie a HLA-DR
Expresie aberanta de CD5,CD7,CD16.CD33
Expresie anormala antigenica pe celule mature* care semnifica defecte in maturatie si NU sunt specifice SMD; se descriu si in sepsis, lupus, alte afectiuni benigne cu displazie
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TESTE BIOCHIMICE
Modificari necaracteristice
Feritina- valori crescute- se coreleaza cu prognostic rezervat
Sideremie – valori crescuteSaturatia transferinei-val crescuteLHD - valori crescute in SMD stadii avansate
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GENETICANu exista anomalii citogenetice recurente in SMD
Modificarile genetice nu sunt descrise in toate cazurile de SMD si deci nu pot fi baza pentru diagnostic; 50% din SMD primare si 70 % din SMD sec au anomalii genetice
-Displazia < 10% celule in 1 sau mai multe linii + anomalie citogenetica intilnita in SMD permite dg de SMD-U
-del 5q- este singura anomalie - criteriu de dg
-indiferent de alte modificari, anomaliile genetice recurente pentru LAM (PML/RARA [t(15;17)], RUNX1/RUNX1T1 [t(8;21)], si CBFbMYH11 [inv(16) sau t(16;16)] ) impun dg de LAM indiferent de numarul de blasti
Jeffrey E. Lancet, Ling Zhang,:Understanding the Pathology of Myelodysplastic Syndromes to Guide Treatment
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Unbalanced Aberrations
Balanced Translocation
-5 or del(5q)-7 or del(5q)
i(17q) or t(1;17p)-13 or del(13q)
del(11q)del(12p) or t(12p)
del(9q)idic(X)(q13)
t(1;3)(p36;q21.1)t(3;21)(q26.2;q22.1)
t(2;11)(p21;q23)inv(3)(q21q26.2)t(6 ;9)(p23;q34)
t(11 ;16)(q23;p13.3)
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Mutatii “recurente” NPM1 - (frecv in AML) –descrisa la 2% SMDTP53, TET2, ASXL1, RUNX1, CBL, JAK2, EZH2, IDH1,IDH2, Ras, BRAF
(TP53, EZH2, ETV6, ASXL1, si RUNX1 au semnificatie prognostica)
SF3B1 de pe cr. 2q33.1 descrisa in SMD cu sideroblasti inelari,U2AF35, ZRSR2, si SRSF2 –mutatii descrise in 45% - 85% din neoplaziile mieloide cu mielodisplazie. DNMT3A –se descrie rar in SMD dar se asociaza cu prognostic sever- se asoc. cu transformare in LAM
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Patogeneza SMDPatogeneza SMD
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SMD este un proces “multi -step” celulele stem micromediul medularsistemele de semnalizare–interactiunea dintre ele
Teorii si cai patogenetice:
1)- afectare celulara determinata de : toxice
genotoxicenon genotoxice
imbatranire2)- anomalii genetice
celulare, moleculare, epigenetice determina pierdere sau castig de functii
3)- modificari ale micromediului4)- dereglari imune5)- alterarea ciclului celular si blocarea diferentierii
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FIZIOPATOLOGIA SMDFIZIOPATOLOGIA SMDAPOPTOZAAPOPTOZA
SMD=citopenii+displazie+MO hipercelularaSMD=citopenii+displazie+MO hipercelulara2 mecanisme majore perturba celulele hematopoietice mieloide:2 mecanisme majore perturba celulele hematopoietice mieloide:
►► defect in capacitatea de diferentiere defect in capacitatea de diferentiere si si
► ► exces de apoptozaexces de apoptozaApoptoza determina:Apoptoza determina:
modificari morfologicemodificari morfologice fragmentare ADNfragmentare ADN proteolizaproteoliza modificari ale membranei plasmaticemodificari ale membranei plasmatice
Apoptoza =mai exprimata in stadiile incipiente ale SMDApoptoza =mai exprimata in stadiile incipiente ale SMD
Citopeniile sunt rezultatul balantei dintre diferentiere si apoptozaCitopeniile sunt rezultatul balantei dintre diferentiere si apoptoza
