RISK FACTORS: Jewish ancestry (may have increased frequency), Familial history (rare)
GENERAL MEASURES
Individualized management necessary. Dependent on many factors - age, disease duration, disease
phenotype, complications, disease activity. Currently, phlebotomy is mainstay of therapy. Beyond that, differences exist among authorities about use and effectiveness of myelosuppressives.
. Phlebotomy
. To reduce hematocrit to approximately 45%
. Performed as often as every 2 or 3 days until normal hematocrit reached. Phlebotomies of 250-500 m/L.
Reduce to 250-350 m/L in elderly patients or patients with cardiovascular disease.
. Concomitant therapy possibilities, e.g., some form of myelosuppression, radioactive phosphorus (in elderly
patients), hydroxyurea
. Phlebotomy repeated as necessary for maintenance
. If patient cannot tolerate phlebotomy - chemotherapy (hydroxyurea is the least mutagenic agent) or radiation
therapy
. Other therapy
. Maintain hydration
. Pruritus therapy
. Manage thrombotic or hemorrhagic complications the same as with nonpolycythemic patient
. Uric acid reduction therapy
ACTIVITY No restrictions
DIET
. No special diet (iron replacement not necessary)
. Phlebotomy regimen will produce pica, resulting in craving for crisp green vegetables (lettuce, celery) and ice
PATIENT EDUCATION
. Lifelong maintenance
. Complications to watch for Polycythemia vera
DRUG(S) OF CHOICE
. Adjunctive
. Allopurinol 300 mg/day for uric acid reduction
. Cyproheptadine for pruritus, 4-16 mg as needed
. H2-receptor blockers or antacids for GI hyperacidity
. Myelosuppression
. Radioactive phosphorous in selected cases
. Busulfan or alkylating agents (e.g., hydroxyurea )
. Low-dose aspirin or anagrelide; aspirin is controversial in view of bleeding risk, but small doses may be
given if required
. Note: Refer to hematologist/oncologist for dosages and instructions
ALTERNATIVE DRUGS
β’ Myelosuppression: chlorambucil, some authors believe contraindicated
β’ Interferon-alpha (IFN-a) may be an effective alternative to present forms of treatment and is under investigation
PATIENT MONITORING
β’ Frequent during early treatment until satisfactory hematocrit is reached
β’ Monitor hematocrit often and phlebotomize when needed
PREVENTION/AVOIDANCE No known preventive measures
POSSIBLE COMPLICATIONS
β’ Uric acid stones
β’ Secondary gout
β’ Vascular thromboses (major cause of death)
β’ Transformation to leukemia
β’ Hemorrhage
β’ Peptic ulcer
β’ Increased risk for complications and mortality from surgery procedures. Assess risk-benefi ts and assure
optimal control of disorder before any elective surgery.
EXPECTED COURSE/PROGNOSIS
β’ Median survival without treatment - 6 to 18 months following diagnosis
β’ Survival up to 10 years with treatment
β’ Some patients live, symptom-free, for 20 or more years