What is the management approach for a 26-year-old with elevated Hemoglobin (HGB), Hematocrit (HCT), and Red Blood Cell (RBC) count?

From the Guidelines

The management approach for a 26-year-old with elevated Hemoglobin (HGB), Hematocrit (HCT), and Red Blood Cell (RBC) count should prioritize determining the underlying cause, with a focus on ruling out polycythemia vera and secondary polycythemia through diagnostic evaluations, including JAK2 mutation testing and serum erythropoietin levels 1. The initial steps in management include:

• Ruling out polycythemia vera through JAK2 mutation testing and serum erythropoietin levels
• Evaluating for secondary polycythemia causes, such as smoking, high-altitude living, or cardiopulmonary disease
• Assessing for symptoms of hyperviscosity, such as headache, fatigue, or poor concentration For symptomatic patients with confirmed polycythemia vera, therapeutic phlebotomy is the cornerstone of treatment, aiming to reduce hematocrit levels to below 45% for men or 42% for women, while also considering the use of low-dose aspirin to reduce thrombotic risk 1. Key considerations in management include:
• Regular monitoring of blood counts every 2-3 months to maintain hematocrit levels below target and prevent complications like thrombosis
• Lifestyle modifications, such as smoking cessation, adequate hydration, and avoiding extreme heat, to reduce blood viscosity and associated symptoms
• Cytoreductive therapy with hydroxyurea may be indicated for high-risk patients with thrombotic history or age >60, but this should be approached with caution and careful consideration of the individual patient's risk factors and overall health status 1.

From the Research

Elevated Hemoglobin, Hematocrit, and Red Blood Cell Count

• Elevated Hemoglobin (HGB), Hematocrit (HCT), and Red Blood Cell (RBC) count in a 26-year-old may be indicative of an underlying condition such as Polycythaemia vera (PV) 2, 3.
• PV is a haematological malignancy characterized by erythrocytosis, often accompanied by leukocytosis and thrombocytosis 2.
• The diagnosis of PV typically involves testing for the JAK2V617F mutation, which is present in more than 95% of patients with PV 2, 3.

Management Approach

• The management approach for PV includes control of vascular risk factors, therapeutic phlebotomy, and low-dose aspirin in all patients 2.
• Patients at higher risk of thrombosis (aged over 60 years and/or with a history of thrombosis) are offered cytoreductive agents, with hydroxyurea or interferons as preferred first-line agents 2.
• Ruxolitinib, a JAK1 and JAK2 inhibitor, is currently approved for the treatment of patients who are resistant to, or intolerant of, hydroxyurea 2, 3.
• The management of PV still largely depends on estimation of thromboembolic risk and trying to decrease the risk with or without cytoreductive medications 3.

Other Considerations

• Other causes of elevated HGB, HCT, and RBC count should be ruled out, such as dehydration, smoking, or high-altitude living 2, 3.
• There is limited evidence to suggest that burn injury can cause acute red blood cell damage and destruction, leading to changes in red blood cell count 4. However, this is not directly relevant to the management approach for a 26-year-old with elevated HGB, HCT, and RBC count.