Mutational Analysis of Driver and Non-driver Mutations of Philadelphia Chromosome-negative Myeloproliferative Neoplasms; Diagnosis and Recent Advances in Treatment

Table 2.

Simplified 2022 WHO diagnosis criteria for ETand post-ET MF; Adapted from: [6, 104].

ET Post-ET MF

Major Criteria: Required Criteria:

Increased platelet count ≥ 450×109/L Previously diagnosed ET

Proliferation of enlarged, mature megakaryocytes with hyperlobulated nuclei. Grade 2 or 3 bone marrow fibrosis.

Criteria for BCR-ABL1, CML, PV, PMF, MDS or other myeloid neoplasms not met. Additional Criteria:

Presence of JAK2 (< 1% sensitivity assay). CALR or MPL (1-3% sensitivity assay) mutations. Anemia and decreased hemoglobin >2g/Dl from baseline.Leukoerythroblastosis.

Minor Criteria: New or increased palpable splenomegaly >5 cm from baseline

Presence of clonal marker (eg, ASXL1, EZH2, TET2, IDH1/IDH2, SRSF2, SF3B1 mutations) or absence of evidence for reactive thrombocytosis Increased LDH levels above the reference range.

Requirements for diagnosis: Presence of at least 2 of the following: unexplained pyrexia (>37.5℃), weight loss (>10% in 6 months), night sweats.

All 4 major OR first 3 majors + minor criterion Requirement for diagnosis:

  All required criteria + 2 or more additional criteria