From the Guidelines
The management of erythrocytosis should focus on treating the underlying cause while reducing blood viscosity to prevent thrombotic complications, with therapeutic phlebotomy being the cornerstone treatment for primary erythrocytosis and management targeting the underlying condition for secondary erythrocytosis. For primary erythrocytosis (polycythemia vera), therapeutic phlebotomy is recommended, removing 450-500 mL of blood every 2-3 days until the hematocrit is below 45% for men and 42% for women, then maintaining these targets with periodic phlebotomies 1. Cytoreductive therapy with hydroxyurea (starting at 500-1000 mg daily) is added for high-risk patients (age >60 or history of thrombosis) 1. Low-dose aspirin (81-100 mg daily) is recommended to reduce thrombotic risk. For secondary erythrocytosis, management targets the underlying condition—discontinuing testosterone or erythropoietin therapy if iatrogenic, providing supplemental oxygen for hypoxic conditions, or treating renal or hepatic diseases 1. Phlebotomy is used selectively in secondary cases when the hematocrit exceeds 54% or patients are symptomatic. Adequate hydration is essential for all patients to reduce blood viscosity. Regular monitoring of complete blood counts is necessary to guide therapy and detect complications. Some key points to consider in the management of erythrocytosis include:
- The importance of individualized treatment approaches, particularly in patients with cyanotic heart disease 1
- The need to balance the risks and benefits of therapeutic phlebotomy, taking into account the potential for iron depletion and decreased oxygen-carrying capacity 1
- The role of iron supplementation in patients with iron deficiency, with careful monitoring to avoid rebound effects 1
- The use of cytoreductive therapy, such as hydroxyurea, in high-risk patients with primary erythrocytosis 1. This approach effectively balances the need to reduce thrombotic risk while addressing the physiological mechanisms driving red cell overproduction.
From the Research
Management Approach for Erythrocytosis
The management of erythrocytosis involves several steps, including diagnosis, risk stratification, and treatment.
- Diagnosis: The diagnostic approach for erythrocytosis involves a careful history and examination, followed by measurement of erythropoietin (EPO) levels 2.
- Risk Stratification: Patients with polycythemia vera (PV) are stratified according to age and history of thrombosis 3.
- Treatment: Treatment options for erythrocytosis include phlebotomy, cytoreductive therapy, and aspirin.
- Phlebotomy: Phlebotomy is used to reduce hematocrit levels and is often used in combination with cytoreductive therapy 3, 4.
- Cytoreductive Therapy: Cytoreductive therapy, such as hydroxyurea or interferon-alpha, is used to reduce blood cell counts and prevent thrombosis 3, 5, 4.
- Aspirin: Aspirin is used to prevent thrombosis and is often given to patients with PV 3, 5.
Specific Treatment Options
- Hydroxyurea: Hydroxyurea is a first-line cytoreductive therapy for high-risk patients with PV, but its use is associated with toxicities and resistance in some patients 5.
- Interferon-alpha: Interferon-alpha is a preferred first-line agent for some patients with PV due to its improved survival outcomes 4.
- Ruxolitinib: Ruxolitinib is an option for patients with hydroxyurea-resistant or intolerant PV 5.
- Venesection: Venesection is a therapeutic option for patients with erythrocytosis, particularly those with high oxygen-affinity hemoglobins 2.