Table 1.
PVSGa | 2008 WHOb |
---|---|
Major criteria | |
Elevated red cell mass | Hemoglobin >18.5 g/dL in men, 16.5 g/dL in women or other evidence of increased red cell volume* |
Normal arterial O2 saturation | Presence of the JAK2 mutation or other functionally similar mutation such as JAK2 exon 12 deletion |
Splenomegaly | |
Minor criteria** | |
Leukocytosis >12,000 | Bone marrow biopsy showing hypercellularity for age with trilineage growth (panmyelosis) with prominent erythroid, granulocytic, and megakaryocytic proliferation |
Thrombocytosis >400,000 | Serum EPO level below the reference range for normal |
Leukocyte alkaline phosphatase >100 | Endogenous erythroid colony formation in vitro |
Serum B12>900 or B12 binding capacity >2,200 |
For the PVSG criteria, two of the minor criteria must be present in cases without splenomegaly
Diagnosis requires both major criteria and one minor criterion or the presence of the first major criterion together with two minor criteria