3.9 Polycythemia

Although anemia and sickle cell disease cause a decrease in red blood cells, polycythemia is a medical condition with an excessive number of red blood cells. In addition to excessive numbers of these cells, they are also abnormal, resulting in decreased oxygen-carrying capacity, poor gas exchange, increased risk of infection, and bleeding problems. Additionally, the client’s blood also becomes hyperviscous (thicker than normal), which causes the blood to move more slowly in blood vessels and increases the risk for clotting in arteries and veins. This clotting can cause deep vein thrombosis (DVTs), or the clots can move to the heart or brain, causing a heart attack or stroke.^[1]

There are two major types of polycythemia, primary and secondary:

• Primary polycythemia, also known as polycythemia vera (PV), is caused by a type of bone marrow cancer that causes it to produce too many red blood cells, as well as white blood cells and platelets. There appears to be a genetic component to PV, but it can also be caused by exposure to radiation or chemicals like benzene.^[2],[3],[4]
• Secondary polycythemia refers to too many red blood cells that are produced as a result of conditions that cause low oxygen levels in the body, such as chronic obstructive pulmonary disease.

Assessment

Physical Examination

Clients with polycythemia vera may experience a variety of symptoms. See Table 3.9 for common symptoms of polycythemia vera by body system.

Table 3.9. Symptoms of Polycythemia Vera Across Body Systems^[5],[6],[7]

Diagnostic Testing

Diagnosing polycythemia vera begins with the CBC. In polycythemia vera, the CBC reflects elevated red blood cells, white blood cells, and platelets, as well as elevated hematocrit and hemoglobin levels. Review normal reference ranges for common diagnostic tests in “Appendix A – Normal Reference Ranges.”

To confirm a polycythemia vera diagnosis, additional testing is needed to determine the presence of the JAK2V617F mutation, which is present in about 95% of PV cases. The JAK2V617F mutation is a mutation that occurs on Chromosome 9 where the JAK2 gene helps to regulate and produce blood cells. Additionally, a bone marrow aspiration and biopsy may be performed.^{[9],[10],[11]}

Nursing Diagnosis

Nursing diagnoses for clients with polycythemia vera are developed based on the client’s assessment data, medical history, and specific needs.

Common nursing diagnoses for polycythemia vera include the following:

• Risk for Thrombosis
• Impaired Gas Exchange
• Activity Intolerance
• Readiness for Enhanced Knowledge
• Impaired Coping

Outcome Identification

Outcome identification includes setting short- and long-term goals and creating specific expected outcome statements for nursing diagnoses identified for the client. Expected outcomes are statements of measurable action for the client within a specific time frame that are responsive to nursing interventions.

Sample expected outcomes related to common nursing diagnoses for clients diagnosed with polycythemia vera are as follows:

• The client will remain free from blood clots.
• The client will remain free from excessive bleeding.
• The client will remain free from infection.

Interventions

Medical Interventions

Medical interventions for polycythemia vera are targeted at reducing the excessive production of red blood cells and preventing complications associated with increased risk of blood clotting.

Common medical interventions and treatments used to manage polycythemia vera include the following:

• Therapeutic Phlebotomy with Plasmapheresis: The primary treatment for polycythemia vera is routinely scheduled phlebotomy with apheresis. Plasmapheresis is the withdrawal of whole blood and the removal of red blood cells and then reinfusion of the plasma back into the client. This therapy reduces the red blood cell count, as well as blood viscosity, and lengthens the client’s life span.
• Low-Dose Aspirin: Low-dose aspirin may be prescribed to reduce the risk of blood clots by inhibiting platelet aggregation.
• Cytoreductive Therapy: In symptomatic clients, medications such as hydroxyurea may be used to suppress the bone marrow’s overproduction of blood cells.
• JAK2 Inhibitors: Therapies targeting the JAK2 pathway (i.e., ruxolitinib) may be used to control the abnormal cell growth associated with the JAK2V617F mutation.
• Anticoagulation: In certain cases, anticoagulant medications (i.e., warfarin or heparin) may be prescribed to prevent blood clots in clients with a history of thrombosis.^[12],[13]

Nursing Interventions

Nursing interventions for clients with polycythemia vera focus on preventing thromboses, ensuring adequate oxygenation, assisting with energy management, promoting hydration, managing medications, and providing health teaching to support the client’s well-being.

• Preventing Thrombosis: Nurses help prevent thrombosis formation by promoting adequate hydration, encouraging mobility, and administering anticoagulation therapy as prescribed.
• Ensuring Adequate Oxygenation: Nurses monitor the client’s oxygen saturation levels and respiratory status and administer supplemental oxygen as needed to maintain adequate oxygenation.
• Assisting With Energy Management: Nurses implement interventions related to energy management, as discussed in the “Anemia” section. Clients are encouraged to balance activity and rest periods to prevent excessive fatigue.
• Promoting Hydration: Nurses encourage adequate fluid intake to prevent dehydration, reduce blood viscosity, and maintain perfusion of tissues and organs.
• Managing Medication: Nurses safely administer prescribed medications, such as cytoreductive agents or antiplatelet drugs.
• Participating in Health Teaching and Health Promotion: Nurses educate clients about the disease process and potential complications, such as bleeding and thrombosis. The importance of prescribed, routine phlebotomy is emphasized to reduce blood viscosity and prevent potential complications related to thrombosis.^[14],[15]

Evaluation

During the evaluation stage, nurses determine the effectiveness of nursing interventions for a specific client. The previously identified expected outcomes are reviewed to determine if they were met, partially met, or not met by the time frames indicated. If outcomes are not met or only partially met by the time frame indicated, the nursing care plan is revised. Evaluation should occur every time the nurse implements interventions with a client, reviews updated laboratory or diagnostic test results, or discusses the care plan with other members of the interprofessional team.