Scaderea apoptozei cel. clonale CD34+ explica progresia spre LAScaderea apoptozei cel. clonale CD34+ explica progresia spre LA
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FIZIOPATOLOGIA SMDFIZIOPATOLOGIA SMDsumarsumar
Factori implicati in fiziopatologia SMD:Factori implicati in fiziopatologia SMD:
dezechilibrul dintre apoptoza si proliferarea celulelor dezechilibrul dintre apoptoza si proliferarea celulelor anormale;anormale;
instabilitatea genomica;instabilitatea genomica; metilarea ADN anormala si alte modificari metilarea ADN anormala si alte modificari
epigenetice epigenetice (hipermetilare ADN, acetilare histonica);(hipermetilare ADN, acetilare histonica); disfunctii imunologice;disfunctii imunologice; toxicitate medulara directa;toxicitate medulara directa; factori angiogenetici si stromalifactori angiogenetici si stromali
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Sindroamele mielodisplazice:Sindroamele mielodisplazice: incidenta incidenta
Afecteaza in special Afecteaza in special varstniciivarstnicii (varsta medie = 69 ani)(varsta medie = 69 ani)
Incidenta Incidenta – generala : 4 / 100.000 generala : 4 / 100.000 – >70 ani → >30 / 100.000>70 ani → >30 / 100.000
SMD sunt SMD sunt cele mai frecvente dintre malignitatile mieloide cele mai frecvente dintre malignitatile mieloide Incidenta SMD creste major cu cresterea duratei de viata Incidenta SMD creste major cu cresterea duratei de viata
(tarile dezvoltate) (tarile dezvoltate) Sunt rare la copii (controversat)Sunt rare la copii (controversat)
SMD SMD considerate nevindecabile;considerate nevindecabile; transplantul alogenic de celule stem are potential curativ si este benefic transplantul alogenic de celule stem are potential curativ si este benefic
pentru pentru supravietuire – limitare datorita varstei si comorbiditatilorsupravietuire – limitare datorita varstei si comorbiditatilor ! !
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SMD se impart in:
► SMD primare (de novo)
► SMD secundare:
- post radio/chimioterapie agenti alchilanti
inhibitori de topoizomeraza
- expunere la toxice profesionale benzen
- boli congenitale asociate cu SMD anemia Fanconi
sindrom Down4444
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Sindroamele Sindroamele mielodisplazice Anomalii citogenetice Prognostic
Trisomii - 8 frecventa; raspunde bine la imunoterapie Trisomii - 8 frecventa; raspunde bine la imunoterapie
Prognostic bun: del(5q), del(20q), -YPrognostic bun: del(5q), del(20q), -Y
Prognostic rezervat: anomalii complexe(>3), anomalii ale crom 7 Prognostic rezervat: anomalii complexe(>3), anomalii ale crom 7
Prognostic intermediar: restul Prognostic intermediar: restul
SubtipSubtip Cazuri (%)Cazuri (%) Anomalii citogeneticeAnomalii citogenetice
AR 25 del(20q), -Y, +8, del(5q), -7 AR 25 del(20q), -Y, +8, del(5q), -7
ARSI 10 ARSI 10
CRDM 50 del(5q), -7, +8CRDM 50 del(5q), -7, +8
CRDM-SI 50 del(5q), -7, +8 CRDM-SI 50 del(5q), -7, +8
AREB-1 50 del(5q), -7, +8, del(20q)AREB-1 50 del(5q), -7, +8, del(20q)
AREB-2 50-75 del(5q), -7, +8, 17p-, del(11q),AREB-2 50-75 del(5q), -7, +8, 17p-, del(11q),
t(11q 23), -13/del(13q)t(11q 23), -13/del(13q)
SMD-del(5q) 100 del(5q)SMD-del(5q) 100 del(5q)
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Sindroamele mielodisplaziceSindroamele mielodisplaziceClasificarea FAB(1982)Clasificarea FAB(1982) - -se bazeaza pe modificari morfologice periferice si centrale se bazeaza pe modificari morfologice periferice si centrale
