Table 2.

Genomic prognostic indices in MF

Prognostic Index/ ReferencePrognostic factorsOutcomesNotes
MIPSS7015  Hgb <10 g/dL
WBC >25 × 103/µL
Platelets <100 × 103/µL
Circulating blasts ≥2%
Constitutional symptoms
Bone marrow fibrosis grade ≥2
No. of HMR mutations*
Absence of type 1/like CALR mutation 
Median OS, y
 Low risk: 27.7
 Int risk: 7.1
 High risk: 2.3 
Designed for PMF patients ≤70 y old 
MIPSS70+15  Hgb <10 g/dL
WBC >25 × 103/µL
Platelets <100 × 103/µL
Circulating blasts ≥2%
Constitutional symptoms
No. of HMR mutations*
Absence of type 1/like CALR mutation
Unfavorable karyotype† 
Median OS, y
 Low risk: 20
 Int risk: 6.3
 High risk: 3.9
 Very high risk: 1.7 
Designed for PMF patients ≤70 y old 
MIPSS70+ v2.016  Severe or moderate anemia‡
Circulating blasts ≥2%
Constitutional symptoms
No. of HMR mutations§
Absence of type 1/like CALR mutation
VHR or unfavorable karyotype|| 
Median OS, y
 Very low risk: not reached
 Low risk: 16.4
 Int risk: 7.7
 High risk: 4.1
 Very high risk: 1.8 
Designed for PMF patients ≤70 y old
Assigns more points for severe anemia compared with moderate anemia
Assigns more points for VHR karyotype compared with unfavorable karyotype 
GIPSS17  VHR or unfavorable karyotype||
Absence of type 1/like CALR mutation
ASXL1 mutation
SRSF2 mutation
U2AF1 Q157 mutation 
Median OS, y
 Low risk: 26.4
 Int-1 risk: 8
 Int-2 risk: 4.2
 High risk: 2 
Designed for PMF
Assigned greater points for unfavorable karyotype compared with very high-risk karyotype 
MYSEC-PM18  Age at diagnosis of SMF
Hgb <11 g/dL
Platelet <150 × 103/µL
Circulating blasts ≥3%
CALR unmutated
Constitutional symptoms 
Median OS, y
 Low risk: not reached
 Int-1 risk: 9.3
 Int-2 risk: 4.4
 High risk: 2 
Designed for SMF 
Prognostic Index/ ReferencePrognostic factorsOutcomesNotes
MIPSS7015  Hgb <10 g/dL
WBC >25 × 103/µL
Platelets <100 × 103/µL
Circulating blasts ≥2%
Constitutional symptoms
Bone marrow fibrosis grade ≥2
No. of HMR mutations*
Absence of type 1/like CALR mutation 
Median OS, y
 Low risk: 27.7
 Int risk: 7.1
 High risk: 2.3 
Designed for PMF patients ≤70 y old 
MIPSS70+15  Hgb <10 g/dL
WBC >25 × 103/µL
Platelets <100 × 103/µL
Circulating blasts ≥2%
Constitutional symptoms
No. of HMR mutations*
Absence of type 1/like CALR mutation
Unfavorable karyotype† 
Median OS, y
 Low risk: 20
 Int risk: 6.3
 High risk: 3.9
 Very high risk: 1.7 
Designed for PMF patients ≤70 y old 
MIPSS70+ v2.016  Severe or moderate anemia‡
Circulating blasts ≥2%
Constitutional symptoms
No. of HMR mutations§
Absence of type 1/like CALR mutation
VHR or unfavorable karyotype|| 
Median OS, y
 Very low risk: not reached
 Low risk: 16.4
 Int risk: 7.7
 High risk: 4.1
 Very high risk: 1.8 
Designed for PMF patients ≤70 y old
Assigns more points for severe anemia compared with moderate anemia
Assigns more points for VHR karyotype compared with unfavorable karyotype 
GIPSS17  VHR or unfavorable karyotype||
Absence of type 1/like CALR mutation
ASXL1 mutation
SRSF2 mutation
U2AF1 Q157 mutation 
Median OS, y
 Low risk: 26.4
 Int-1 risk: 8
 Int-2 risk: 4.2
 High risk: 2 
Designed for PMF
Assigned greater points for unfavorable karyotype compared with very high-risk karyotype 
MYSEC-PM18  Age at diagnosis of SMF
Hgb <11 g/dL
Platelet <150 × 103/µL
Circulating blasts ≥3%
CALR unmutated
Constitutional symptoms 
Median OS, y
 Low risk: not reached
 Int-1 risk: 9.3
 Int-2 risk: 4.4
 High risk: 2 
Designed for SMF 

GIPSS, Genetically Inspired Prognostic Scoring System; HMR, high molecular risk; MIPSS, Mutation-Enhanced International Prognostic Score System; MYSEC-PM, Myelofibrosis Secondary to PV and ET-Prognostic Model; SMF, secondary myelofibrosis; VHR, very high risk. See Table 1 for expansion of other abbreviations.

*

HMR mutations include ASXL1, SRSF2, EZH2, IDH1, and IDH2.

Unfavorable karyotype defined as any abnormal karyotype other than normal karyotype or sole abnormalities of 20q2, 13q2, +9, chromosome 1 translocation/duplication, 2Y, or sex chromosome abnormality other than 2Y.

Moderate anemia is defined by hemoglobin levels of 8 to 9.9 g/dL in women and 9 to 10.9 g/dL in men; severe anemia is defined by hemoglobin levels of 8 g/dL in women and 9 g/dL in men.

§

HMR mutations in MIPSS70+ v2.0 include ASXL1, SRSF2, EZH2, IDH1, IDH2, and U2AF1 Q157.

||

VHR karyotype defined as single/multiple abnormalities of −7, i(17q), inv(3)/ 3q21, 12p−/12p11.2, 11q−/11q23, or other autosomal trisomies not including +8/+9 (eg, +21, +19).

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