How to manage a patient with polycythemia?

From the Guidelines

The primary goal in managing polycythemia vera (PV) is to maintain hematocrit below 45% through therapeutic phlebotomy and use low-dose aspirin to reduce thrombotic risk, as recommended by the most recent guidelines 1. To achieve this, the following steps should be taken:

• Therapeutic phlebotomy should be performed to control hematocrit levels, typically removing 450-500 mL of blood every 2-3 months as needed.
• Low-dose aspirin (81-100 mg daily) should be used to reduce the risk of thrombosis, as it has been shown to be effective in reducing thrombotic events without significantly increasing the risk of major bleeding 1.
• Cytoreductive therapy should be considered for high-risk patients, including those with a history of thrombosis or age greater than 60 years, with hydroxyurea being a first-line treatment option, starting at 500-1000 mg daily and adjusted to maintain a platelet count below 400,000/μL.
• Regular monitoring should include complete blood counts every 3-6 months and assessment for splenomegaly, thrombotic events, and disease progression.
• Patients should also be advised to modify their cardiovascular risk factors by stopping smoking, maintaining adequate hydration, avoiding extreme heat exposure, and exercising with caution. The use of cytoreductive drugs in otherwise low-risk patients who have well-controlled cardiovascular risk factors is not indicated, and all patients should be managed aggressively for their generic cardiovascular risk factors 1.

From the Research

Patient Management

To manage a patient with polycythemia, the following steps can be taken:

• Determine the cause of the polycythemia, whether primary (polycythemia vera) or secondary, as the treatment approach may differ 2, 3, 4
• Evaluate the patient's blood cell counts, including white blood cell count, red blood cell count, hemoglobin, and hematocrit levels, to assess the severity of the condition
• Consider the patient's medical history, including any history of thrombosis, and perform a physical examination to check for signs of splenomegaly or other abnormalities 5, 3

Diagnostic Approach

The diagnostic approach for polycythemia involves:

• Checking for causes of secondary erythrocytosis, such as renal disease or chronic pulmonary disease 2, 4
• Measuring serum erythropoietin levels to help distinguish between primary and secondary polycythemia 2, 4
• Performing a bone marrow biopsy to confirm the diagnosis and assess the bone marrow histomorphology 5, 6
• Evaluating the patient's JAK2 mutation status, as this can be present in patients with polycythemia vera 5, 6

Treatment Options

Treatment options for polycythemia vera include:

• Phlebotomy to reduce the red blood cell mass and prevent thrombosis 5, 3, 6
• Cytoreductive therapy using agents such as hydroxyurea or interferon-alpha to control the blood cell counts and prevent complications 5, 3, 6
• Aspirin therapy to reduce the risk of thrombosis 5, 3
• Regular follow-up appointments to monitor the patient's condition and adjust the treatment plan as needed 5, 6