Table 2.

World Health Organization (WHO) criteria for polycythemia vera (PV). Diagnosis of PV requires the presence of the first two A criteria together with either any one other A criterion or two B criteria.

A Criteria 
  1. Elevated red cell mass > 25% above mean normal predicted value, or hemoglobin > 18.5 g/dL in men, 16.5 g/dL in women, or > 99th percentile of method-specific reference range for age, sex, altitude of residence.

  2. No cause of secondary erythrocytosis, including:

    • Absence of familial erythrocytosis

    • No elevation of erythropoietin due to;

      • = 92%) Hypoxia (arterial PO2

      • High oxygen affinity hemoglobin

      • Truncated erythropoietin receptor

      • Inappropriate erythropoietin production by tumor

  3. Splenomegaly

  4. Clonal genetic abnormality other than Philadelphia chromosome or BCR-ABL fusion gene in marrow cells

  5. Endogenous erythroid colony formation in vitro

 
B Criteria 
  1. Thrombocytosis > 400 × 109/L

  2. Leukocytosis > 12 × 109/L

  3. Bone marrow biopsy showing panmyelosis with prominent erythroid and megakaryocytic proliferation4. Low serum erythropoietin levels

 
A Criteria 
  1. Elevated red cell mass > 25% above mean normal predicted value, or hemoglobin > 18.5 g/dL in men, 16.5 g/dL in women, or > 99th percentile of method-specific reference range for age, sex, altitude of residence.

  2. No cause of secondary erythrocytosis, including:

    • Absence of familial erythrocytosis

    • No elevation of erythropoietin due to;

      • = 92%) Hypoxia (arterial PO2

      • High oxygen affinity hemoglobin

      • Truncated erythropoietin receptor

      • Inappropriate erythropoietin production by tumor

  3. Splenomegaly

  4. Clonal genetic abnormality other than Philadelphia chromosome or BCR-ABL fusion gene in marrow cells

  5. Endogenous erythroid colony formation in vitro

 
B Criteria 
  1. Thrombocytosis > 400 × 109/L

  2. Leukocytosis > 12 × 109/L

  3. Bone marrow biopsy showing panmyelosis with prominent erythroid and megakaryocytic proliferation4. Low serum erythropoietin levels

 
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