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 2 Polycythemia vera study group criteria for polycythemia vera[10] and diagnostic differentiation of polycythemia vera from all variants of primary and secondary erythrocytoses by bone marrow histology[17]
Major criteria PVMinor criteria PV
A1 RCM, males > 36 mL/kg females > 32 mL/kg. HemoglobinB1 Thrombocytosis Platelet count > 400 × 109/L
18.5 g/dL male and > 16.5 g/dL females (PVSG, WHO)B2 Leukocytosis > 12 × 109/L
A2 Normal arterial oxygen saturation > 92%B3 Raised neutrophil alkaline phosphatase score > 100 or raised B12
A3 Splenomegaly on palpation(> 900 ng/L) or raised unsaturated B12 binding capacity (> 2200 ng/L)
B3 is replaced by spontaneous EEC as a specific clue to PV
Benign erythrocytosis: 1980 RCP criteriaMyeloproliferative PV: 1980 RCP criteria
RCM, males > 36 mL/kg females > 32 mL/kg or increased erythrocytes above 6 × 1012/LIncreased red cell counts > 6 × 1012/L or increased RCM and increase of clustered large megakaryocytes with hyperlobulated nuclei is a pathognomonic diagnostic clue to PV
Normal platelet and leukocyte countsNormal RCM = inapparent erythrocytosis is not associated with splenomegaly
Normal bone marrow histopathology: normal cellularity and erythropoiesis, and normal size, morphology andand shows normal bone histology, whereas IPV is associated with splenomegaly and show typical features of PV bone marrow histology
distribution of megakaryocytes
Classification of erythrocytoses[74]Notes anno 1980-1999[74]
Congenital or primary erythrocytosis including mutationIncreased RCM does not distinguish between PV and primary erythrocytosis
truncated EPO recepotor, disrupted oxygen homeostasis inIncreased RCM does not distinguish between PV and IPV. IPV is featured by
Chuvash erythrocytosis, high oxygen affinityadvanced PV with normal hb, Ht and erythrocyte count due to splenomegaly and
hemoglobinopathy, and congenital autonomous EPOhypersplenism and with increase of reticulin fibrosis with typical PV bone
productionmarrow features
Secondary erythrocytosis due to autonomous EPO production in renal diseases or by tumour cels or due to hypoxiaIn IPV the values of hemoglobin hematocrit and erythrocytes are normal but RCM is increased due to splenomegaly with absence of hypervolemic symptoms
Idiopathic erythrocytoses