2001 WHO criteria for polycythemia vera
| 2001 criteria for PV . |
|---|
| A-criteria |
| 1. Elevated red cell mass > 25% above mean normal predicted value, or hemoglobin > 18.5 g/dL in men, 16.5 g/dL in women, or > 99th percentile of method-specific reference range for age, sex, altitude of residence |
| 2. No cause of secondary erythrocytosis, including: |
| a. Absence of familial erythrocytosis |
| b. No elevation of erythropoietin caused by: |
| i. Hypoxia (arterial pO2 ≤ 92%) |
| ii. High oxygen affinity hemoglobin |
| iii. Truncated erythropoietin receptor |
| iv. Inappropriate erythropoietin production by tumor |
| 3. Splenomegaly |
| 4. Clonal genetic abnormality other than Philadelphia chromosome or BCR-ABL fusion gene in marrow cells |
| 5. Endogenous erythroid colony formation |
| B-criteria |
| 1. Thrombocytosis > 400 × 109/L |
| 2. Leukocytosis > 12 × 109/L |
| 3. Bone marrow biopsy showing panmyelosis with prominent erythroid and megakaryocytic proliferation |
| 4. Low serum erythropoietin levels |
| 2001 criteria for PV . |
|---|
| A-criteria |
| 1. Elevated red cell mass > 25% above mean normal predicted value, or hemoglobin > 18.5 g/dL in men, 16.5 g/dL in women, or > 99th percentile of method-specific reference range for age, sex, altitude of residence |
| 2. No cause of secondary erythrocytosis, including: |
| a. Absence of familial erythrocytosis |
| b. No elevation of erythropoietin caused by: |
| i. Hypoxia (arterial pO2 ≤ 92%) |
| ii. High oxygen affinity hemoglobin |
| iii. Truncated erythropoietin receptor |
| iv. Inappropriate erythropoietin production by tumor |
| 3. Splenomegaly |
| 4. Clonal genetic abnormality other than Philadelphia chromosome or BCR-ABL fusion gene in marrow cells |
| 5. Endogenous erythroid colony formation |
| B-criteria |
| 1. Thrombocytosis > 400 × 109/L |
| 2. Leukocytosis > 12 × 109/L |
| 3. Bone marrow biopsy showing panmyelosis with prominent erythroid and megakaryocytic proliferation |
| 4. Low serum erythropoietin levels |
Diagnosis requires the presence of the first 2 A-criteria together with either any 1 other A-criterion or 2 B-criteria.