Polycythemia Vera

  1. General information
    1. An increase in both the number of circulating erythrocytes and the concentration of Hgb within the blood
    2. Three forms: polycythemia vera, secondary polycythemia, and relative polycythemia
    3. Classified as a myeloproliferative disorder (bone marrow overgrowth)
    4. Cause unknown, but thought to be a form of malignancy similar to leukemia
    5. Usually develops in middle age, common in Jewish men
    6. Pathophysiology
      1. A pronounced increase in the production of erythrocytes accompanied by an increase in the production of myelocytes (leukocytes within bone marrow) and thrombocytes.
      2. The consequences of this overproduction are an increase in blood viscosity, an increase in total blood volume (2-3 times greater than normal), and severe congestion of all tissues and organs with blood.
  2. Assessment findings
    1. Ruddy complexion and duskiness of mucosa secondary to capillary congestion in the skin and mucous membranes
    2. Hypertension associated with vertigo, headache, and "fullness" in the head secondary to increased blood volume
    3. Symptoms of CHF secondary to overwork of the heart
    4. Thrombus formation: CVA, MI, gangrene of the extremities, DVT, and pulmonary embolism can occur
    5. Bleeding and hemorrhage secondary to congestion and overdistension of capillaries and venules
    6. Hepatomegaly and splenomegaly
    7. Peptic ulcer secondary to increased gastric secretions
    8. Gout secondary to increased uric acid released by nucleoprotein breakdown
    9. Laboratory tests
      1. CBC: increase in all mature cell forms (erythrocytes, leukocytes, and platelets)
      2. Hct: increased
      3. Bone marrow: increase in immature cell forms
      4. Bilirubin (indirect): increase in unconjugated fraction
      5. Liver enzymes may be increased
      6. Uric acid increased
      7. Hematuria and melena possible
  3. Nursing interventions
    1. Monitor for signs and symptoms of bleeding complications.
    2. Force fluids and record I&O.
    3. Prevent development of DVT.
    4. Monitor for signs and symptoms of CHF.
    5. Provide care for the client having a phlebotomy.
    6. Prevent/provide care for bleeding or infection complications.
    7. Administer medications as ordered.
      1. Radioactive phosphorus (32P): reduction of erythrocyte production, produces a remission of 6 months to 2 years
      2. Nitrogen mustard, busulfan (Myleran), chlorambucil, cyclophosphamide to effect myelosuppression
      3. Antigout and peptic ulcer drugs as needed.
    8. Provide client teaching and discharge planning concerning
      1. Decrease in activity tolerance, need to space activity with periods of rest
      2. Phlebotomy regimens: outpatient frequency is determined by hct; importance of long-term therapy
      3. High fluid intake
      4. Avoidance of iron-rich foods to avoid counteracting the therapeutic effects of phlebotomy
      5. Recognition and reporting of bleeding
      6. Need to avoid persons with infections, especially in leukopenic clients.