MIPSS70+ v2.0: Mutation and Karyotype-enhanced IPSS for Primary Myelofibrosis. Essential for transplant timing in candidates ≤ 70 years.
Clinical Features
Genetic & Molecular
Cytogenetic Risk Class
MIPSS70+ v2.0 Score
0
Risk Category
Low Risk
Estimated Median Survival: > 20 yr
Standard surveillance or targeted therapy (e.g. Ruxolitinib) transition based on symptoms.
Guidelines & Evidence
Clinical Details
Section 1
When to Use
When to Use
Initial risk stratification in patients with Primary Myelofibrosis (PMF) who are candidates for stem cell transplant.
Integrating clinical features with high-risk mutation status (e.g., ASXL1, SRSF2).
Predicting overall survival and leukemia-free survival in the molecular era.
Patient Population
Adult patients with Primary Myelofibrosis (PMF) aged ≤ 70 years. While designed for younger transplant-eligible cohorts, it is the most robust current model.
When Not to Rely on This Score Alone
Secondary MF (e.g., Post-PV/ET MF) — requires different models like MYSEC-PM.
Patients > 70 years — although applicable, transplant decisions differ in older cohorts.
Incomplete molecular data — MIPSS70+ v2.0 requires high-risk mutation profiling (ASXL1, EZH2, SRSF2, U2AF1 Q157) and cytogenetics.
Section 2
Formula & Logic
Clinical Variables
Parameter
Threshold
Points
Anemia
Hb < 10 g/dL
2
Leukocytosis
WBC > 25 × 10⁹/L
1
Blasts
Peripheral blasts ≥ 1%
1
Constitutional
Present
1
Fibrosis
Grade ≥ 2
1
Genetic Variables
Parameter
Condition
Points
Mutation
U2AF1 Q157 mutation
1
HMR Mutation
ASXL1, EZH2, or SRSF2 present
2
High-risk Cyto
Unfavorable / Very High Risk
3-4
Triple Negative
No JAK2/CALR/MPL
2
Risk Groups
Score
Risk Group
Median Survival
0 - 1
Low
Not reached (> 20 yr)
2 - 4
Intermediate
10.3 yr
5 - 8
High
5.0 yr
≥ 9
Very High
1.8 yr
Section 3
Pearls/Pitfalls
Beyond DIPSS-Plus
The MIPSS70+ v2.0 captures the "molecular clock" of myelofibrosis. Patients with low clinical risk but high-risk molecular findings (like SRSF2 or EZH2) may be upstaged and prioritized for early allogeneic transplant.
Section 4
Evidence Appraisal
Primary Score
MIPSS70+ Version 2.0: Mutation and Karyotype-Enhanced International Prognostic Scoring System for Primary Myelofibrosis.
Tefferi A et al. • Journal of Clinical Oncology. 2018;36(17):1769-1770.
Developed by the Mayo Clinic and international collaborators to replace clinical-only models (IPSS/DIPSS) with a tool that reflects the genomic heterogeneity of myelofibrosis.