A criteria:
(1) elevated red cell mass - one or more of the following:
(1a) > 25% above mean normal predicted value
(1b) hemoglobin > 18.5 g/dL for male or > 16.5 g/dL for female
(1c) hemoglobin > 99%ile for age, sex and altitude of residence
(2) absence of causes for secondary erythrocytosis
(3) splenomegaly
(4) clonal genetic abnormality in marrow cells AND absence of the Philadelphia chromosome or BCR-ABL
(5) endogenous formation of erythroid colonies (with in vitro cultures)
where:
• Secondary causes include: familial erythrocytosis, inappropriate production of erythropoietin by a tumor, associated with a truncated erythropoietin receptor, secondary to a hemoglobin with high oxygen affinity, or secondary to chronic hypoxemia
B Criteria:
(1) thrombocytosis > 400,000 per µL (> upper limit of normal reference range)
(2) leukocytosis > 12,000 per µL
(3) panmyelosis with erythroid and megakaryocytic hyperplasia on bone marrow biopsy
(4) low serum erythropoietin concentration
Criteria for diagnosis of polycythemia vera - both of the following:
(1) criteria A1 and A2
(2) one or both of the following:
(2a) >= 1 of A3, A4 or A5
(2b) >= 2 of B1 to B4
