Criteria PVSG (Polycythemia Vera Study Group)
A1 Raised red cell mass (RCM), male > 36 ml/kg, female > 32 ml/kg
A2 Normal arterial oxygen saturation > 92%
A3 Splenomegaly
B1 Platelet count > 400 x 109/l
B2 White blood cell count (WBC) > 12 x 109/l
B3 Leucocyte alkaline phosphatase > 100
B4 Serum B12 > 900 pg/ml or unbound B12 binding capacity > 220 pg/ml
Diagnosis
A1 + A2 + A3 establishes PV
A1 + A2 + two of category B establishes PV
Criteria BCSH (British Committee for Standards in Haematology)
A1 Raised RCM or hematocrit (HCT) > 0.60 (males), > 0.56 (females)
A2 No secondary erythrocytosis
A3 Palpable spleen
A4 Abnormal karyotype
B1 Platelet count > 400 x 109/l
B2 Neutrophil count > 10 x 109/l
B3 Splenomegaly on ultrasound scanning
B4 Low serum EPO or characteristic burst forming units – erythroid (BFU-E) growth
Diagnosis
A1 + A2 + A3 or A4 establishes PV
A1 + A2 + two of B establishes PV
Criteria WHO (World Health Organization)
A1 Raised RCM or haemoglobin (Hb) > 18.5 (males), > 16.5 (females)
A2 No secondary erythrocytosis
A3 Splenomegaly
A4 Abnormal karyotype (other than Ph chromosome or BCR/ABL fusion gene in marrow cells)
A5 Endogenous erythroid colony (EEC) formation
B1 Platelet count > 400 x 109/l
B2 WBC > 12 x 109/l
B3 Bone marrow biopsy (BMB) showing panmyelosis with erythroid and megakaryocytic proliferation
B4 Low serum erythropoietin (EPO)
Diagnosis
A1 + A2 and any other category A establishes PV
A1 + A2 + two of category B establishes PV
References:
- Turkington RC, Arnold EC, Percy MJ, Ranaghan LA, Cuthbert RJ, McMullin MF. Comparison of diagnostic criteria for polycythaemia vera. Hematology. 2007 Apr;12(2):123-30. [Medline]
- Tefferi A, Thiele J, Orazi A, Kvasnicka HM, Barbui T, Hanson CA, Barosi G, Verstovsek S, Birgegard G, Mesa R, Reilly JT, Gisslinger H, Vannucchi AM, Cervantes F, Finazzi G, Hoffman R, Gilliland DG, Bloomfield CD, Vardiman JW. Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel. Blood. 2007 Aug 15;110(4):1092-7.[Medline]
- Pearson TC, Messinezy M. The diagnostic criteria of polycythaemia rubra vera. Leuk Lymphoma. 1996 Sep;22 Suppl 1:87-93.[Medline]
- Berlin NI. Diagnosis and classification of the polycythemias. Semin Hematol 1975;12(4):339–351.
Created: Sep 10, 2007