Subtip FAB %blasti MO %blasti Sp
AR(anemie refractara)
<5 ≤1
ARSI*(anemie refractara cu sideroblasti inelari)*sideroblasti inelari>15%
<5 ≤1
AREB(anemie refractara cu exces de blasti )
5-20 <5
AREB-t**(anemie refractara cu exces de blasti –in transformare)**corpi Auer
>20-30 ≥5
CMML***(leucemie mielo-monocitara cronica)***monocitoza
<5-30 <5
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Sindroamele mielodisplazice Sindroamele mielodisplazice Clasificarea OMSClasificarea OMS11
Entitatea Modificari sanguine Modificari medulare
Anemie refractara (AR) AnemieRari blasti ( sau fara )<1x109/L monocite
Diseritropoieza ( izolata)<10% el. disp. SG si SMkc<5% blasti <15% sideroblasti inelari
Anemie refractara cu sideroblasti inelari (ARSI)
AnemieFara blasti
Diseritropoieza ( izolata)<10% el. disp. SG si SMkc<5% blasti ≥15% sideroblasti inelari
Citopenii refractare cu displazie multiliniara(RCMD)
Citopenii( bi/pancitopenie)Rari blasti (sau fara)Fara corpi Auer<1x109/L monocite
Displazie ≥ 10% celule, 2 sau 3 linii<5% blasti in MOFara corpi Auer<15% sideroblasti inelari
Citopenii refractare cu displazie multiliniara si
sideroblasti inelari(RCMD-RS)
Citopenii( bi/pancitopenie)Rari blasti ( sau fara)Fara corpi Auer<1x109/L monocite
Displazie ≥ 10% celule, 2 sau 3 linii<5% blasti in MOFara corpi Auer≥15% sideroblasti inelari
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Sindroamele mielodisplazice Clasificarea OMSSindroamele mielodisplazice Clasificarea OMS22
Entitatea Modificari sanguine Modificari medulare
Anemie refractara cu exces de blasti -1 (AREB-1)
Citopenii<5% blasti Fara corpi Auer<1x109/L monocite
Displazie 1-2-3 linii5-9% blastiFara corpi Auer
Anemie refractara cu exces de blasti -2 (AREB-2)
Citopenii5-19% blasti corpi Auer+/-<1x109/L monocite
Displazie 1-2-3 linii10-19% blastiCorpi Auer +/-
SMD neincadrabil (MSD-U) CitopeniiFara blasti sau putini blastiFara corpi Auer
Displazie o linie: SG sau SMkc<5% blasti Fara corpi Auer
SMD cu 5q- Anemie<5% blastiNr normal sau crescut trombocite
SMkc N sau hiperplazica cu hipolobulare<5% blasti Fara corpi AuerDeletie 5q- izolata
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SMD:Diagnostic diferentialSMD:Diagnostic diferential
DiseritropoiezeDiseritropoieze::
Deficitul de vitamina B12Deficitul de vitamina B12
Deficitul de folati/acid folicDeficitul de folati/acid folic
Medicamente ce interfera cu sinteza de ADN:MTXMedicamente ce interfera cu sinteza de ADN:MTX
Talasemii, hemoglobinopatii, hemoglobine instabile Talasemii, hemoglobinopatii, hemoglobine instabile
Anemie diseritropoietica congenitalaAnemie diseritropoietica congenitala
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SMD:Diagnostic diferentialSMD:Diagnostic diferential22
Intoxicatia cu metale grele Intoxicatia cu metale grele – plumbplumb:punctatii bazofile, anemie microcitara, hemoliza, :punctatii bazofile, anemie microcitara, hemoliza,
sideroblasti inelarisideroblasti inelari– arsenicarsenic:pancitopenie, diseritropoieza, sideroblasti inelari:pancitopenie, diseritropoieza, sideroblasti inelari– zinculzincul determina deficit de cupru anemie leucopenie, determina deficit de cupru anemie leucopenie,
sideroblasti inelari, vacuolizarisideroblasti inelari, vacuolizari
Deficitul de cupruDeficitul de cupru::
-anemie leucopenie, sideroblasti inelari, vacuolizari-anemie leucopenie, sideroblasti inelari, vacuolizari
