Table 1.

Proposed diagnostic criteria for polycythemia vera (PV).

a These hematocrit values are invariably associated with a raised red cell mass in an adult population. 
b Note that it is possible in rare cases for PV to coexist with a cause of secondary erythrocytosis. 
A1. Raised red cell mass (> 25% above predicted, or hematocrit ≥ 0.60 in males or > 0.56 in females)a 
A2. Absence of causes of secondary erythrocytosis (normal arterial oxygen saturation and no elevation of serum erythropoietin.)b 
A3. Palpable splenomegaly 
A4. Presence of JAK2 V617F mutation or other cytogenetic abnormality (excluding BCR-ABL) in hemopoietic cells 
B1. Thrombocytosis (platelets > 400 x 109/L) 
B2. Neutrophilia (neutrophils > 10 x 109/L; > 12.5 x 109/L in smokers) 
B3. Radiological splenomegaly 
B4. Endogenous erythroid colonies or low serum erythropoietin 
A1 + A2 + either another A or two B criteria is required for a diagnosis of PV 
a These hematocrit values are invariably associated with a raised red cell mass in an adult population. 
b Note that it is possible in rare cases for PV to coexist with a cause of secondary erythrocytosis. 
A1. Raised red cell mass (> 25% above predicted, or hematocrit ≥ 0.60 in males or > 0.56 in females)a 
A2. Absence of causes of secondary erythrocytosis (normal arterial oxygen saturation and no elevation of serum erythropoietin.)b 
A3. Palpable splenomegaly 
A4. Presence of JAK2 V617F mutation or other cytogenetic abnormality (excluding BCR-ABL) in hemopoietic cells 
B1. Thrombocytosis (platelets > 400 x 109/L) 
B2. Neutrophilia (neutrophils > 10 x 109/L; > 12.5 x 109/L in smokers) 
B3. Radiological splenomegaly 
B4. Endogenous erythroid colonies or low serum erythropoietin 
A1 + A2 + either another A or two B criteria is required for a diagnosis of PV 
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