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International Consensus Classification (ICC) Diagnostic Criteria for Primary Myelofibrosis (PMF)

Checklist of major and minor criteria required to diagnose primary myelofibrosis.

International Consensus Classification (ICC) Diagnostic Criteria for Primary Myelofibrosis (PMF)
Megakaryocytic proliferation and atypia with reticulin/collagen fibrosis (grade >=2)
Not meeting ICC criteria for another myeloid neoplasm (CML, PV, ET, MDS, etc.)
JAK2, CALR, or MPL mutation present, or another clonal marker present, or no evidence of reactive marrow fibrosis
Anemia not attributable to a comorbid condition
Leukocytosis >=11 x 10^9/L
Palpable splenomegaly
LDH above the upper limit of normal
ResultCriteria not met

Diagnostic criteria for PMF are not fulfilled with the findings entered.

Major criteria met
0 of 3
Minor criteria met
0 of 4

When to use

  • Confirming a diagnosis of prefibrotic/early or overt fibrotic primary myelofibrosis in a patient with suspected myeloproliferative neoplasm.

Formula

Diagnosis requires all 3 major criteria (megakaryocytic atypia with fibrosis, exclusion of other myeloid neoplasms, and a clonal marker or absence of reactive fibrosis) plus at least 1 of 4 minor criteria (anemia, leukocytosis >=11x10^9/L, palpable splenomegaly, elevated LDH), ideally confirmed on two consecutive determinations.

Pearls and pitfalls

  • Leukoerythroblastosis is a supportive but not required feature and is not one of the four minor criteria in the ICC scheme.

References

  1. Arber DA, Orazi A, Hasserjian RP, et al. Blood. 2022;140(11):1200-28.