Citopatii mitocondriale Citopatii mitocondriale :sindromul Pearson::sindromul Pearson:
- debut in copilarie,pancitopenie, sideroblasti inelari, - debut in copilarie,pancitopenie, sideroblasti inelari, vacuole in eritroblasti si granulocitevacuole in eritroblasti si granulocite
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SMD:Diagnostic diferentialSMD:Diagnostic diferential33
Antibiotiotice si alte medicamenteAntibiotiotice si alte medicamente
Infectie virala Infectie virala (HIV) si tratamente antivirale :zidovudine(HIV) si tratamente antivirale :zidovudine
Inflamatii (hepatice ) neoplaziiInflamatii (hepatice ) neoplazii
PTIPTI
Anemie aplastica Anemie aplastica
-fara blasti in MO si TS, -fara blasti in MO si TS,
-fara fibroza -fara fibroza reticulinica, reticulinica,
-fara dismegacariopoieza-fara dismegacariopoieza
Mielofibroza primara sau acutaMielofibroza primara sau acuta
Infiltrate limfomatoase +displazieInfiltrate limfomatoase +displazie
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Sindroamele mielodisplaziceSindroamele mielodisplaziceInternational Prognostic Scoring System-IPSSInternational Prognostic Scoring System-IPSS
IPSS se determina in functie de:IPSS se determina in functie de:
- % blasti medulari;% blasti medulari;- Citogenetica: IPSS in del(5q) si Citogenetica: IPSS in del(5q) si
del(20q), IPSS in del(7q)del(20q), IPSS in del(7q)- Examenul sangelui (segmentate Examenul sangelui (segmentate
neutrofile, hematocrit, trombocite)neutrofile, hematocrit, trombocite)
Grupurile de risc IPSS: Grupurile de risc IPSS: - scazut;scazut;- Intermediar-1 (INT-1);Intermediar-1 (INT-1);- Intermediar-2 (INT-2);Intermediar-2 (INT-2);- Crescut Crescut
IPSS Scazut Intermediar Crescut
Citogenetica Del(5q), del(20q)
altele Del (7q)
Supravietuire(ani)
3,8 2,4 0,8
25% LAM(ani) 5,6 1,6 0,9
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IPSS-R Cytogenetic risk groups
Cytogenetic prognostic subgroups
Cytogenetic abnormalities
Very good -Y, del(11q)
Good Normal, del(5q), del(12p), del(20q), double including del(5q)
Intermediate del(7q), +8, +19, i(17q), any other single or double independent clones
Poor -7, inv(3)/t(3q)/del(3q), double including -7/del(7q), Complex: 3
abnormalities
Very poor Complex: >3 abnormalities
Greenberg, Tuechler, Schanz et al, Revised International Prognostic Scoring System (IPSS-R) for Myelodysplastic Syndrome, Blood 120: 2454, 2012.
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IPSS-R Prognostic Score ValuesPrognostic
variable0 0.5 1 1.5 2 3 4
Cytogenetics Very Good
Good Intermediate
Poor Very Poor
BM Blast % <=2 >2-<5% 5-10% >10%
Hemoglobin =>10 8-<10 <8
Platelets =>100 50-<100 <50
ANC =>0.8 <0.8
Greenberg, Tuechler, Schanz et al, Revised International Prognostic Scoring System (IPSS-R) for Myelodysplastic Syndrome, Blood 120: 2454, 2012
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SMD speciale
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Sindroamele mielodisplaziceSindroamele mielodisplaziceSindromul 5q-Sindromul 5q-
Deletia(5q) include banda q31Deletia(5q) include banda q31
Megacariocite mononucleateMegacariocite mononucleateAnemie macrocitaraAnemie macrocitaraTrombocitozaTrombocitozaLeucopenie moderataLeucopenie moderata
Predomina la femei, varstniciPredomina la femei, varstniciPrognostic favorabilPrognostic favorabilRata scazuta de transformare in LAMRata scazuta de transformare in LAM
