diagnostic criteria for polycythaemia vera

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Polycythaemia vera is a myeloproliferative neoplasm known to be associated with dysregulated signalling of the Janus associated kinases JAK1 and JAK2.

Recommended diagnostic criteria for PV


JAK2-positive polycythaemia vera (requires both criteria)

  • A1 High haematocrit (> 0.52 in men, > 0.48 in women) OR raised red cell mass (>25% above predicted)
  • A2 Mutation in JAK2

JAK2-negative polycythaemia vera (requires A1- A4 plus another A or two B criteria) *

  • 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 x 10^9/l)
  • B2 Neutrophil leucocytosis (neutrophil count >10 x 10^9/l in non-smokers, >=12.5 x 10^9/l in smokers)
  • B3 Radiological evidence of splenomegaly
  • B4 Low serum erythropoietin

*This is a very rare clinical entity

Reference:

McMullin MF et al. A guideline for the diagnosis and management of polycythaemia vera. A British Society for Haematology Guideline.British Journal of Haematology, 2019, 184, 176-191

Last edited 01/2020 and last reviewed 01/2020

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