MDCalc

Mutation-Enhanced International Prognostic Score System (MIPSS70/MIPSS70+)

Stratifies risk for patients with overt primary myelofibrosis (PMF).

Be aware, this tool provides results for either MIPSS70 or MIPSS70+, version 2.0, depending on whether karyotype information is available.

  • If karyotype is not available, this tool will default to MIPSS70. 
  • If karyotype information is inputted, the tool will default to MIPSS70+. which will also affect hemoglobin (i.e., more specific anemia cutoff values) and HMR mutation inputs (i.e., the inclusion of U2AR1 Q157).

Karyotype information

Hemoglobin <10 g/dL

Platelet count <100,000 /µL

Leukocyte count >25,000  /µL

Circulating blasts ≥2%

Fibrosis grade ≥2

Constitutional symptoms

Absence of CALR type 1-like mutation

HMR category

Presence of a mutation in any of the following genes: ASXL1, EZH2, SRSF2, or IDH1/2 (MIPSS70+ v2.0 also includes U2AF1 Q157)

Two or more HMR mutations

Presence of two or more mutated genes among ASXL1, EZH2, SRSF2, and IDH1/2 i (MIPSS70+ v2.0 also includes U2AF1 Q157); two or more mutations in the same gene are counted as one

Result:

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Advice
  • Results may be used alone or to refine the prognosis established from other prognostic models (e.g., GIPSS).
  • Use results in conjunction with clinical judgment and patient preference to guide management care plans and inform goals of care discussions.
    • Higher-risk patients (high or very high risk designations in MIPSS70+): Consider referral to a transplant center for HSCT evaluation.
    • Intermediate-risk patients: Discuss options, including aggressive, experimental, or conservative management, and monitor closely for progression.
    • Low-risk patients (low, or very low risk designations in MIPSS70+): Consider conservative, symptom-directed treatment with regular follow-up.
  • Results are prognostic, not predictive of treatment response; clinical judgment and specialist consultation remain essential.