From the Guidelines
The primary management of erythroid hyperplasia involves treating the underlying cause and monitoring blood counts, with a focus on judicious phlebotomy to alleviate symptoms of hyperviscosity and improve hemodynamic function, as seen in conditions like cyanotic congenital heart disease and high oxygen-affinity hemoglobinopathy 1. Here's a concise approach:
- Identify and treat the underlying cause:
- In cases of secondary erythroid hyperplasia, such as cyanotic congenital heart disease, judicious phlebotomy to a hematocrit level of 60% is reasonable and may alleviate symptoms of hyperviscosity 1
- In chronic obstructive pulmonary disease (COPD), graded phlebotomy to a hematocrit range of 55% to 60% may improve both exercise tolerance and cardiac function 1
- Post–renal transplant erythrocytosis can be managed with angiotensin-converting enzyme (ACE) inhibitors or angiotensin II receptor inhibitors, which have been shown to lower hematocrit levels 1
- Monitor complete blood count (CBC) regularly:
- Initially every 2-4 weeks, then less frequently as improvement occurs
- Consider bone marrow biopsy if diagnosis is unclear or response to treatment is inadequate
- In cases of primary erythroid hyperplasia (e.g., polycythemia vera):
- Phlebotomy to maintain hematocrit <45%
- Low-dose aspirin (81-100 mg daily) to reduce thrombotic risk
- Consider cytoreductive therapy (e.g., hydroxyurea) in high-risk patients
- Supportive care:
- Manage symptoms like fatigue or pruritus
- Educate patients on recognizing signs of complications (e.g., thrombosis) The goal is to normalize red blood cell production by addressing the underlying cause, which often involves correcting nutritional deficiencies or managing myeloproliferative disorders, and to prevent potential complications associated with persistent erythroid hyperplasia, as supported by the most recent and highest quality study 1.
From the Research
Management of Erythroid Hyperplasia
The management of erythroid hyperplasia, particularly in the context of polycythemia vera (PV), involves several key strategies:
- Therapeutic Phlebotomy: All patients with PV should receive therapeutic phlebotomy to maintain a hematocrit of less than 45% 2, 3, 4, 5, 6.
- Low-Dose Aspirin: Low-dose aspirin is recommended for all patients with PV, unless there are contraindications 2, 3, 4, 5, 6.
- Cytoreductive Therapy: Patients at high risk of thrombosis, including those aged 60 years or older or with a prior thrombosis, may benefit from cytoreductive therapy with hydroxyurea or interferon to lower thrombosis risk and decrease symptoms 2, 3, 4, 5, 6.
- Risk Stratification: Patients with PV are stratified into high-risk and low-risk categories based on age and thrombosis history, with high-risk patients requiring more aggressive management 2, 3, 4, 5, 6.
- Monitoring and Adjustment: Treatment plans should be regularly monitored and adjusted as needed to minimize the risk of thrombosis and other complications 2, 3, 4, 5, 6.
Treatment Options
The following treatment options are available for patients with PV:
- Hydroxyurea: First-line cytoreductive therapy for high-risk patients 2, 3, 4, 5, 6.
- Interferon: Second-line cytoreductive therapy for patients who are intolerant of or have an inadequate response to hydroxyurea 2, 3, 4, 5, 6.
- Ruxolitinib: Janus kinase inhibitor that can alleviate pruritus and decrease splenomegaly in patients who are intolerant of or resistant to hydroxyurea 2, 4, 5.