The WHO criteria for the diagnosis of PV
A1 | Elevated RCM (> 25% more than mean normal predicted value, or Hb more than 185 g/L in men, 165 g/L in women*) |
| A2 | No cause of secondary erythrocytosis, including: |
| Absence of familial erythrocytosis | |
| No elevation of Epo due to: | |
| Hypoxia (arterial pO2 ≤ 92%) | |
| High oxygen affinity hemoglobin | |
| Truncated Epo receptor | |
| Inappropriate Epo production by tumor | |
| A3 | Splenomegaly |
| A4 | Clonal genetic abnormality other than Ph− chromosome or BCR/ABL fusion gene in marrow cells |
| A5 | Endogenous erythroid colony formation in vitro |
| B1 | Thrombocytosis more than 400 × 109/L |
| B2 | WBC more than 12 × 109/L |
| B3 | Bone marrow biopsy showing panmyelosis with prominent erythroid and megakaryocytic proliferation |
| B4 | Low serum Epo levels |
A1 | Elevated RCM (> 25% more than mean normal predicted value, or Hb more than 185 g/L in men, 165 g/L in women*) |
| A2 | No cause of secondary erythrocytosis, including: |
| Absence of familial erythrocytosis | |
| No elevation of Epo due to: | |
| Hypoxia (arterial pO2 ≤ 92%) | |
| High oxygen affinity hemoglobin | |
| Truncated Epo receptor | |
| Inappropriate Epo production by tumor | |
| A3 | Splenomegaly |
| A4 | Clonal genetic abnormality other than Ph− chromosome or BCR/ABL fusion gene in marrow cells |
| A5 | Endogenous erythroid colony formation in vitro |
| B1 | Thrombocytosis more than 400 × 109/L |
| B2 | WBC more than 12 × 109/L |
| B3 | Bone marrow biopsy showing panmyelosis with prominent erythroid and megakaryocytic proliferation |
| B4 | Low serum Epo levels |
A positive diagnosis of PV is made when A1 and A2 as well as any other criterion from category A is present or when A1 and A2 as well as any 2 criteria of category B are present.8
RCM indicates red cell mass; WBC, white blood cell count.
Or more than 99th percentile of method-specific reference range for age, sex, and altitude of residence.