Diagnostic Criteria for Polycythemia Vera (PV)

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.