What is the management approach for a patient presenting with chest pain due to polycythemia?

Management of Chest Pain in Polycythemia Vera

Chest pain in polycythemia vera requires immediate therapeutic phlebotomy to reduce hematocrit below 45% along with low-dose aspirin (81-100 mg daily) to reduce thrombotic risk. This combined approach is the cornerstone of management for chest pain in polycythemia vera patients as it directly addresses the hyperviscosity and increased thrombotic risk that are the primary causes of cardiovascular symptoms.

Initial Assessment and Management

Immediate Interventions

• Obtain ECG to rule out acute coronary syndrome 1
• Check hematocrit level (target <45%) 2, 3
• Administer low-dose aspirin (81-100 mg) if not contraindicated 1, 4
• Perform therapeutic phlebotomy if hematocrit >45% 2, 3
• Provide supplemental oxygen if hypoxemia is present 1

Risk Stratification

Classify the patient based on:

• Age >60 years (high risk)
• Previous history of thrombosis (high risk)
• Presence of cardiovascular risk factors
• Current hematocrit level
• Platelet count (extreme thrombocytosis >1,500 × 10⁹/L may contraindicate aspirin) 1, 2

Diagnostic Workup

Essential Tests

• Complete blood count with differential
• JAK2 V617F mutation testing (sensitivity >95% for polycythemia vera) 2
• Serum erythropoietin level (low in primary polycythemia) 2
• Cardiac biomarkers (troponin, CK-MB) 1
• Chest X-ray to rule out other causes of chest pain 1
• Echocardiography if hemodynamic disturbances are present 1

Definitive Management

For All Patients with Polycythemia Vera

1. Therapeutic phlebotomy

   • Target hematocrit <45% to significantly reduce thrombotic events 2, 3
   • Initial frequency may be high (2-3 times weekly) until target achieved 2
   • Monitor for iron deficiency with repeated phlebotomies 2

2. Low-dose aspirin (81-100 mg daily)

   • Reduces risk of thrombotic complications by 60% 4
   • Particularly effective for microcirculatory symptoms including chest pain 1, 4
   • Contraindicated if platelet count >1,500 × 10⁹/L due to bleeding risk 1, 2

For High-Risk Patients (Age >60 or Prior Thrombosis)

Add cytoreductive therapy:

• Hydroxyurea (first-line): Initial dose 15-20 mg/kg/day 1, 2
• Interferon-alfa (alternative first-line, especially in younger patients) 1, 2
• Ruxolitinib (second-line): For patients intolerant/resistant to hydroxyurea 5

Special Considerations

Acute Coronary Syndrome

If ACS is suspected:

• Follow standard ACS protocols with some modifications 1
• Consider early invasive strategy rather than thrombolytics due to bleeding risk 1
• Maintain antiplatelet therapy unless extreme thrombocytosis is present 1

Erythromelalgia

• May present with burning pain and erythema in extremities but can manifest as atypical chest discomfort 1
• Responds dramatically to low-dose aspirin (within hours) 1

Monitoring and Follow-up

• Regular CBC monitoring every 2-3 months initially, then every 3-6 months if stable 2
• Periodic evaluation of spleen size 2
• Aggressive control of cardiovascular risk factors 2
• Monitor for disease progression to myelofibrosis or acute leukemia 3, 6

Pitfalls to Avoid

1. Failing to maintain hematocrit <45%, which significantly increases thrombotic risk 2, 3
2. Withholding aspirin therapy without clear contraindication 4, 7
3. Aggressive phlebotomy leading to iron deficiency, which can paradoxically increase stroke risk 2
4. Overlooking other causes of chest pain (aortic dissection, pulmonary embolism, pericarditis) 1
5. Delaying cytoreductive therapy in high-risk patients 1, 6

By following this approach, the risk of thrombotic complications, which are the major cause of morbidity and mortality in polycythemia vera patients presenting with chest pain, can be significantly reduced.