POLYCYTHEMIA VERA

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POLYCYTHEMIA VERA
A myeloproliferative disorder that results in an overproduction of blood cells and a thickening of blood. The hallmarks of polycythemia vera include excessive production of red blood cells, white blood cells, and platelets. The excess of cells present in the blood causes problems with the flow of blood through vessels, especially the smaller ones. There will be an increase in peripheral vascular resistance causing increased pressure, and vascular stasis in the smaller vessels, potentially causing thrombosis or tissue hypoxia. Organ damage may result because of these changes.

PROGNOSIS
After diagnosis of polycythemia vera, the average survival time is 10 to 15 years with appropriate treatment, less than 2 years without treatment. Some patients may go on to develop acute leukemia. Complications usually arise from thrombosis or tissue hypoxia.

SIGNS AND SYMPTOMS
• Facial skin and mucous membranes dark and flushed (plethora)
• Hypertension due to increased peripheral vascular resistance and thickening of the blood
• Itching worse after warm shower due to histamine release from increased basophils within dilated vessels
• Headache and difficulty concentrating
• Vision blurred, tinnitus (ringing in ears), and hearing changes
• Thrombosis due to vascular stasis
• Spleen enlargement (splenomegaly)
• Tissue hypoxia and possible infarction of heart, spleen, kidneys, and brain due to thrombosis

INTERPRETING TEST RESULTS
• Increased RBC count.
• Increased hemoglobin.
• Increased hematocrit level.
• Increased WBC count.
• Increased basophils.
• Increased eosinophils.
• Increased platelet count.
• Increased uric acid level.
• Increased potassium.
• Increased vitamin B12 level.
• Bone marrow panhyperplasia; iron stores absent.

TREATMENT
Treatment is aimed at maintaining blood flow to the smaller vessels and diminishing the amount of excess blood cells being made by the bone marrow.
• Periodic scheduled phlebotomy—the removal of 500 ml of blood—to reduce the hematocrit level to below 45; may be done weekly. • Adequate hydration.
• Anticoagulants such as aspirin.
• Administer myelosuppressive medication:
• Hydroxyurea
• Anagrelide
• Radioactive phosphorus 32
• Administer medication to lower uric acid level if necessary:
• Allopurinol
• Alkylating agents:
• Melphalan
• Busulfan
• Radiation therapy.
• Antihistamine for pruritis.

NURSING DIAGNOSES
• Ineffective tissue perfusion
• Disturbed sensory perception
• Risk for injury

NURSING INTERVENTION
• Monitor vital signs.
• Monitor for bleeding.
• Monitor for signs of infections.
• Keep the patient mobilized to decrease chance of clot formation.
• Increase fluid intake.
• Explain to the patient:
• Maintain activity.
• Use electric razor, use soft toothbrush, and avoid flossing to decrease chances of bleeding.
• Avoid activities that could cause injury.

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