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Emerging JAK Inhibitors in Myelofibrosis: Determining the Right Agent for the Right Patient.(Madrid) How to use prognosis assessment criteria for MF management.

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Presentation on theme: "Emerging JAK Inhibitors in Myelofibrosis: Determining the Right Agent for the Right Patient.(Madrid) How to use prognosis assessment criteria for MF management."— Presentation transcript:

1 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

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3 FROM PV and ET to MYELOFIBROSIS: The value of bone marrow morphology

4 INITIAL BONE MARROW RETICULIN FIBROSIS IN POLYCYTHEMIA VERA EXERTS AN IMPACT ON CLINICAL OUTCOME (IWG-RT study) Tiziano Barbui 1, 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, 7 Veronika Buxhofer-Ausch, 7 Leonhard Müllauer, 8 Guido Finazzi, 1 Alessandra Carobbio, 1 Andrea Gianatti, 1 Marco Ruggeri, 9 Francesco Rodeghiero, 9 Emanuele DAmore, 9 Alessandro Rambaldi, 1 and Ayalew Tefferi, 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%)

5 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

6 Degree of bone marrow fibrosis to predict events in PVSG-ET Campbell et al, JCO 2009 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

7 WHO-ET vs PMF: Prognostic Value

8 Incidence of MF Incidence of AML OS Barbui et al, Leukemia 2013 Barbui et al, J Clin Oncol Aug 10;29(23): Survival, Leukemic Transformation and Fibrotic Progression in Essential Thrombocythemia are significantly influenced by Accurate Morphologic Diagnosis

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10 CLINICAL OVERT MYELOFIBROSIS: How to stratify patients to select therapy

11 Improving Survival Trends in PMF Median survival: 4.6 versus 6.5 y Cervantes et al. JCO 2012

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

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14 Current risk stratification in PMF IPSS Low risk No factor Age > 60 years Hb <10 g/dL WBC >25 x10 9 /L Blasts 1% Constit. symptoms Intermediate-1 risk score 1 Intermediate-2 risk score 2 High risk score 3

15 135 months 95 months 48 months 27 months International Prognostic Scoring System to predict survival (IPSS) 22% 29% 28% 21% Cervantes et al, Blood 2008

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17 DINAMIC IPSS (DIPSS)

18 HEPATO-SPLENOMEGALY is not included in the risk classification of MF

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21 CLINICAL OVERT MYELOFIBROSIS: Predictors of blast phase

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23 Myelofibrosis: Prognosis assessment in clinical practice 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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