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. 2021 Oct 9;13(20):5035. doi: 10.3390/cancers13205035

Table 1.

Risk stratification schemes for myelofibrosis involving clinical and genetic factors.

IPSS DIPSS-Plus MIPSS70 MYSEC GIPSS
Genetic factors
  • unfavorable karyotype

  • (+8, −7/7q−, i(17q), inv(3), −5/5q−, 12p− or 11q23)

  • absence of CALR type 1 mutations

  • presence of 1 or more HMR mutations (ASXL1, EZH2, SRSF2, IDH1/2)

  • absence of CALR mutations

  • absence of CALR type 1/like mutations

  • presence of ASXL1, SRSF2, U2AF1Q157 mutations

  • unfavorable karyotype

Clinical factors
  • age >65

  • hemoglobin <100 g/L

  • WBC > 25 × 109/L

  • circulating blasts ≥1%

  • constitutional symptoms

  • age >65

  • hemoglobin <100 g/L

  • WBC > 25 × 109/L

  • circulating blasts ≥1%

  • constitutional symptoms

  • need for RBC transfusion

  • platelets < 100 × 109/L

  • hemoglobin <100 g/L

  • WBC > 25 × 109/L

  • platelets < 100 × 109/L

  • circulating blasts ≥2%

  • fibrosis grade ≥2

  • constitutional symptoms

  • age at diagnosis

  • hemoglobin <100 g/L

  • platelets < 150 × 109/L

  • circulating blasts ≥3%

  • constitutional symptoms

Risk category (points) low (0)
intermediate-1 (1)
intermediate-2 (2)
high (3)
low (0)
intermediate-1 (1)
intermediate-2 (2–3)
high (≥4)
low (0–1)
intermediate (2–4)
high (≥5)
low (0)
intermediate-1 (1)
intermediate-2 (2)
high (≥3)
low (0)
intermediate-1 (1)
intermediate-2 (2)
high (≥3)
Target patients MF patients
at diagnosis
MF patients at diagnosis and at any time point during clinical course MF patients at evaluation
for allogeneic HSCT
post-ET and post-PV myelofibrosis patients PMF patients

IPSS: international prognostic scoring system, DIPSS: dynamic international prognostic system, MYSEC: myelofibrosis secondary to PV and ET-prognostic model, MIPPS70: mutation-enhanced international prognostic score system (validated up to 70 years of age), GIPSS: genetically inspired prognostic scoring system.