Table 3.

Molecularly integrated risk scores in patients with ET and PV

Essential thrombocythemiaPolycythemia vera
CharacteristicRevised IPSETMIPSS-ETaMIPSS-PVa
Age, y >60 >60 (4) >67 (2) 
History of thrombosis Yes — Yes (1) 
JAK2 V617F Yes — — 
White blood cell count — ≥11 × 109/L [1] ≥15 × 109/L (1) 
Adverse mutation — SRSF2, SF3B1, U2AF1, TP53 [2] SRSF2 [3] 
Male sex — Yes [1] — 
 Thrombosis risk group (rate % patients/y) Risk group [score]: median survival (y) 
Categories Very low: no thrombosis history, age ≤60 y, JAK2WT
Low: no thrombosis history, age ≤60 y, JAK2 V617F
Intermediate: no thrombosis history, age >60 y, JAK2WT
High: thrombosis history or age >60 y with JAK2 V617F 
Low [0-1]: 34.4
Intermediate [2-5]: 14.1
High [≥6]: 7.9 
Low [0-1]: 24.0
Intermediate [2-3]: 13.1
High [≥4]: 3.2 
Essential thrombocythemiaPolycythemia vera
CharacteristicRevised IPSETMIPSS-ETaMIPSS-PVa
Age, y >60 >60 (4) >67 (2) 
History of thrombosis Yes — Yes (1) 
JAK2 V617F Yes — — 
White blood cell count — ≥11 × 109/L [1] ≥15 × 109/L (1) 
Adverse mutation — SRSF2, SF3B1, U2AF1, TP53 [2] SRSF2 [3] 
Male sex — Yes [1] — 
 Thrombosis risk group (rate % patients/y) Risk group [score]: median survival (y) 
Categories Very low: no thrombosis history, age ≤60 y, JAK2WT
Low: no thrombosis history, age ≤60 y, JAK2 V617F
Intermediate: no thrombosis history, age >60 y, JAK2WT
High: thrombosis history or age >60 y with JAK2 V617F 
Low [0-1]: 34.4
Intermediate [2-5]: 14.1
High [≥6]: 7.9 
Low [0-1]: 24.0
Intermediate [2-3]: 13.1
High [≥4]: 3.2 
a

Values within brackets indicate the variable's rank.

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