From the Guidelines
Elevated hemoglobin levels should be treated with therapeutic phlebotomy only if the hemoglobin is greater than 20 g/dL and the hematocrit is greater than 65%, associated with symptoms of hyperviscosity, such as headache and increasing fatigue, in the absence of dehydration or anemia, as recommended by the ACC/AHA 2008 guidelines 1.
Evaluation and Management
Elevated hemoglobin, also known as polycythemia, requires evaluation by a healthcare provider to determine the underlying cause. The management of elevated hemoglobin depends on the underlying cause, and treatment should focus on addressing the root cause of the condition.
Therapeutic Phlebotomy
Therapeutic phlebotomy is recommended for patients with hemoglobin levels greater than 20 g/dL and hematocrit greater than 65%, associated with symptoms of hyperviscosity, such as headache and increasing fatigue, in the absence of dehydration or anemia 1. However, repeated routine phlebotomies are not recommended due to the risk of iron depletion, decreased oxygen-carrying capacity, and stroke 1.
Iron Deficiency
Iron deficiency is a concern in patients with erythrocytosis, and treatment should be cautious to avoid rapid increases in red cell mass. Oral iron administration should be monitored closely, and serum ferritin or transferrin saturation should be checked to confirm the diagnosis of iron deficiency 1.
Lifestyle Modifications
Lifestyle modifications, such as increasing fluid intake to prevent dehydration and avoiding activities that worsen dehydration, are essential for managing elevated hemoglobin levels. Regular monitoring of complete blood counts is necessary to track hemoglobin levels and adjust treatment accordingly.
Complications
Elevated hemoglobin increases blood viscosity and can lead to clotting complications, such as stroke or heart attack. Prompt evaluation and treatment are crucial to prevent serious complications.
From the Research
Elevated Hemoglobin and Polycythemia Vera
Elevated hemoglobin levels are a key characteristic of polycythemia vera (PV), a myeloproliferative neoplasm that affects approximately 65,000 people in the US, with an annual incidence of 0.5 to 4.0 cases per 100,000 persons 2. The diagnosis of PV is based on the presence of erythrocytosis, which is defined as a hemoglobin level greater than 16.5 mg/dL in men or greater than 16.0 mg/dL in women 2.
Causes and Risk Factors
The exact cause of elevated hemoglobin levels in PV is not fully understood, but it is thought to be related to a mutation in the JAK2 gene, which is present in more than 95% of patients with PV 2, 3. Other risk factors for PV include age, with the risk of thrombosis increasing with age, and a history of thrombosis 2, 4.
Symptoms and Complications
Patients with PV may experience a range of symptoms, including:
- Pruritus (33%) 2
- Erythromelalgia (5.3%) 2
- Transient visual changes (14%) 2
- Splenomegaly (36%) with abdominal discomfort 2
- Thrombosis, which can occur in unusual sites, such as splanchnic veins 2
- Bleeding, especially in patients with acquired von Willebrand disease 2
Treatment and Management
The treatment of PV typically involves:
- Therapeutic phlebotomy to reduce hematocrit levels to less than 45% 2, 4
- Low-dose aspirin to reduce the risk of thrombosis 2, 4
- Cytoreductive therapy, such as hydroxyurea or interferon, to reduce the risk of thrombosis and decrease symptoms 2, 5, 4
- Ruxolitinib, a Janus kinase inhibitor, to alleviate pruritus and decrease splenomegaly in patients who are intolerant of or resistant to hydroxyurea 2, 3
Prognosis and Outcome
The prognosis for patients with PV is generally good, with a median survival of 14.1 to 27.6 years 2. However, patients with PV are at risk of developing myelofibrosis and acute myeloid leukemia, which can significantly impact their quality of life and survival 2, 3. Regular monitoring and follow-up are essential to manage the disease and prevent complications.