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

Table 1.

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

PV Post-PV MF

Major Criteria: Required Criteria:

Elevated hemoglobin > 18.5 g/dL in men, 16.5 g/dL in women, or increased hematocrit (> 49% in men, > 48% in women)+ Previously diagnosed PV

Presence of JAK2 V617F or Exon 12 mutation (< 1% sensitivity). Grade 2 or 3 bone marrow fibrosis.

Hypercellular bone marrow with trilineage proliferation, including prominent erythroid, granulocytic and increased pleomorphic, mature megakaryocytes without atypia. Additional Criteria:

Minor Criteria: Anemia

Subnormal erythropoietin level Leukoerythroblastosis.

Requirements for diagnosis: New or increased palpable splenomegaly >5 cm from baseline

All 3 major OR first 2 major + minor criterion* Presence of at least 2 of the following: unexplained pyrexia (>37.5℃), weight loss (>10% in 6 months), night sweats.

  Requirement for diagnosis:

  All required criteria + 2 or more additional criteria

+As a refinement to the 2016 WHO diagnostic criteria, increased red cell mass has been removed as a major criterion

*Major criterion 3 may not be required in patients with sustained absolute erythrocytosis: hemoglobin >18.5 g/dL and hematocrit >55.5% in men or hemoglobin >16.5 g/dL and hematocrit, 49.5% in women; if major criterion 2 and the minor criterion are present.