Prognostic Assessment in Primary Myelofibrosis
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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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Prognostic Assessment in Primary Myelofibrosis

Francisco Cervantes

Hematology Department, Hospital Clínic, Barcelona, Spain

Tunis, October 2010


1024

100

101

102

103

104

768

Transformed SSC ->

512

256

0

CD34 PE ->

Primary Myelofibrosis


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%


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%


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


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


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


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


PMF: Survival Curves by Risk Group

n= 1,001

Cervantes et al., Blood 2009


PMF- Prognostic groups

Cervantes et al., Blood 2009


PMF-PS = 0

PMF-PS = 1

PMF-PS = 3

PMF-PS = 2

PMF: Relative Survival by Risk Group

Cervantes et al., Blood 2009


DIPSS in the Overall PMF Patients:

Weight of Variables and Risk Groups

Passamonti et al., Blood 2010


Dynamic International Prognostic Scoring System:

Time of Appearance of the Risk Factors

Passamonti et al., Blood 2010


Dynamic International Prognostic Scoring System:

Survival by risk group(overall series)

Passamonti et al., Blood 2010


PMF DIPSS in Patients < 65 years:

Weight of Variables and Risk Groups

Passamonti et al., Blood 2010


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


Karyotype and Prognosis in PMF

Favorable:

  • 13q-, 20q-, +9

  • Normal diploid

    Unfavorable:

  • Abnormal 5, 7 or 17

  • Complex

Tam et al., Blood 2009


Karyotype and Prognosis in PMF

  • Favorable:

    13q-, 20q-, +9

  • Unfavorable:

    Complex, +8

  • Normal diploid

  • Others

Hussein et al., Blood 2010


Karyotype and Prognosis in Intermediate-1 Risk PMF patients

  • Favorable + Normal

  • Unfavorable + Others

Hussein et al., Blood 2010


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


Treatment options for Myelofibrosis

Wait & see

Conventional

treatment

Investigational

drugs

Allo-HSCT


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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