- General Information
- Polycythemia
- Defined as increase in # RBC in the peripheral blood
- Men Hgb >18.5 or Hct >52
- Women Hgb > 16/5 or Hct >48
- Relative polycythemia
- A decrease in plasma volume that elevates the Hct or Hgb
- Absolute polycythemia
- Primary – mutations such as polycythemia vera, idiopathic familial polycythemia, EPO R gene mutations
- Secondary – chronic hypoxia (high altitude, smoking, cardiac shunts, pulmonary disease, sleep apnea, EPO producing tumors (RCC, HCC, pheo, uterine fibroids), other (use of EPO injection, steroids, blood doping)
- Polycythemia
- Symptoms
- Indicating the hyperviscosity: chest pain, abd pain, HA, change in vision or mentation
- Indicating polycythemia vera: post bath pruritis, erthromelalgia, gout , arterial or venous thromboses
- Diagnosis
- Management
- Phlebotomy: often to a goal hct below 45 for men or 42 for women.
- Low dose ASA to reduce thrombotic complications
- Cytoreductive therapy: i.e. hydroxyurea
- Other therapies:
- Interferon
- Anagrelide (inhibits platelet maturation): PCV with secondary thrombocytosis
- Erlotinib
- Selective JAK2 inhibitors are being investigated
