Criteria for the diagnosis of PV
| Current (2008) WHO criteria15 . | Proposal for revised criteria (2014)32 (pending WHO endorsement) . |
|---|---|
| Major criteria | |
| 1. Hb >18.5 g/dL in men, >16.5 g/dL in women, or evidence of increased red cell volume, or* | 1. Hb >16.5 g/dL in men, >16.0 g/dL in women, or Hct >49% in men, >48% in women |
| 2. Presence of JAK2V617F mutation or other functionally similar mutations (e.g., JAK2 exon 12 mutation) | 2. BM biopsy showing trilineage myeloproliferation and pleomorphic megakaryocytes |
| 3. Presence of JAK2 mutation | |
| Minor criteria | |
| 1. BM biopsy showing trilineage myeloproliferation | 1. sEPO level below the normal reference range |
| 2. sEPO level below the normal reference range | |
| 3. Endogenous erythroid colony growth | |
| PV diagnosis requires meeting either both major criteria and 1 minor criterion or the first major criterion and 2 minor criteria | PV diagnosis requires meeting all 3 major criteria or the first 2 major criteria and the minor criterion |
| Current (2008) WHO criteria15 . | Proposal for revised criteria (2014)32 (pending WHO endorsement) . |
|---|---|
| Major criteria | |
| 1. Hb >18.5 g/dL in men, >16.5 g/dL in women, or evidence of increased red cell volume, or* | 1. Hb >16.5 g/dL in men, >16.0 g/dL in women, or Hct >49% in men, >48% in women |
| 2. Presence of JAK2V617F mutation or other functionally similar mutations (e.g., JAK2 exon 12 mutation) | 2. BM biopsy showing trilineage myeloproliferation and pleomorphic megakaryocytes |
| 3. Presence of JAK2 mutation | |
| Minor criteria | |
| 1. BM biopsy showing trilineage myeloproliferation | 1. sEPO level below the normal reference range |
| 2. sEPO level below the normal reference range | |
| 3. Endogenous erythroid colony growth | |
| PV diagnosis requires meeting either both major criteria and 1 minor criterion or the first major criterion and 2 minor criteria | PV diagnosis requires meeting all 3 major criteria or the first 2 major criteria and the minor criterion |
Or Hb or Hct >99th percentile of reference range for age, gender, and altitude of residence or Hb >17 g/dL in men, >5 g/dL in women, if associated with a documented and sustained increase of ≥2 g/dL from a person’s baseline value that cannot be attributed to correction of iron deficiency, or RCM >25% above mean normal predicted level.