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 ; $  xph0e0e?Rectangle 3D 0 N`b/ Si absence de RHC 3 mois : doubler la dose d Imatinib (800 mg/j) et nouvelle valuation 3 mois plus tard. Si RHC( 6 mois du dbut) : poursuite du traitement dose renforce adapte la tolrance hmatologique et valuation cytogntique 6 mois plus tard (12 mois du dbut de traitement) [M12]. si chec du traitement ( 6 mois du dbut du Ttt [M6]: - Allogreffe de moelle si donneur HLA-identique et ge < 40 ans. - Arrt de l Imatinib dans les autres situations (ge 40 ans et absence de donneur). 0  x"[! 1 33___PPT10i. `k+D=' = @B +"8 ?7p((  ( (  x`ph0e0e?Rectangle 2 `}  0 E  ( F (  xrh0e0e?Rectangle 3]] 0 zAdaptation du traitement en fonction de la rponse cytogntique 6 mois du dbut de traitement [M6]: Dfinition : . Rmission cytogntique complte(RCC)[0%de mitose Ph+] . Rponse cytogntique majeure (RCM):[< 35 %de mitoses Ph+]: . Rponse cytogntique mineure (RCm: 65 % mitoses Ph+): H &  -  nf P ( Nsh?Rectangle 4uDVB tPrise en charge LMC en 1re phase chronique ( 75 ans) : L; - ;  33___PPT10i. k+D=' = @B +"M TL,4(  , ,  xth0e0e?Rectangle 2  0 vPrise en charge LMC en 1re phase chronique ( 75 ans) : >< - <F ,  xuh0e0e?Rectangle 3 0 0 zXAdaptation du traitement en fonction de la rponse cytogntique 6 mois du dbut de traitement [M6]: a/ Rmission cytogntique complte(RCC) : . Poursuite du traitement la mme dose (400 mg/j) . . valuation cytogntique: 1 fois /an. [M18, M30& ] . valuation quantitative du transcrit BCR-ABL: 1 fois / 3 mois si rduction tumorale 3 log. 1 fois / 6 mois si rduction tumorale > 3 log. n > x xn,g f(2  33___PPT10i.g4a+D=' = @B +" 0(  0 0  x`vh0e0e?Rectangle 2 `}  0 :   0  xwh0e0e?Rectangle 3 0 Adaptation du traitement en fonction de la rponse cytogntique 6 mois du dbut de traitement [M6]: b/ Rponse cytogntique majeure (RCM): [<35% de mitoses Ph+]: - Poursuite de l Imatinib la mme dose. - valuation cytogntique 12 mois [M12] puis 1 fois/an [M24& ]. - Pas d valuation molculaire quantitative sauf si obtention d une rponse cytogntique complte    nAg f:   p 3380___PPT10.C =W"    4(  4 4  xyh0e0e?Rectangle 2 `}  0 :   4  x zh0e0e?Rectangle 3Y 0 Adaptation du traitement en fonction de la rponse cytogntique 6 mois du dbut de traitement [M6]: c / Rponse cytogntique mineure : - Doubler la dose de l Imatinib (800 mg/j) et l ajuster en fonction de la tolrance hmatologique et biologique. - valuation cytogntique 6 mois plus tard [M12]. RCC ou RCM: poursuite du traitement en l adaptant en fonction de la tolrance. en l absence de RCC ou RCM: poursuivre le traitement pendant 12 mois et nouvelle valuation cytogntique[M24]. Si toujours pas de rponse cytogntique : Allogreffe de moelle si ge < 40 ans et donneur HLA- Identique. Arrt de l Imatinib dans les autres situations. k Pc  k&g ZS1  33___PPT10i. +D=' = @B +" 8(  8 8  xzh0e0e?Rectangle 2  0 @Prise en charge LMC en 1re phase acclre : Dfinie selon les critres de l IBMTR ( International Bone Marrow Transplantation Registry). Ncessit de renforcer le traitement pour contrler le taux de globules blancs Doublement rapide des GB ( < 5 jours) Somme des blastes et des promylocytes 20% dans le sang ou la moelle Somme des basophiles et des osinophiles 20% dans le sang Anmie ou thrombopnie non lie au Busulfan ou l hydroxyure Thrombocytose persistante Anomalies chromosomiques additionnelles ( Trisomie8, isochromosome 17, Deuxime chromosome Philadelphie ). Majoration de la splnomgalie Apparition de chlorome ou d une mylofibrose.s P  -.r  33___PPT10i.qW+D=' = @B +"  <(  < <  x`0e0e?Rectangle 2 `}  0 :   <  x`0e0e?Rectangle 3 0 PRISE EN CHARGE DE LA LMC EN PHASE ACCELEREE: * Sujets 40 ans , ayant un donneur HLA-identique - Imatinib (600  800 mg/j) pendant 3 