Tefferi et al reported the GIPSS (genetically inspired prognostic scoring system) for patients with primary myelofibrosis. This can help to identify a patient who may benefit from more aggressive management. The authors are from the Mayo Clinic and the University of Florence.
Parameters:
(1) karyotype
(2) Type 1/like CALR mutation (driver mutation)
(3) ASXL1 mutation (high risk)
(4) SRSF2 mutation (high risk)
(5) U2AF1Q157 mutation (high risk)
Karyotype
Findings
very high risk (VHR)
single or multiple abnormalities of -7; i(17q); inv(3)/3q21; 12p-/12p11.2; 11q-/11q23; +21; other autosomal trisomies (excluding +8 and +9)
favorable
normal karyotype; sole abnormality with 13q- or +9 or 20q-; chromosome 1 translocation or duplication; sex chromosome abnormality including Y-
unfavorable
all other abnormalities including sole +8
Parameter
Finding
Points
karyotype
very high risk (VHR)
2
unfavorable
1
other
0
Type 1/like CALR mutation
present
0
absent
1
ASXL1 mutation
absent
0
present
1
SRSF2 mutation
absent
0
present
1
U2AF1Q157 mutation
absent
0
present
1
total score =
= SUM(points for all 5 parameters)
Interpretation:
• minimum score: 0
• maximum score: 6
Score
Risk Group
Median Survival
0
low
26.4 years
1
intermediate-1
8 years
2
intermediate-2
4.2 years
3-6
high
2 years
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