What are the treatment options for erythrocytosis (high red blood cell count)?

From the Guidelines

Therapeutic phlebotomy should only be performed in patients with high red blood cell count (erythrocytosis) who have moderate to severe hyperviscosity symptoms, a hematocrit greater than 65%, and no evidence of dehydration or iron deficiency, as stated in the guidelines 1. The treatment approach for erythrocytosis varies depending on the underlying cause and severity of the condition.

• For patients with secondary erythrocytosis due to chronic hypoxemia, therapeutic phlebotomy may be necessary to relieve hyperviscosity symptoms, but it should be done with caution and only when the hemoglobin is more than 20 g/dL and the hematocrit is greater than 65% 1.
• In cases of iron deficiency, oral iron supplementation may be necessary, but it should be done with caution and close monitoring of hemoglobin levels to avoid a rapid increase in red cell mass 1.
• Adequate hydration is essential for all patients with erythrocytosis to reduce blood viscosity and prevent complications like thrombosis or bleeding.
• Regular monitoring of blood counts is crucial to adjust treatment and prevent complications. Key considerations in the management of erythrocytosis include:
• Avoiding repeated routine phlebotomies due to the risk of iron depletion, decreased oxygen-carrying capacity, and stroke 1
• Addressing the underlying cause of secondary erythrocytosis, such as stopping smoking, treating sleep apnea, or managing hypoxic lung disease
• Treating the primary condition in cases of inappropriate erythropoietin production, such as kidney disease or tumors.

From the FDA Drug Label

Hydroxyurea capsules are an antimetabolite indicated for the treatment of: Resistant chronic myeloid leukemia. Locally advanced squamous cell carcinomas of the head and neck, (excluding lip) in combination with concurrent chemoradiation

The treatment options for erythrocytosis (high red blood cell count) may include hydroxyurea, as it is indicated for the treatment of resistant chronic myeloid leukemia, which can be associated with erythrocytosis.

• Key points:
  • Hydroxyurea is an antimetabolite that can help reduce red blood cell count.
  • It is essential to monitor blood counts at baseline and throughout treatment.
  • The dose of hydroxyurea may need to be adjusted based on the patient's response to treatment and renal function.
  • Hydroxyurea can cause myelosuppression, hemolytic anemia, and other adverse reactions, so close monitoring is necessary 2, 2.

From the Research

Treatment Options for Erythrocytosis (High Red Blood Cell Count)

• The primary goal of treatment is to prevent thrombohemorrhagic complications and reduce the risk of thrombosis 3, 4, 5, 6, 7
• Treatment options include:
  • Phlebotomy to keep hematocrit below 45% 3, 4, 5, 6, 7
  • Low-dose aspirin (if no contraindications) 3, 4, 5, 6, 7
  • Cytoreductive therapy with hydroxyurea or interferon to lower thrombosis risk and decrease symptoms 3, 4, 5, 6, 7
  • Ruxolitinib, a Janus kinase inhibitor, to alleviate pruritus and decrease splenomegaly in patients who are intolerant of or resistant to hydroxyurea 4, 5, 7
• Risk stratification is important to determine the best course of treatment:
  • High-risk patients (age >60 years or prior thrombosis) may benefit from cytoreductive therapy with hydroxyurea or interferon 4, 5, 6
  • Low-risk patients may only require phlebotomy and low-dose aspirin 4, 5, 6
• Additional considerations:
  • Screening for acquired von Willebrand syndrome (AvWS) is recommended before administering aspirin in patients with extreme thrombocytosis 6
  • Modifiable risk factors such as smoking cessation and cardiometabolic disease should be addressed to reduce the risk of thrombosis 7