Patient selection: primary myelofibrosis, age appropriate for transplantation (age <= 70 in Italy)
Parameters:
(1) constitutional symptoms (constitutional symptoms (unexplained fever, excessive sweating for > 1 month, and/or weight loss > 10%)
(2) anemia (hemoglobin < 100 g/L)
(3) circulating blasts >= 2%
(4) CALR type 1-like mutation
(5) HMR category
(6) number of HMR (high molecular risk) category genes (ASXL1, EZH2, SRSF2, IDH1/2) with mutations
(7) karyotype
|
Parameter
|
Finding
|
Points
|
|
constitutional symptoms
|
none
|
0
|
|
|
present
|
1
|
|
anemia
|
absent
|
0
|
|
|
present
|
1
|
|
circulating blasts
|
< 2%
|
0
|
|
|
>= 2%
|
1
|
|
CALR type-1 like mutation
|
present
|
0
|
|
|
absent
|
2
|
|
HMR category genes with mutations
|
none
|
0
|
|
|
any
|
1
|
|
number of HMR genes showing mutations
|
0 or 1
|
0
|
|
|
2 or more
|
2
|
|
karyotyrpe
|
favorable
|
0
|
|
|
unfavorable
|
3
|
where:
• A gene with multiple mutations was counted only once.
• Favorable karyotype: normal; sole abnormality of 20q-, 13q-, +9, -Y; chromosome 1 translocation or duplication. Any other karyotype was unfavorable.
total score =
= SUM(points for all of the parameters)
Interpretation:
• minimum score: 0
• maximum score: 11 (the paper says 12)
• The higher the score the greater the risk.
|
Score
|
Risk Group
|
5-year Overall Survival
|
|
0 to 2
|
low
|
100%
|
|
3
|
intermediate
|
90%
|
|
4 to 6
|
high
|
76%
|
|
>= 7
|
very high
|
47%
|
