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) thrombocytopenia (< 100 *10^9/L
(4) leukocytosis (> 25 * 10^9/L)
(5) circulating blasts >= 2%
(6) bone marrow fibrosis grade 2 or 3
(7) CALR type 1-like mutation
(8) number of HMR (high molecular risk) category genes (ASXL1, EZH2, SRSF2, IDH1/2) with mutations
|
Parameter
|
Finding
|
Points
|
|
constitutional symptoms
|
none
|
0
|
|
|
present
|
1
|
|
anemia
|
absent
|
0
|
|
|
present
|
1
|
|
thrombocytopenia
|
absent
|
0
|
|
|
present
|
2
|
|
leukocytosis
|
absent
|
0
|
|
|
present
|
2
|
|
circulating blasts
|
< 2%
|
0
|
|
|
>= 2%
|
1
|
|
bone marrow fibrosis
|
none or grade 1
|
0
|
|
|
grade 2 or 3
|
1
|
|
CALR type-1 like mutation
|
present
|
0
|
|
|
absent
|
1
|
|
HMR category genes with mutations
|
none
|
0
|
|
|
any
|
1
|
|
number of HMR genes showing mutations
|
0 or 1
|
0
|
|
|
2 or more
|
2
|
where:
• A gene with multiple mutations was counted only once.
total score =
= SUM(points for all of the parameters)
Interpretation:
• minimum score: 0
• maximum score: 12
• The higher the score the greater the risk.
|
Score
|
Risk Group
|
5-year Overall Survival
|
|
0 or 1
|
low
|
96%
|
|
2, 3 or 4
|
intermediate
|
67%
|
|
>= 5
|
high
|
34%
|
