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Prognostic Assessment in Primary Myelofibrosis

Prognostic Assessment in Primary Myelofibrosis. Francisco Cervantes Hematology Department, Hospital Clínic, Barcelona, Spain. Tunis, October 2010. 1024. 10 0. 10 1. 10 2. 10 3. 10 4. 768. Transformed SSC ->. 512. 256. 0. CD34 PE ->. Primary Myelofibrosis.

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Prognostic Assessment in Primary Myelofibrosis

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  1. Prognostic Assessment in Primary Myelofibrosis Francisco Cervantes Hematology Department, Hospital Clínic, Barcelona, Spain Tunis, October 2010

  2. 1024 100 101 102 103 104 768 Transformed SSC -> 512 256 0 CD34 PE -> Primary Myelofibrosis

  3. Presenting Symptoms in Primary Myelofibrosis (n= 170) Anemic symptoms 29.3% Constitutional symptoms 27.4% Symptoms from splenomegaly 18.4% Bleeding 4% Thrombosis 3.7% Pruritus 3.5% Gout 1.2% Bone pain 1.2% Others < 1% Lack of symptoms 29%

  4. Presenting Hematologic Values in Primary Myelofibrosis (n= 170) • Hb (g/dL) • > 12 29% • 10 - 12 26% • < 10 45% • WBC x 109/L • > 10 41% • 4 - 10 44% • < 4 15% • Platelets x 109/L> 400 30% • 100 - 400 50% • < 100 20%

  5. Survival in PMF Cervantes et al.,Blood 2009

  6. Relative Survival in PMF Cervantes et al., Blood 2009

  7. Main Prognostic Factors in PMF • Hb < 10 g/dL • Constitutional symptoms • Older age • Leukocyte counts • Blood blasts • Abnormal karyotype

  8. Hb < 10 g/dL WBC < 4 or > 30 x 109/L Low risk: 0 factors Intermediate risk: 1 factor High risk: 2 factors Dupriez´s Prognostic Score Adverse factors Prognostic groups Median survival 93 months 26 months 13 months Dupriez et al.,Blood 1996

  9. Primary Myelofibrosis:Age Distribution (n= 170) 50 40 Median: 64 years (17-89) Number of patients 30 20 10 0 10 20 30 40 50 60 70 80 90 Años

  10. 0 4 8 12 16 20 24 28 PMF < 55 years: Prognostic Factors and Survival According to Risk Group 1.0 n=121 - Hb < 10 g/dL - Constitutional symptoms - Blood blasts > 1% 0.8 Low risk (score 0-1) 0.6 Probability 0.4 High risk (score 2-3) 0.2 Years Cervantes et al.,Br J Haematol 1998

  11. Survival in PMF Cervantes et al.,Blood 2009

  12. IWG-MRT Study: Prognostic Factors in PMF (n= 1,054) Age > 65 years Constitutional symptoms Hb < 10 g/dL Leukocytes > 25 x 109/L Blood blasts > 1% Cervantes et al.,Blood 2009

  13. PMF: Survival Curves by Risk Group n= 1,001 Cervantes et al., Blood 2009

  14. PMF- Prognostic groups Cervantes et al., Blood 2009

  15. PMF-PS = 0 PMF-PS = 1 PMF-PS = 3 PMF-PS = 2 PMF: Relative Survival by Risk Group Cervantes et al., Blood 2009

  16. DIPSS in the Overall PMF Patients: Weight of Variables and Risk Groups Passamonti et al., Blood 2010

  17. Dynamic International Prognostic Scoring System: Time of Appearance of the Risk Factors Passamonti et al., Blood 2010

  18. Dynamic International Prognostic Scoring System: Survival by risk group(overall series) Passamonti et al., Blood 2010

  19. PMF DIPSS in Patients < 65 years: Weight of Variables and Risk Groups Passamonti et al., Blood 2010

  20. Dynamic International Prognostic Scoring System: Survival by risk group (patients < 65 years) Passamonti et al., Blood 2010

  21. Cytogenetic Abnormalities in PMF • del 20q • del 13q • Trisomy 8 • Trisomy 1q • Trisomy 9 • Monosomy 7 • t (1;7) • del 12p • t (1;6)

  22. IWG-MRT: PMF and Karyotype (n= 409) • Patients with abnormalities:30% • Significant association with survival even after adjustment for prognostic score (p= 0.01) • The variable “abnormal karyotype”increased the discriminating power of the prognostic score, but onlyin theintermediate-risk groups. Cervantes et al., Blood 2009

  23. Karyotype and Prognosis in PMF Favorable: • 13q-, 20q-, +9 • Normal diploid Unfavorable: • Abnormal 5, 7 or 17 • Complex Tam et al., Blood 2009

  24. Karyotype and Prognosis in PMF • Favorable: 13q-, 20q-, +9 • Unfavorable: Complex, +8 • Normal diploid • Others Hussein et al., Blood 2010

  25. Karyotype and Prognosis in Intermediate-1 Risk PMF patients • Favorable + Normal • Unfavorable + Others Hussein et al., Blood 2010

  26. Mutation JAK2 V617F in the MPNs V617F Carboxy-terminal Amino-terminal FERM SH2 JH2 JH1 Frequency of the JAK2 mutation PV ET PMF 90-95% 50-60% 60%

  27. Prognostic Value of the JAK2 Mutation in PMF Author No. of Prognostic (year) patients influence Tefferi (2005) 157 No Campbell (2006) 152 Yes Barosi (2007) 174 Yes * Cervantes (2009) 345 No Guglielmelli (2009) 186 Yes ** * Higher leukemic transformation rate; ** shorter survival for lower burden

  28. Treatment options for Myelofibrosis Wait & see Conventional treatment Investigational drugs Allo-HSCT

  29. Low risk Intermediate-1 risk Intermediate-2 risk High risk Wait & see Wait & see or Conventional treatment * Allo-HSCT or Conventional / Investigational drugs * Allo-HSCT or Investigational drugs * Check cytogenetics or transfusion dependence * Depending on age Proposed Algorithm for PMF Treatment

  30. Conclusions • Median survival of PMF patients is 5.5 years but there is a wide heterogeneity. • Main prognostic factors are age > 65 years, constitutional symptoms, Hb < 10 g/dL, leukocytosis > 25 x109/L, and blood blasts > 1%; certain karyotypic abnormalities also contribute to prognosis. • Based on these prognostic factors it is possible to identify four risk groups both at diagnosis and during the evolution of the disease. • These risk groups are of help in treatment-decision making.

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