What is the next step in managing a young patient with erythrocytosis, hyperhemoglobinemia, and hyperhematocrit who is taking testosterone?

From the Guidelines

The next step in managing a young patient with erythrocytosis, hyperhemoglobinemia, and hyperhematocrit who is taking testosterone is to temporarily discontinue testosterone therapy and perform therapeutic phlebotomy if clinically indicated. The patient's laboratory results show a red blood cell count of 6.19 million/μL, hemoglobin of 18.1 g/dL, and hematocrit of 57.0%, which are all above the normal range. According to the study by 1, testosterone therapy can increase the risk of erythrocytosis, and it is recommended to monitor hematocrit levels and adjust the testosterone dose or formulation as needed. The patient should have 350-500 mL of blood removed, which can be repeated weekly until the hematocrit falls below 50%. Simultaneously, the testosterone dose should be reassessed and potentially reduced when therapy is resumed. Laboratory monitoring should include complete blood count, liver function tests, and testosterone levels every 3-6 months. The patient should be advised to maintain adequate hydration and avoid smoking, as dehydration and smoking can further increase hematocrit levels. These interventions are necessary because testosterone therapy can stimulate erythropoiesis through increased erythropoietin production and direct effects on bone marrow, leading to secondary polycythemia, as noted in the study by 1. Elevated hematocrit increases blood viscosity and the risk of thrombotic events such as stroke, myocardial infarction, and venous thromboembolism. Once the hematocrit is controlled, testosterone therapy can often be resumed at a lower dose or with a different formulation that may have less impact on erythropoiesis, such as transdermal preparations instead of injectable forms, as suggested by the study by 1. Some key points to consider in the management of this patient include:

• Monitoring hematocrit levels regularly to prevent erythrocytosis
• Adjusting the testosterone dose or formulation as needed to minimize the risk of erythrocytosis
• Performing therapeutic phlebotomy if clinically indicated to reduce hematocrit levels
• Advising the patient to maintain adequate hydration and avoid smoking to reduce the risk of thrombotic events.

From the FDA Drug Label

1. 3 Polycythemia

Increases in hematocrit, reflective of increases in red blood cell mass, may require lowering or discontinuation of testosterone. Check hematocrit prior to initiating treatment. It would also be appropriate to re-evaluate the hematocrit 3 to 6 months after starting treatment, and then annually If hematocrit becomes elevated, stop therapy until hematocrit decreases to an acceptable concentration.

The patient has erythrocytosis, hyperhemoglobinemia, and hyperhematocrit, with a hematocrit of 57.0%, which is above the normal range. Given the patient is taking testosterone, the next step would be to stop therapy until the hematocrit decreases to an acceptable concentration, as the elevated hematocrit may increase the risk of thromboembolic events 2.

From the Research

Next Steps in Managing Erythrocytosis, Hyperhemoglobinemia, and Hyperhematocrit

The patient's lab results indicate erythrocytosis (red blood cell count 6.19 million/μL), hyperhemoglobinemia (hemoglobin 18.1 g/dL), and hyperhematocrit (hematocrit 57.0%). These findings, in combination with the patient's use of testosterone, require careful consideration of potential underlying causes and appropriate management strategies.

Potential Underlying Causes

• Polycythemia vera (PV), a myeloproliferative neoplasm characterized by increased red blood cell mass, should be considered as a potential underlying cause 3, 4, 5.
• Secondary causes of erythrocytosis, such as heavy smoking, chronic pulmonary disease, or renal disease, must be excluded 4, 5.
• The use of testosterone may also contribute to the development of erythrocytosis 5.

Management Strategies

• Therapeutic phlebotomy to maintain a hematocrit below 45% is recommended for patients with PV 3, 4, 5.
• Low-dose aspirin may be considered to reduce the risk of thrombotic events, if there are no contraindications 3, 5.
• Cytoreductive therapy with hydroxyurea or interferon may be necessary for patients at high risk of thrombosis or with persistent symptoms 3, 6, 7.
• Regular monitoring of blood counts and clinical symptoms is essential to adjust treatment as needed 6.

Key Considerations

• The patient's young age and use of testosterone should be taken into account when developing a management plan.
• A thorough diagnostic evaluation, including bone marrow morphology and molecular testing for JAK2 mutations, may be necessary to confirm a diagnosis of PV 3, 5.
• Collaboration with a hematologist is recommended to ensure optimal management of the patient's condition 4.