Preoperative Optimization for Polycythemia Vera Patients
For a patient with polycythemia vera to be considered fit for elective surgery, maintain hematocrit <45% for at least 3 months preoperatively through phlebotomy and/or cytoreductive therapy, normalize or near-normalize complete blood count (particularly platelet and white cell counts) without causing prohibitive cytopenias, discontinue aspirin one week before surgery, and ensure multidisciplinary assessment including coagulation testing for acquired von Willebrand disease. 1
Hematocrit Target and Control
- The critical hematocrit threshold is <45%, which must be maintained through phlebotomy and/or cytoreductive therapy to reduce thrombotic risk 1, 2
- This target is based on evidence showing increased vascular occlusive episodes at hematocrit levels above 44%, with suboptimal cerebral blood flow occurring between 46-52% 3
- Hematocrit control must be documented for 3 months prior to elective surgery, with additional phlebotomy potentially needed immediately before the procedure 1
- Phlebotomy should be performed with careful fluid replacement to avoid hypotension and fluid overload, particularly in patients with cardiovascular disease 3
Cytoreductive Therapy Management
- Initiate or optimize cytoreductive therapy if not already established, as this reduces early thrombotic complications compared to phlebotomy alone 3, 1
- Hydroxyurea can typically be continued perioperatively and should not be stopped unnecessarily 1
- The goal is normalization or near-normalization of complete blood count without causing prohibitive cytopenias before proceeding with elective surgery 3, 1
Platelet and White Cell Count Targets
- Platelet counts should be normalized or near-normalized, particularly if exceeding 1,000/mm³ 1
- Maintaining platelet counts below 400 × 10⁹/L provides optimal control of vascular complications 4
- White cell counts should similarly be normalized or near-normalized as part of overall disease control 3, 1
Aspirin Management
- Discontinue aspirin one week (7 days) prior to surgery 1
- Restart aspirin 24 hours postoperatively or when bleeding risk is acceptable 1
- Low-dose aspirin (81-100 mg daily) is standard therapy for polycythemia vera patients, though one-third may display less-than-maximal platelet inhibition 5
Coagulation Assessment
- Perform coagulation tests to evaluate for acquired von Willebrand disease and other coagulopathies before high-risk surgical procedures 3, 1
- This is particularly critical in patients with extreme thrombocytosis (platelet count ≥1,000 × 10⁹/L), as acquired von Willebrand disease increases bleeding risk 2
- Failing to assess for this condition can lead to unexpected severe intraoperative bleeding 1
Anticoagulation Management
- Withhold anticoagulant therapy based on the half-life and type of agent prior to surgery 1
- Restart anticoagulant therapy postoperatively when bleeding risk is acceptable 1
- For patients on chronic anticoagulation, follow current American College of Chest Physicians guidelines for perioperative management 3
Thromboprophylaxis Strategy
- Extended prophylaxis with low molecular weight heparin (LMWH) should be considered for high-risk procedures, particularly orthopedic and cardiovascular surgeries 1, 6
- Standard duration is at least 7-10 days postoperatively, with extended prophylaxis up to 4 weeks for high-risk features 6
- Mechanical prophylaxis with intermittent pneumatic compression devices should be used in addition to pharmacological prophylaxis for at least 18 hours per day 6
Hydration Management
- Ensure appropriate and monitored fluid replacement during phlebotomy to avoid both hypotension and fluid overload 3
- Maintain adequate hydration perioperatively, as polycythemia vera patients are at increased risk for thrombotic complications with dehydration 1
Multidisciplinary Team Requirements
- Mandatory multidisciplinary management involving hematologist/oncologist, surgeon, and anesthesiologist 1
- Comprehensive review of bleeding and thrombosis history and current medication list is essential 3, 1
- The surgeon must assess procedure-specific bleeding risk, particularly for orthopedic and cardiovascular procedures 3, 1
High-Risk Surgical Considerations
- The thrombotic and bleeding risk of the specific surgical procedure must be strongly considered before proceeding 3, 1
- Even with optimal management, polycythemia vera patients face 7.7% vascular occlusion risk and 7.3% major hemorrhage risk perioperatively 1
- Orthopedic surgeries and procedures associated with prolonged immobilization carry particularly high risk 3, 1
Critical Pitfalls to Avoid
- Do not proceed with elective surgery without adequate 3-month hematologic control, as this substantially increases thrombotic risk 1
- Do not fail to test for acquired von Willebrand disease in patients with extreme thrombocytosis, as this can cause unexpected severe bleeding 1
- Do not stop cytoreductive therapy unnecessarily, particularly hydroxyurea which can be continued perioperatively 1
- Do not provide inadequate thromboprophylaxis for high-risk procedures—polycythemia vera patients require extended LMWH prophylaxis 1
- Do not restart aspirin too early—wait until adequate hemostasis is achieved, typically 24 hours postoperatively 1