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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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slide1

Prognostic Assessment in Primary Myelofibrosis

Francisco Cervantes

Hematology Department, Hospital Clínic, Barcelona, Spain

Tunis, October 2010

slide2

1024

100

101

102

103

104

768

Transformed SSC ->

512

256

0

CD34 PE ->

Primary Myelofibrosis

slide3

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%

slide4

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%
slide5

Survival in PMF

Cervantes et al.,Blood 2009

relative survival in pmf
Relative Survival in PMF

Cervantes et al., Blood 2009

main prognostic factors in pmf
Main Prognostic Factors in PMF
  • Hb < 10 g/dL
  • Constitutional symptoms
  • Older age
  • Leukocyte counts
  • Blood blasts
  • Abnormal karyotype
dupriez s prognostic score
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

slide9

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

slide10

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

slide11

Survival in PMF

Cervantes et al.,Blood 2009

iwg mrt study prognostic factors in pmf n 1 054

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

slide13

PMF: Survival Curves by Risk Group

n= 1,001

Cervantes et al., Blood 2009

slide14

PMF- Prognostic groups

Cervantes et al., Blood 2009

slide15

PMF-PS = 0

PMF-PS = 1

PMF-PS = 3

PMF-PS = 2

PMF: Relative Survival by Risk Group

Cervantes et al., Blood 2009

slide16

DIPSS in the Overall PMF Patients:

Weight of Variables and Risk Groups

Passamonti et al., Blood 2010

slide17

Dynamic International Prognostic Scoring System:

Time of Appearance of the Risk Factors

Passamonti et al., Blood 2010

slide18

Dynamic International Prognostic Scoring System:

Survival by risk group(overall series)

Passamonti et al., Blood 2010

slide19

PMF DIPSS in Patients < 65 years:

Weight of Variables and Risk Groups

Passamonti et al., Blood 2010

slide20

Dynamic International Prognostic Scoring System:

Survival by risk group (patients < 65 years)

Passamonti et al., Blood 2010

cytogenetic abnormalities in pmf
Cytogenetic Abnormalities in PMF
  • del 20q
  • del 13q
  • Trisomy 8
  • Trisomy 1q
  • Trisomy 9
  • Monosomy 7
  • t (1;7)
  • del 12p
  • t (1;6)
iwg mrt pmf and karyotype n 409
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

slide23

Karyotype and Prognosis in PMF

Favorable:

  • 13q-, 20q-, +9
  • Normal diploid

Unfavorable:

  • Abnormal 5, 7 or 17
  • Complex

Tam et al., Blood 2009

slide24

Karyotype and Prognosis in PMF

  • Favorable:

13q-, 20q-, +9

  • Unfavorable:

Complex, +8

  • Normal diploid
  • Others

Hussein et al., Blood 2010

slide25

Karyotype and Prognosis in Intermediate-1 Risk PMF patients

  • Favorable + Normal
  • Unfavorable + Others

Hussein et al., Blood 2010

slide26

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%

prognostic value of the jak 2 mutation in pmf
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

slide28

Treatment options for Myelofibrosis

Wait & see

Conventional

treatment

Investigational

drugs

Allo-HSCT

slide29

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

conclusions
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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