Polycythemia Vera (Diagnostic Criteria)
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PV Major Diagnostic Criteria
☐Elevated RBC mass (> 36 mL/kg in men/ > 32 mL/kg in women)
☐O2 saturation >= 92%
☐Splenomegaly
PV Minor Diagnostic Criteria
☐Platelets > 400,000/(mm)^3
☐Leukocytosis > 12,000/(mm)^3
☐Leukocyte alkaline phosphatase >100 U/L (In the absence of fever or infection)
☐Vitamin B12 > 900 pg/mL (664 pmol/L) or serum B12-binding capacity>2200 pg/mL (1623 pmol/L)
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Polycythemia Vera (Diagnostic Criteria)

Polycythemia Vera (PV) is a chronic myeloproliferative disease that is typified by increased red blood cell mass (RCM), which leads to hyperviscosity and increased risk of thrombosis.

PV is diagnosed at median age of 60 years, and should be suspected in white men with hemoglobin level of >18 g/dL (180 g/L) or in blacks and women with >16 g/dL (160 g/L) who do not have another reason for this erythrocytosis. Additional findings that should lead to an evaluation for PV include splenomegaly (with presence of Leukocytosis) or portal venous thrombosis.

Diagnosis is important as median survival untreated is ~12 months, treated is >10 years.

Symptoms/Signs include:

Common:

  • Plethora
  • Pruritus after bathing
  • Splenomegaly
  • Sweating (Excessive)
  • Weakness
  • Weight loss

Less Common:

  • Bleeding
  • Bruising
  • Bud-Chiari syndrome
  • Chest pain (Non-cardiac)
  • Epistaxis
  • Gout
  • Hepatomegaly
  • Ischemia to the digits
  • Neurologic complaints (Dizziness, Headache, Paresthesias, Tinnitus, Vision changes)
  • Thrombosis
  • Transient ischemic attacks or stroke

The diagnosis of PV requires all 3 Major Criteria, or the first 2 of the major criteria, plus at least 2 of the 4 minor criteria. To make this diagnosis, a secondary cause for increased RBC mass must be excluded.

PV Major Diagnostic Criteria:

  • Elevated RBC mass (>36 mL/kg in men/>32 mL/kg in women)
  • O 2 saturation >=92%
  • Splenomegaly

PV Minor Diagnostic Criteria:

  • Platelets >400,000/mm 3
  • Leukocytosis >12,000/mm 3
  • Leukocyte alkaline phosphatase >100 U/L (In the absence of fever or infection)
  • Vitamin B 12 >900 pg/mL (664 pmol/L) or serum B 12 -binding capacity >2200 pg/mL (1623 pmol/L)

References:

  • Lamy T, Devillers A, Bernard M, et al. Inapparent polycythemia vera: an unrecognized diagnosis. Am J Med. 1997;102(1):14-20.
  • Murphy S. Diagnostic criteria and prognosis in polycythemia vera and essential thrombocythemia. Semin Hematol. 1999;36(1 Suppl 2):9-13.
  • Stuart BJ, Viera AJ. Polycythemia vera. Am Fam Physician. 2004;69(9):2139-44.
  • Tefferi A. Diagnosing polycythemia vera: a paradigm shift. Mayo Clin Proc. 1999;74(2):159-62.
  • Tefferi A. Polycythemia vera: a comprehensive review and clinical recommendations. Mayo Clin Proc. 2003;78(2):174-94.