FABFAB ARAR ARSIARSI AREBAREB AREB-tAREB-t
% 5q-% 5q- 5252 1111 1212 11
Sindromul 5q-(AR):Sindromul 5q-(AR):Anemie macrocitaraAnemie macrocitaraDiseritropoiezaDiseritropoiezaMicromegacariocite hipolobulateMicromegacariocite hipolobulate
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Sindroamele mielodisplaziceSindroamele mielodisplaziceLMMC. Clasificare OMSLMMC. Clasificare OMS
Afectiune mielodisplazica/mieloproliferativa (in functie Afectiune mielodisplazica/mieloproliferativa (in functie de numarul de leucocite: scazut respectiv crescut )de numarul de leucocite: scazut respectiv crescut )
Caracteristica obligatorie : monocite > 1x10Caracteristica obligatorie : monocite > 1x1099/L/L
2 subtipuri: 2 subtipuri:
- LMMC 1: <5% blasti (SP) si <10% (MO);- LMMC 1: <5% blasti (SP) si <10% (MO);
- LMMC 2: 5-19% blasti (SP) si 10-19% (MO)- LMMC 2: 5-19% blasti (SP) si 10-19% (MO)
LMMC proliferativ: leucocite > 12000/mmLMMC proliferativ: leucocite > 12000/mm³³(grup IPSS)(grup IPSS)
leucocite > 13000/mmleucocite > 13000/mm³(FAB)³(FAB)
LMMC proliferativ/displazic: fara diferente semnificative in LMMC proliferativ/displazic: fara diferente semnificative in prognostic si evolutia spre LAMprognostic si evolutia spre LAM
LMMC diplazic poate evolua spre LMMC proliferativLMMC diplazic poate evolua spre LMMC proliferativ
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SMD cu fibroza (SMD-F) SMD cu fibroza (SMD-F)
Aprox 15% cazuri de SMD-F;criterii de dg incomplet definiteAprox 15% cazuri de SMD-F;criterii de dg incomplet definite
Fara organomegaliFara organomegali
Fibroza→Aspirat Mo dificilFibroza→Aspirat Mo dificil
PancitopeniePancitopenie
Displazie ( cel putin 2 linii)Displazie ( cel putin 2 linii)
Dg diferential Dg diferential : : panmieloza acuta cu fibroza panmieloza acuta cu fibroza ((cel mai dificil):cel mai dificil):
fibroza+panmieloza+ 20-25% blasti in MO +fibroza+panmieloza+ 20-25% blasti in MO +
displazie multiliniala+pancitopenie+displazie multiliniala+pancitopenie+
rari blasti circulanti+morfologie eritrocitara normalarari blasti circulanti+morfologie eritrocitara normala
LAM :LAM7LAM :LAM7
BMPC (MMM)BMPC (MMM)
Boli autoimune, metastaze medulare, limfoame,HCLBoli autoimune, metastaze medulare, limfoame,HCL
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SMD hipoplazic/hipocelularSMD hipoplazic/hipocelularSMD hipoplazicSMD hipoplazic
<30% celularitate MO la pacienti <60 ani<30% celularitate MO la pacienti <60 ani
<20% celularitate MO la pacienti >60 ani<20% celularitate MO la pacienti >60 ani
Uneori dificil de diferentiat SMD de AA (diseritropoieza; Uneori dificil de diferentiat SMD de AA (diseritropoieza; procent de celule CD34+ procent de celule CD34+
scazut)scazut)
Pledeaza pentru SMDPledeaza pentru SMD::- anomalia pseudo Pelger Huet ± degranulareanomalia pseudo Pelger Huet ± degranulare- prezenta megacariocitelor ( displazice)prezenta megacariocitelor ( displazice)
- disgranulopoieza si dismegacariopoieza- disgranulopoieza si dismegacariopoieza- procent normal sau crescut de CD34+ in MOprocent normal sau crescut de CD34+ in MO
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NYMDSNYMDS
NQMDSNQMDS
ICUS =citopenie idiopatica cu semnificatie necunoscutaICUS =citopenie idiopatica cu semnificatie necunoscuta
IDUS= displazie idiopatica cu semnificatie necunoscutaIDUS= displazie idiopatica cu semnificatie necunoscuta
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