Dr. M.J. Bazos, MD. Patient Handout

About Your Diagnosis
Polycythemia vera is a malignant process that involves the stem cells (the cells that can produce white blood cells, red blood cells, or platelets) in the bone marrow (soft pink pulp in the long bones, ribs, and vertebrae where blood cells are formed). Polycythemia vera causes an increase in the number of red blood cells, white blood cells, and platelets and an enlarged spleen. The cause of this disease is unknown. Polycythemia vera is an uncommon disease. Most patients are between 50 and 60 years of age. One of 100,000 persons has this disorder. There are families in which several family members have either polycythemia vera or another related problem, but these families are rare. This suggests that polycythemia vera can be transferred from parents to offspring genetically. Polycythemia vera can be cured only by means of bone marrow transplantation. It is usually detected when an elevated red blood cell count, white blood cell count, and platelet count are found on a blood test. Patients usually report fatigue, headache, drowsiness, forgetfulness, and vertigo. Itching after a bath is a common finding. Patients also may have nose bleeds or gastrointestinal bleeding. The eyes and face look red. There is an increased incidence of peptic ulcer disease among persons with polycythemia vera. An enlarged spleen is present in at least three fourths of patients with polycythemia vera. It gives a feeling of fullness in the abdomen. Enlargement of the liver is present among 40% of patients. Disturbances of vision, such as temporary blindness, double vision, specks, and bright points in front of the field of vision, are common.

Living With Your Diagnosis
Total blood volume is increased, which increases the thickness of the blood and gives rise to symptoms such as headaches, vertigo, dizziness, a sensation
of fullness in the head, and tingling and numbness in the fingers and toes. Patients report shortness of breath on exertion. The skin of the face and neck redden. Blood clots can form in the veins, causing problems such as strokes and blood clots in the lungs and legs.

Usually no treatment is needed by patients who feel well and have no symptoms. Phlebotomy (removal of blood by means of puncturing a vein) is the safest therapy for polycythemia vera. It can control the symptoms for most patients. One pint of blood is removed at periodic intervals to keep the hemoglobin and hematocrit within normal range. For older patients or patients with other medical problems, smaller volumes of blood are removed at one time, or other treatment possibilities are used. Other options for symptomatic polycythemia vera are chemotherapy with agents such as hydroxyurea, chlorambucil, busulfan, and cyclophosphamide. Radiation therapy to the spleen and bones is another treatment option. Splenectomy (surgical removal of the spleen) can be considered in later stages of the disease if enlargement of the spleen causes fullness in the abdomen, discomfort, or worsening of anemia and thrombocytopenia. Antihistamines such as diphenhydramine (eg, Benadryl) and hydroxyzine (eg, Atarax) are used for itching. H2 blockers such as cimetidine (eg, Tagamet) and famotidine (eg, Pepcid) are used for peptic ulcer disease. Patients who are treated with phlebotomy are at increased risk for blood clot formation, because platelet production in the bone marrow increases when blood is removed. There are several other reasons for this problem, which should be discussed with a physician. Chemotherapy destroys normal blood cells in addition to malignant cells and therefore can lower white blood cell count. This increases risk for infection and lowers red blood cell and platelet counts, causing anemia and risk for bleeding. Chemotherapeutic agents can change the genetic structure of the cells, which can cause other cancers. Chemotherapy also causes thinning of the hair during therapy, but the hair grows back after treatment is stopped. Chemotherapy also causes nausea,
vomiting, and diarrhea.

The DOs
• Undergo phlebotomy at periodic intervals to keep blood counts at the upper limits of normal.
• Participate in chemotherapy as recommended by your physician.
• Take antihistamines for itching as directed by your physician.
• Take H2 blockers for peptic ulcer disease as directed by your physician.
• Undergo routine clinical follow-up care with your physician.

The DON’Ts
• Do not use medications more frequently than recommended. Certain medications such as aspirin and dipyridamole increase risk for bleeding.
• Avoid uncooked vegetables, fresh fruits, and milk products. These products harbor bacteria, which do not cause problems for healthy persons but can cause severe infections if you have neutropenia.
• Avoid strenuous activity if your blood counts are low during chemotherapy or you have an enlarged spleen or anemia, because of risk for trauma to the spleen and decreased exercise tolerance.

When to Call Your Doctor
• If you have excessive fatigue, shortness of breath, severe headaches, bleeding from any orifice, severe abdominal pain, or sudden swelling of an arm or leg.

American Cancer Society: URL: http://www.cancer