Polycythemia vera is a chronic myeloproliferative disorder characterized by increased red blood cell mass, leukocytosis, thrombocytosis, and elevated hemoglobin levels. It typically occurs in middle-aged adults and can cause a variety of symptoms including fullness, headaches, and pruritus. Treatment may involve phlebotomy to reduce red blood cell counts, myelosuppressive drugs to control the disease, and medications to reduce complications like uric acid levels and thrombosis. Nursing care focuses on monitoring for complications, educating patients, and ensuring safety during treatments.

1. POLYCYTHEMIA VERA
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ALBERT BLESSON V MSC (N).,MBA(HM).,

2. POLYCYTHEMIA VERA
Polycythemia vera (also known as primary polycythemia, erythema, polycythemia rubra vera,
splenomegalic polycythemia, and Vaquez–Osler disease) is a chronic myeloproliferative disorder. It is
characterized by increased RBC mass, leukocytosis, thrombocytosis, and an increased hemoglobin
level, with a normal or decreased plasma volume. It usually occurs between ages 40 and 60, most
commonly among men of Jewish ancestry. It seldom affects children or Blacks and does not appear to
be familial.
The onset of polycythemia is gradual, and the disease runs a chronic but slowly progressive
course. The prognosis depends on age at diagnosis, treatment used, and complications. Mortality is
high if polycythemia is untreated or is associated with leukemia or myeloid metaplasia.
For patients with polycythemia vera, uncontrolled and rapid cellular reproduction and
maturation cause proliferation or hyperplasia of all bone marrow cells (panmyelosis). The cause of such
uncontrolled cellular activity is unknown, but it is probably the result of a multipotential stem cell
defect.

4. Signs and Symptoms
• In early stages, possibly no signs or symptoms
• Fullness in the head
• Rushing in the ears
• Tinnitus
• Headache
• Dizziness
• Vertigo
• Epistaxis
• Night sweats
• Epigastric and joint pain
• Scotomas, double vision, and blurred vision
• Decreased urine output
• Pruritus (which worsens after bathing and may be disabling)
• A sense of abdominal fullness
• Pleuritic chest pain or left-upper-quadrant

5. Treatment
• Phlebotomy therapy to promptly reduce RBC mass (Typically, 350 to 500 mL of blood can be
removed every other day until the patient’s hematocrit is reduced to the low-normal range.
The goal is to keep the hematocrit below 45%.)
• Myelosuppressive therapy (for patients with severe symptoms, such as extreme thrombocytosis,
a rapidly enlarging spleen, or hypermetabolism)
• Radioactive phosphorus (32P) or a chemotherapeutic agent, such as melphalan, busulfan, or
chlorambucil, to control the disease (in most cases)
• Pheresis technology that allows removal of RBCs, WBCs, and platelets individually or collectively
(cellular components given to blood banks)
• Administration of cyproheptadine and allopurinol to reduce the serum uric acid level
• Administration of hydroxyurea is used to decrease the incidence of thrombotic occurrences

6. Nursing Considerations
• Keep the patient active and ambulatory to prevent thrombosis. If bed rest is
necessary, prescribe a daily program of both active and passive range-of-motion
exercises.
• Watch for complications, such as hypervolemia, thrombocytosis, signs and
symptoms of impending stroke (decreased sensation, numbness, transitory paralysis,
fleeting blindness, headache, and epistaxis), hypertension, and heart failure (caused
by prolonged hyperviscosity).
• Regularly examine the patient for bleeding.
• To compensate for increased uric acid production, give the patient additional fluids
(at least 3,000 mL/day), administer allopurinol as ordered, and alkalinize the urine to
prevent uric acid calculus formation.

7. • If the patient has symptomatic splenomegaly, suggest or provide small, frequent meals followed by a
rest period to prevent nausea and vomiting.
• If the patient has pruritus, give medications as ordered, and provide distractions to help him cope.
Bathing in tepid to cool water will decrease pruritus.
• Report acute abdominal pain immediately; it may signal splenic infarction, renal calculus formation,
or abdominal thrombosis.
• Before phlebotomy, check the patient’s blood pressure, pulse rate, and respiratory rate. Stay alert
for tachycardia, clamminess, and complaints of vertigo. If any of these occurs, the procedure should
be stopped.
• Immediately after phlebotomy, check the patient’s blood pressure and pulse rate. Have the patient
sit up for about 5 minutes before allowing him to walk; this prevents vasovagal attack and
orthostatic hypotension. Administer 24 oz (710 mL) of juice or water, or IV fluids if indicated, to
replenish fluid volume.

9. • Advise the patient to avoid dehydration.
• Determine what the patient knows about the disease, especially if he has been diagnosed for
some time. As necessary, reinforce the practitioner’s explanation of the disease, its signs and
symptoms, and prescribed treatment.T
• ell the patient to remain as active as possible to help maintain his self-esteem.
• Instruct the patient to use an electric razor to prevent accidental cuts and to keep his
environment free from clutter to minimize falls and contusions.
• Advise the patient to avoid high altitudes, which may exacerbate polycythemia.
• Advise the patient to avoid dehydration.
• If the patient develops thrombocytopenia, tell him the most common bleeding sites (such as
the nose, gingiva, and skin) so he can check for bleeding. Advise him to promptly report any
abnormal bleeding.
• If the patient requires phlebotomy, describe the procedure and explain that it will relieve
distressing symptoms. Tell the patient to watch for and report any signs or symptoms of iron

10. • If the patient requires myelosuppressive therapy, tell him about adverse effects (nausea,
vomiting, and susceptibility to infection) that may follow administration of an alkylating
agent. As appropriate, mention that alopecia may follow use of busulfan, cyclophosphamide,
or uracil mustard and that sterile hemorrhagic cystitis may follow use of cyclophosphamide.
(Ensuring that the patient takes in plenty of fluids can prevent this adverse effect.)
• If an outpatient develops leukopenia, reinforce instructions about preventing infection. Warn
the patient that his resistance to infection is low; advise him to avoid crowds, and make sure
he knows the symptoms of infection.
• If the patient requires treatment with 32P, explain the procedure. Tell him that he may require
repeated phlebotomies until 32P takes effect.
• Refer the patient to the social service department and local home health care agencies, as
appropriate.

11. REFERENCE
LippincottVISUAL NURSING
A Guide to Diseases, Skills, and Treatments
Third Edition

12. THANK YOU