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Polycythemia Vera

more about Polycythemia Vera

Primary PV, PV rubra, Vasquez-Osler Disease, Splenomegalic Polycythemia, or myeloproliferative disorder

  • A condition in which there is excessive production of red blood cells (erythrocytosis or increased RBC), white blood cells (leukocytosis WBC), and platelets (thrombocytosis or increased platelets) in the bone marrow.
  • Blood cells with different functions are normally manufactured in the material inside some of the bones (e.g., long bones) called the marrow.  The blood volume and thickness increases abnormally in Pv., which can lead to numerous problems for the body.
  • White blood cells (fight infections) and platelets (stop bleeding) do not function normally, and cause further problems for the patient with Pv.

  • Unknown

  • No symptoms at early stages
  • Headache
  • Fullness in the upper abdomen (enlarged liver and spleen)
  • Itching or pruritus
  • Nose bleeds or Epistaxis
  • Blurred Vision
  • Tinnitus or Ringing in the Ears
  • Bruising easily or without reason
  • Bone pain -- ribs and sternum (bone in the center of chest)
  • Plethora -- reddish complexion of the face, hands, and feet
  • May have black tarry stool (melena) due to peptic (stomach) ulcers
  • Vomiting blood
  • Weight loss
  • Sweating
  • Fatigue
  • Shortness of breath
  • Infections
  • Bleeding tendencies such as Blood in the Urine (Hematuria)
  • Increased blood viscosity or thick blood -- increases the risk for blood clot formation in the blood vessels (arteries) of the legs (phlebitis), lungs (pulmonary embolus), portal vein of the liver, brain (stroke), or heart (heart attack).
  • Some cases progress to Leukemia, another type of blood malignancy (cancer).

  • Your doctor may consult with a hematologist (blood doctor).
  • History:
    1. Symptoms
    2. Family history
    3. Medications
    4. Allergies
    5. Illnesses
    6. Surgeries
    7. Habits
  • Medical exam:
    1. Plethora
    2. Bruises
    3. Weight loss
    4. Enlarged spleen
    5. Enlarged liver
    6. Signs of Heart Failure (swollen ankles, shortness of breath), heart attack, stroke (slurred speech, paralysis, etc.) may be present.
  • Tests:
    1. Blood analysis -- shows increased RBC mass (hematocrit), platelets, WBC, B12 vitamin, and leukocyte Alkaline phosphatase.
    2. Blood samples may also show elevated levels of ESR, Cholestrol, and Uric acid.
    3. Ultrasound and CAT scans will show an enlarged spleen in 75% of those with Pv.
    4. Oxygen levels of the blood are often adequate.
    5. Bone marrow aspiration or biopsy are common techniques used to provide information about the marrow and its contents.
    6. Biopsy is done by removing a sample of the marrow using a special corkscrew-like device (under anesthesia) but is extremely helpful in diagnosing Pv.

  • Males > females
  • Family history
  • Rare under 40
  • Jewish ancestry

  • Aimed at prevention of clot formation (thrombosis) or bleeding (hemorrhage) -- achieved by reducing high blood viscosity (thickness).
  • Phlebotomy (i.e., drawing blood) on a weekly basis, or until the desired affect is achieved.
  • Chemotherapy drugs (e.g., Hydroxyurea) or radiation therapy to suppress the bone marrow.
  • Allopurinol may be used to reduce high Uric acid levels associated with gout.
  • Antacids or H2 blockers may help with stomach problems.
  • Medications are available for itching and pain.
  • Iron supplements are not needed unless recommended by the doctor.
  • Drink plenty of water.
  • Interferon and other forms of therapy are emerging.

  • Consult with your physician.  Do not give up hope!  With treatment, some live up to 10 years or longer.  You can get more information from National Cancer Institute by calling 1-800-4-CANCER.

  • Hemoglobinopathies
  • Cystic renal disease
  • Erythropoieitin secreting tumors
  • Other Myeloproliferative Disorders
  • Tobacco use -- carboxyhemoglobin
  • Use of diuretics

more about Polycythemia Vera

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