Copyright ©The Author(s) 2015.
World J Hematol. Aug 6, 2015; 4(3): 16-53
Published online Aug 6, 2015. doi: 10.5315/wjh.v4.i3.16
Table 7 2015 World Health Organization Clinical Molecular and Pathological criteria for the diagnosis of prodromal, masked and classical JAK2 mutated polycythemia vera vs primary or secondary erythrocytoses[77,78]
CM criteriaBone marrow pathology (P) criteria (WHO)
Major criteria for PVP1 Bone marrow pathology: increased cellularity (60%-100%) due to trilinear increase of erythropoiesis, megakaryopoiesis and granulopoiesis and clustering of small to giant (pleomorph) megakaryocytes with hyperlobulated nuclei
A1 Hematocrit > 0.51/> 0.48 in male/female Erythrocytes > 5.8 × 1012/L males > 5.6 × 1012/L femalesAbsence of stainable iron. No pronounced inflammatory reaction
A2 Presence of heterozygous and/or homozygous JAK2V617F or JAK2 exon 12 mutationP2 Erythrocytosis. Normal erythropoiesis, normal granulopoiesis and megakaryocytes of normal size, morphology and no clustering
A3 Low serum Epo levelGrading of RF and MF
MinorPrefibrotic: RF-0/1 = MF-0
B1 Persistent increase of platelet count × 109/L:Early fibrotic: RF-2 = MF-1
grade 1: 400-1500, grade 2: > 1500Fibrotic: RCF 3 = MF-2
B2 Granulocytes > 10 × 109/L or Leukocytes > 12 × 109/L and raisedPost-PV MF: RF 4 = MF-3
LAP-score or increased CD11b expression in the absence of fever or infection
B3 Splenomegaly on ultrasound echogram (> 12 cm length in diameter) or on palpation
B4 Spontaneous EEC formation (optional)