Treatment for Erythrocytosis (Elevated Red Blood Cell Count)
Therapeutic phlebotomy is the primary treatment for patients with erythrocytosis when hemoglobin exceeds 20 g/dL and hematocrit exceeds 65%, accompanied by symptoms of hyperviscosity such as headache, fatigue, or poor concentration. 1
Diagnostic Approach
- Determine if erythrocytosis is primary (intrinsic bone marrow problem) or secondary (external stimuli driving erythropoiesis) to identify the underlying cause 2
- Measure erythropoietin levels as a first step in diagnosis - low levels indicate primary causes while normal or elevated levels suggest secondary etiologies 3
- Evaluate for symptoms of hyperviscosity including headache, visual disturbances, fatigue, and poor concentration 2
- Consider JAK2V617F mutation testing to rule out polycythemia vera, especially in cases without obvious secondary causes 4
First-Line Management
- Ensure adequate hydration as initial therapy for all patients with erythrocytosis 2
- Identify and treat underlying causes of secondary erythrocytosis (hypoxemia, sleep apnea, cyanotic heart disease) 2
- Avoid routine or repeated phlebotomies without clear indications due to risk of iron depletion 2
Therapeutic Phlebotomy Protocol
- Remove one unit of blood (approximately 400-500 mL) per session 1
- Replace with equal volume of isotonic saline or dextrose (750-1000 mL) to maintain intravascular volume 1
- For induction phase, perform phlebotomy weekly or fortnightly until target levels are reached 1
- Target serum ferritin of 50 μg/L during induction phase, but not lower to avoid iron deficiency 1
- For maintenance phase, individualize frequency based on the rate of red cell production 1
Special Considerations
Iron Status Monitoring
- Regular monitoring of iron status is essential in patients undergoing repeated phlebotomies 2
- Iron deficiency can paradoxically worsen symptoms by reducing oxygen-carrying capacity and red cell deformability 2
- Monitor for signs of iron deficiency through peripheral blood smear, serum ferritin, or transferrin saturation 1
Alternative Treatments
- Consider erythrocytapheresis in selected cases where phlebotomy is not feasible 5, 2
- Erythrocytapheresis allows for fewer hemodynamic changes compared to phlebotomy and returns valuable blood components 5
- Iron chelation therapy (such as deferasirox) can be considered as a second-line option when phlebotomy is not possible, after careful risk-benefit assessment 5, 1
Special Populations
- For pregnant women with mild to moderate iron overload without advanced liver disease, phlebotomy can be paused during pregnancy 1
- In patients with congenital heart disease and cyanosis, judicious phlebotomy to a hematocrit level of 60% may be reasonable to alleviate hyperviscosity symptoms 1
- For patients with polycythemia vera, maintain hematocrit below 45% in men and at appropriate corresponding values for women 1
Common Pitfalls to Avoid
- Avoid performing routine phlebotomies without meeting established criteria (Hgb >20 g/dL, Hct >65%) 2
- Creating iron deficiency through excessive phlebotomy can worsen symptoms 2
- Phlebotomy should not be performed in the presence of dehydration or anemia 1
- Repeated routine phlebotomies are not recommended due to risk of iron depletion, decreased oxygen-carrying capacity, and increased stroke risk 1
Monitoring Recommendations
- Regularly follow up with complete blood counts to monitor hemoglobin and hematocrit 2
- Assess for symptoms of hyperviscosity before each phlebotomy session 1
- Monitor for thrombotic complications, which can occur with untreated erythrocytosis 4
- Evaluate renal function, as chronic erythrocytosis can affect renal glomeruli 2