What is the appropriate management for a patient with confirmed polycythemia and symptoms concerning for hyperviscosity syndrome?

Management of Polycythemia with Symptoms of Hyperviscosity Syndrome

Immediate therapeutic phlebotomy is required for this patient with confirmed polycythemia and symptoms of hyperviscosity syndrome (dizziness), along with low-dose aspirin therapy and cytoreductive treatment if high-risk factors are present. 1

Initial Assessment and Management

• Confirm diagnosis: The patient's lab values (RBC 6.20, hemoglobin 18.3, hematocrit 55.1%) are consistent with polycythemia, and the "confirmed by repeat analysis" notation supports this diagnosis.

• Urgent intervention: Dizziness in this setting suggests hyperviscosity syndrome, which requires prompt intervention to prevent serious complications such as thrombosis.

• Immediate therapeutic phlebotomy: Remove 450-500 mL of blood with volume replacement to rapidly reduce hematocrit below 45%. 1

  • Monitor vital signs during the procedure
  • Ensure adequate hydration with intravenous or oral fluids
  • Recheck hemoglobin and hematocrit after the procedure

Risk Stratification

This patient should be classified as high-risk based on:

• History of incarceration and past drug use (potential for poor follow-up)
• Symptomatic disease (dizziness suggesting hyperviscosity)
• Significantly elevated hematocrit (55.1%)

Risk stratification for polycythemia vera typically includes:

• Low risk: Age <60 years with no history of thrombosis
• High risk: Age ≥60 years or prior history of thrombosis 1, 2

Comprehensive Treatment Plan

1. Therapeutic phlebotomy:

   • Target hematocrit <45% in men 1
   • Initial phlebotomy regimen: 300-450 mL weekly or twice weekly until target hematocrit is reached 2
   • Maintenance phlebotomy schedule adjusted based on hematocrit levels

2. Low-dose aspirin therapy:

   • Start aspirin 81-100 mg daily (unless contraindicated) 2, 1
   • Monitor for bleeding complications

3. Cytoreductive therapy:

   • Indicated for this patient due to symptomatic disease (dizziness) 2
   • First-line option: Hydroxyurea starting at 500 mg twice daily 1, 3
   • Monitor for myelosuppression with weekly blood counts initially 3
   • Reduce dose by 50% if creatinine clearance <60 mL/min 3

4. Hematology referral:

   • Essential for further evaluation and management
   • Should be expedited given the symptomatic presentation

Monitoring and Follow-up

• Repeat CBC within 24 hours as planned
• Regular follow-up every 3-6 months with CBC and symptom assessment 1
• Monitor for:
  • Resolution of hyperviscosity symptoms
  • Thrombotic complications
  • Bleeding complications
  • Disease progression to myelofibrosis or acute leukemia
  • Iron deficiency from repeated phlebotomies

Special Considerations

• Hyperviscosity symptoms: Closely monitor for worsening dizziness, headache, visual disturbances, or neurological symptoms that may indicate progressive hyperviscosity 4

• Secondary causes: Evaluate for potential secondary causes of polycythemia, particularly given the fluctuating values in the past

• Medication side effects: Watch for potential side effects of hydroxyurea including myelosuppression, mucocutaneous manifestations, and leg ulcers 3

• Thrombosis prevention: Emphasize importance of hydration and avoiding prolonged immobility

• Avoid inappropriate phlebotomies: Do not perform phlebotomy if iron deficiency develops (monitor MCV) 1

The CYTO-PV trial demonstrated that maintaining hematocrit <45% significantly reduces cardiovascular death and major thrombotic events (HR 3.91,95% CI 1.45-10.53), highlighting the importance of strict hematocrit control in this patient 1.