- Diagnostic criteria of polycythemia vera (PV) from 1975 to 2022.
| Title | Diagnostic criteria |
|---|---|
| PVSG 1975 | Major criteria |
| A1 Raised RCM, male >/= 36 ml/kg, female >/= 32 ml/kg | |
| A2 Normal arterial oxygen saturation >/= 92% | |
| A3 Splenomegaly on palpation | |
| Minor criteria | |
| B1 Platelets < 400 x109/l | |
| B2 WBC >12 x109/l | |
| B3 Elevated leuocyte alkaline phosphatase score | |
| Diagnosis | |
| A1 + A2 + A3 establishes PV | |
| A1 + A2 + two of category B establishes PV | |
| World Health Organization Classification 2001 | Major criteria |
| A1 Increased red cell mass | |
| >25% above mean normal value or | |
| Hb >18.5 g/dL in men, Hb >16.5 g/dL in women | |
| A2 Absence of secondary erythrocytosis | |
| A3 Splenomegaly on palpation | |
| A4 Clonal evidence other than Ph-positive or BCR/ABL | |
| A5 Spontaneous EEC | |
| Minor criteria | |
| B1 Platelets > 400 x109/l | |
| B2 Leukocytes > 12 x109/l | |
| B3 Bone marrow biopsy with typical PV features | |
| Increased cellularity with trilineage myeloproliferation and clustering of small to giant (pleiomorphic) megakaryocytes | |
| B4 Low serum erythropoietin level | |
| Diagnosis | |
| A1+A2+any other from A | |
| A1+A2+two from B | |
| British Society of Hematology Guidelines for the diagnosis, investigation and management of polycythaemia/erythrocytosis 2005 | Major criteria |
| A1: Raised red cell mass (>25% above mean normal predicted value) or Hct>/= 0.60 males;>/= 0.56 females | |
| A2: Absence of cause for secondary erythrocytosis (consider possibility of dual pathology) | |
| A3: Palpable splenomegaly | |
| A4: Clonality marker, i.e. acquired abnormal marrow karyotype | |
| Minor criteria | |
| B1: Thrombocytosis (platelet count >400x109/l) | |
| B2: Neutrophil leucocytosis (neutrophil count >10x109/l in non-smokers; >12.5x109/l in smokers) | |
| B3: Splenomegaly (demonstrated on isotope/ultrasound scanning) | |
| B4: Characteristic BFU-E growth or reduced serum erythropoietin | |
| Diagnosis | |
| A1 + A2 + A3 or A4 establishes PV | |
| A1 + A2 + any 2B criteria establishes PV | |
| Modified diagnostic criteria for polycythemia vera from the British Committee for Standards in Hematology criteria 2007 | JAK2-positive polycythaemia vera |
| A1 High haematocrit (>0.52 in men, >0.48 in women) OR raised red cell mass (>25% above predicted) | |
| A2 Mutation in JAK2 | |
| Diagnosis requires both criteria to be present | |
| JAK2-negative polycythaemia vera | |
| Major criteria | |
| A1 Raised red cell mass (>25% above predicted) OR hematocrit >/= 0.60 in men,>/= 0.56 in women | |
| A2 Absence of mutation inJAK2 | |
| A3 No cause of secondary erythrocytosis | |
| A4 Palpable splenomegaly | |
| A5 Presence of an acquired genetic abnormality (excluding BCR-ABL) in the hematopoietic cells | |
| Minor criteria | |
| B1 Thrombocytosis (platelet count >450x109/l) | |
| B2 Neutrophil leucocytosis (neutrophil count > 10x109/l in non-smokers; >12.5x109/l in smokers) | |
| B3 Radiological evidence of splenomegaly | |
| B4 Endogenous erythroid colonies or low serum erythropoietin | |
| Diagnosis requires A1 + A2 + A3 + either another A or two B criteria | |
| 2008 World Health Organization diagnostic criteria | Major criteria |
| 1 Hemoglobin > 18.5 g/dl (men), > 16.5 g/dl (women) or | |
| Hemoglobin or Hematocrit >99th percentile of reference range for age, sex or altitude of residence | |
| or | |
| Hemoglobin >17 g/dl (men), >15 g/dl (women) if associated with a sustained increase of >/=2 g/dl from baseline that cannot be attributed to correction of iron deficiency | |
| or | |
| Elevated red cell mass >25% above mean normal predicted value | |
| 2 Presence of JAK2V617F or similar mutation | |
| Minor criteria | |
| 1 BM trilineage myeloproliferation | |
| 2 Subnormal serum Epo level | |
| 3 EEC growth | |
| Diagnosis requires meeting either both major criteria and one minor criterion or the first major criterion and 2 minor criteria | |
| The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia | Major criteria |
| 1. Hemoglobin >16.5 g/dL in men | |
| Hemoglobin >16.0 g/dL in women | |
| Or, Hematocrit >49% in men | |
| Hematocrit >48% in women | |
| Or, Increased red cell mass (RCM) | |
| 2. BM biopsy showing hypercellularity for age with trilineage growth (panmyelosis) including prominent erythroid, granulocytic, and megakaryocytic proliferation with pleomorphic, mature megakaryocytes (differences in size) | |
| 3. Presence of JAK2V617F or JAK2 exon 12 mutation | |
| Minor criterion | |
| Subnormal serum erythropoietin level | |
| Diagnosis of PV requires meeting either all 3 major criteria, or the first 2 major criteria and the minor criterion. | |
| Criterion number 2 (BM biopsy) may not be required in cases with sustained absolute erythrocytosis: hemoglobin levels >18.5 g/dL in men (hematocrit, 55.5%) or >16.5 g/dL in women (hematocrit, 49.5%) if major criterion 3 and the minor criterion are present. | |
| The 5th edition of the World Health Organization Classification of Hematolymphoid Tumours 2022 | Major criteria |
| 1. Hemoglobin >16.5 g/dL in men | |
| Hemoglobin >16.0 g/dL in women | |
| Or, | |
| Hematocrit >49% in men | |
| Hematocrit >48% in women | |
| 2. BM biopsy showing hypercellularity for age with trilineage growth (panmyelosis) including prominent erythroid, granulocytic, and megakaryocytic proliferation with pleomorphic, mature megakaryocytes (differences in size) | |
| 3. Presence of JAK2V617F or JAK2 exon 12 Mutation | |
| Minor criterion | |
| Subnormal serum erythropoietin level | |
| Diagnosis of PV requires meeting either all 3 major criteria, or the first 2 major criteria and the minor criterion. | |
| Criterion number 2 (BM biopsy) may not be required in cases with sustained absolute erythrocytosis: hemoglobin levels >18.5 g/dL in men (hematocrit, 55.5%) or >16.5 g/dL in women (hematocrit, 49.5%) if major criterion 3 and the minor criterion are present. |