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. 2024 Aug 2;46(8):8407–8423. doi: 10.3390/cimb46080496

Table 1.

International Consensus Classification 2022 for myeloproliferative neoplasms and associated cardiovascular risk factors and complications.

MPNs Major Criteria Minor Criteria Cardiovascular Risk Factors and Complications References
PV
  • hemoglobin: >16.5 g/dL in men; >16.0 g/dL in women; hematocrit: >49% in men and >48% in women

  • JAK2 V617F or JAK2 exon 12 mutation

  • EPO concentrations lower than normal

Hypertension (40–70%) [147]
Diabetes melitius (7–16%) [148]
Obesity (7.5%) and smoking (5–10%) [38]
  • Myocardial infarction (30%) [149]

  • Stroke (25%) [149]

  • Peripheral arterial thrombosis (20%) [149]

[38,147,148,149,150,151]
ET
  • Platelet count ≥ 450 × 109/L

  • JAK2, CALR, or MPL mutation

  • Exclusion of secondary causes of platelet number increase

Thrombotic risk before diagnostic 18% [152]
Each cardiovascular risk increases the thrombotic risk [153]:
  • No CV risk (14%)

  • 1 CV risk (16%)

  • More than 1 CV risk (29%)

[150,151,152,153]
PMF
  • High-grade reticulin fibrosis (2 or 3 grade)

  • JAK2, CALR, or MPL mutation

  • Secondary anemia excluded

  • Leukocytosis ≥ 11 × 109/L

  • Enlarged spleen

  • LDH levels raised above normal interval

  • Leucoerythroblastosis

Thrombotic events [154]:
  • Myocardial infarction (9.5%)

  • Stroke (2.3%)

  • Venous thromboembolism (4.5%)

  • Splanchnic thrombosis (0.6%)

[150,151,154]

JAK2—Janus kinase 2; MPL—myeloproliferative leukemia protein; CALR—calreticulin; EPO—erythropoietin; LDH–lactate dehydrogenase.