mois. - Puis allogreffe de moelle osseuse. * Sujet > 40 ans et /ou n ayant pas de donneur HLA-identique - Imatinib (600  800 mg/j). - valuation de la rponse selon les mmes critres que la LMC en 1re phase chronique.1 P5  z . )z   w 33___PPT10i.C p@+D=' = @B +" @(  @ @  x/0e0e?Rectangle 2 `}  0 :  p @  x,0e0e?Rectangle 3 0 Prise en charge de la LMC en phase blastique: Phase d acutisation : Dfinie par plus de 20% de blastes dans la moelle avec apparition de signes gnraux, d un syndrome tumoral ( adnopathie, hpatomgalie) ou de signes d insuffisance mdullaire ( anmie ou thrombopnie ). - Pas d Imatinib. - Pas d allogreffe. - Traitement palliatif. P  PA 1> ^ 33___PPT10i.D +D=' = @B +": )!D (  DI D  x`,0e0e?Rectangle 2Zj 0 }1Prise en charge de la LMC en phase chronique : 2 2 2 D  x@+0e0e?Rectangle 3  0 N"Allogreffe en 1re intention 30 ans + donneur HLA-id + Gratwohl 2 Allogreffe de MO o Z# Z f 6   D `& ?Rectangle 6J p N   xB D PfԔ?Line 7   33___PPT10i.= r+D=' = @B +"? F>%%H&(  H H  x 1m0e0e?Rectangle 4 `}  0 :  4 H  x5m0e0e?Rectangle 5w I 0 h Imatinib 400 mg/j RHC 3 mois(M3) 400mg/J Rponse cytogntique 6 mois (M6) Complte Majeure Mineure 400mg/j 400mg/j 800mg/j Rep.cyto 12 mois (M12) Complte Majeure Mineure Bcr-Ablqt EV cytog (24M) - Allo si <40ans si RCC et donneur HLA-id Bcr-Ablqt - Si non arrt Imatinib 400mg/j 400mg/j 6* P) P% Pg P P P P$ P IX: A H  x6m0e0e?Rectangle 6@  0 u 0 P  xB H@ Pfo?Line 7 :: xB H Pfo?Line 8ggxB H Pfo?Line 9Mgg/zB H Rfo?Line 10ggzB  H Rfo?Line 11ggzB  H@ Rfo?Line 12gzB  H Rfo?Line 13gzB  H Rfo?Line 142ggzB  H Rfo?Line 152TTzB H Rfo?Line 163 zB H Rfo?Line 17  S zB H@ Rfo?Line 18 G c zB H Rfo?Line 19 c zB H Rfo?Line 20 ::c zB H Rfo?Line 21  zB H Rfo?Line 22  zB H Rfo?Line 24 ''v; H J?Rectangle 78m 5 e Imatinib 400 mg/j Echec RHC 3 mois(M3) 800mg/J Rponse Hmato 6 mois (M6) Complte chec 800mg/j selon - Allo si donneur HLA-id tolrance et ge < 40 ans - sinon arrt Imatinib EV.Cytog 12 mois(M12) puis 1 fois/an RCC RCM RCM BCR-ABLqt EV cytog(24M) - Allo si <40 ans si RCC et donneur HLA-id Bcr-abl qt - Si non arrt Imatinib 400mg/j 400mg/j /8 P)0 P8 P0 P0 P$0 P f< fzB H Rfo?Line 79FFzB H Rfo?Line 80zzB H@ Rfo?Line 81PpzB H Rfo?Line 82PzB H Rfo?Line 84V e tB H Lf?Line 90~ ~ tB H Lf?Line 92 zB H Rfo?Line 93) f zB H Rfo?Line 94  tB  H Lf?Line 95  zB !H Rfo?Line 96) 9 tB "H Lf?Line 97,tB #H Lf?Line 98 |B $H Tfo?Line 100S c |B %H Tfo?Line 101 33___PPT10i.`Ƶ+D=' = @B +"  L (  L L  x4m0e0e?Rectangle 2 `}  0 C   r L  x0e0e?Rectangle 30 0 LMC en phase avance (75 ans) LMC ACCELEREE Age 40 ans Age >40 ans et donneur HLA-id et/ou absence de donneur HLA-id Imatinib 600  800 mg/j Imatinib 600  800 mg (x 3 mois) Allogreffe Evaluation rponse (voir LMC phase chronique)! P3 P P P! PNh rB L JfԔ?Line 40 0 rB L JfԔ?Line 5*xB L PfԔ?Line 6**mxB L Pfo?Line 7m L `Omf8c?Rectangle 8 3 / N   rB  L JfԔ?Line 9/0 0   L bmf|?Rectangle 10mU-2 N     L bmfo?Rectangle 11mm  N   zB  L RfԔ?Line 122**zB  L RfԔ?Line 13zB L RfԔ?Line 14  zB L RfԔ?Line 15   33___PPT10i. Ύ+D=' = @B +"s bZPB(  Pt P  x{h0e0e?Rectangle 2 `}  0 <LMC en phase avance (75 ans)>   P  x)0e0e?Rectangle 3 ` 0 BLMC EN PHASE BLASTIQUE Pas d allogreffe Pas d Imatinib Ttt palliatif H L  f gxB P PfԔ?Line 4  2 33___PPT10i. pދ+D=' = @B +"  T(  TQ T  x`3m0e0e?Rectangle 2 `,  0 !ALLOGREFFE DANS LMC : INDICATIONS6" f( f "j T  xm0e0e?Rectangle 3 0 1/ Phase chronique : a/ 30 ans + donneur HLA-id + Gratwohl 2 (en premire intention) b/ Absence de RHC M6 : - ge < 40 ans - donneur HLA-id : quelque soit le Gratwhol c/ Absence de RCC ou RCM M24 : Si ge < 40 ans + donneur HLA-id : quelque soit le Gratwhol 2/ Phase acclre : - Age 40 ans - Donneur HLA-id - Aprs Imatinib 600 800mg/j pendant 3 mois . R +0  3380___PPT10./Bg"r`pɗb EL