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Tiziano Barbui MD Ospedale Papa Giovanni XXIII Bergamo, Italy Bergamo, Italy

Emerging JAK Inhibitors in Myelofibrosis : Determining the Right Agent for the Right Patient .(Madrid) How to use prognosis assessment criteria for MF management in the clinical practice. Tiziano Barbui MD Ospedale Papa Giovanni XXIII Bergamo, Italy Bergamo, Italy.

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Tiziano Barbui MD Ospedale Papa Giovanni XXIII Bergamo, Italy Bergamo, Italy

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  1. Emerging JAK Inhibitors in Myelofibrosis: Determining the Right Agent for the Right Patient.(Madrid)How to use prognosisassessmentcriteria for MF management in the clinicalpractice Tiziano Barbui MD Ospedale Papa Giovanni XXIII Bergamo, Italy Bergamo, Italy

  2. FROM PV and ET to MYELOFIBROSIS: The value of bone marrowmorphology

  3. INITIAL BONE MARROW RETICULIN FIBROSIS IN POLYCYTHEMIA VERA EXERTS AN IMPACT ON CLINICAL OUTCOME (IWG-RT study) Tiziano Barbui1† , Jürgen Thiele,2† Francesco Passamonti,3 Elisa Rumi,4 Emanuela Boveri,4 Maria Luigia Randi,5 Irene Bertozzi,5 Filippo Marino,5 Alessandro M. Vannucchi,6 Elisabetta Antonioli,6 Valentina Carrai,6 Heinz Gisslinger,7Veronika Buxhofer-Ausch,7Leonhard Müllauer,8 Guido Finazzi,1 Alessandra Carobbio,1 Andrea Gianatti,1 Marco Ruggeri,9 Francesco Rodeghiero,9 Emanuele D’Amore,9 Alessandro Rambaldi,1 and Ayalew Tefferi,10 † 526 patients with strictly defined WHO diagnosis of PV Reviewer: JuergenThiele ;Participant centers (Bergamo, Pavia, Padova, Vicenza, Firenze, Vienna) Follow-up, years 5.3 (0-29.8) Bone marrow fibrosis (reticulin=>1): Yes: 74 pts ( 14%) No: 452 pts (86%)

  4. Overt myelofibrosis-free survival (35 events) ------ BM fibrosis 2.2% pts-yr No BM fibrosis 0.8% pts-yr IRR = 2.7, p=0.01 Barbui T et al, Blood 2012

  5. Degree of bone marrowfibrosisto predicteventsin PVSG-ET N= 361 patients Fibrosis grade (0 to 4) # grade 0-1: 135 # grade 2: 146 # grade 3-4: 80 Bone marrow fibrosis at diagnosis predicts Campbell et al, JCO 2009

  6. WHO-ET vs PMF: Prognostic Value

  7. Incidence of AML Survival, LeukemicTransformation and FibroticProgression in EssentialThrombocythemia are significantlyinfluenced by Accurate MorphologicDiagnosis OS Incidence of MF Barbui et al, Leukemia 2013 Barbuiet al, J ClinOncol. 2011 Aug 10;29(23):3179-84

  8. CLINICAL OVERT MYELOFIBROSIS: How to stratifypatients to selecttherapy

  9. ImprovingSurvival Trends in PMF Median survival: 4.6 versus 6.5 y Cervantes et al. JCO 2012

  10. Causes of Death in PMF 13% 4% 4% 5% 10% 14% 19% 31% Cervantes F et al. Blood 2009;113:2895-901

  11. Current risk stratification in PMF IPSS • Age > 60 years • Hb <10 g/dL • WBC >25 x109/L • Blasts ≥1% • Constit. symptoms Intermediate-1 riskscore 1 Low risk No factor Intermediate-2 riskscore 2 High riskscore ≥ 3

  12. International Prognostic Scoring System to predict survival (IPSS) 135 months 22% 95 months 29% 48 months 28% 27 months 21% Cervantes et al, Blood 2008

  13. DINAMIC IPSS (DIPSS)

  14. HEPATO-SPLENOMEGALY isnotincludedin the risk classification of MF

  15. CLINICAL OVERT MYELOFIBROSIS: Predictors of blastphase

  16. Myelofibrosis:Prognosisassessment in clinicalpractice • PMF risk stratification is based on IPSS and DIPSS, but cytogenetics and transfusional status may be a compendium • Novel prognostic variables deserve further investigations on a large scale

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