What are the diagnostic criteria for polycythaemia vera (PV)?

Diagnostic criteria for PV

Two diagnostic criteria are followed for Polycythaemia Vera (PV).
A. The WHO criteria 
B. The BSH (British Society for Haematology) criteria 

A. The WHO diagnostic criteria for polycythemia vera (PV)

Diagnostic criteria for PV as per the 2016 revised World Health Organization (WHO) guidelines are in the following picture 


The WHO diagnostic  criteria for Polycythaemia Vera (PV)

[The WHO diagnostic criteria for Polycythaemia Vera (PV), adopted from ashpublications]


B. The BSH diagnostic criteria for Polycythaemia Vera (PV)

According to the BSH guidelines, we need to consider two scenarios 

First scenario 

JAK2‐positive polycythaemia vera 

(Requires both criteria) [A1+A2= PV]

A1: High haematocrit (>0·52 in men, >0·48 in women) OR raised red cell mass (>25% above predicted)
A2: Mutation in JAK2

Second scenario 

JAK2‐negative polycythaemia vera

(Requires A1‐A4 plus another A or two B criteria) [A1+A2+A3+A4+(A5/A6 or Two B criteria= PV)]

A1: Raised red cell mass (>25% above predicted), OR haematocrit ≥0·60 in men, ≥0·56 in women
A2: Absence of mutation in JAK2
A3: No cause of secondary erythrocytosis
A4: Bone marrow histology consistent with polycythaemia vera
A5: Palpable splenomegaly
A6: Presence of an acquired genetic abnormality (excluding BCR‐ABL1 ) in the haematopoietic cells

B1: Thrombocytosis (platelet count >450 × 10 9 /l)
B2: Neutrophil leucocytosis (neutrophil count >10 × 10 9 /l in non‐smokers, ≥12·5 × 10 9 /l in smokers)
B3: Radiological evidence of splenomegaly
B4: Low serum erythropoietin

[Adopted from onlinelibrary

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