Polycythemia Vera Follow-up

Updated: Dec 02, 2016
  • Author: Srikanth Nagalla, MBBS, MS, FACP; Chief Editor: Koyamangalath Krishnan, MD, FRCP, FACP  more...
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Follow-up

Further Outpatient Care

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  • Thrombosis in polycythemia vera (PV) is substantially more frequent in patients treated with phlebotomy alone without myelosuppression. This risk is believed to be related to thrombocytosis, which was not observed in the study. Platelet numbers alone are not likely to be the primary factor responsible for the increased risk of thrombosis; the presence of abnormal platelets is more likely.
  • The initial PVSG study using antiplatelet drugs also used aspirin at 300 mg 3 times a day plus dipyridamole at 75 mg 3 times a day. This showed an increase in the incidence of hemorrhage. Lower doses of aspirin have been suggested to be more effective without increasing bleeding complications, although this has not yet been demonstrated in a prospective randomized trial.
  • A syndrome specific to polycythemia vera (PV) and other MPDs is termed erythromelalgia, and it is associated with an increased risk of thrombosis. The symptoms are burning pain in the feet, hands, and digits, sometimes associated with pallor, erythema, or cyanosis of the distal portions of the extremities. Occasionally, it may progress to frank gangrene. In some instances, this is treated with aspirin (50-300 mg/d) and dipyridamole (75 mg orally 3 times a day).
  • Myelosuppressive therapy plus phlebotomies, with the intent of normalizing the erythrocyte and platelet counts, also decreases or eliminates these symptoms. Proven thrombotic complications warrant the use of long-term anticoagulation with warfarin (see below).
    This blood film at 10,000X magnification shows a g This blood film at 10,000X magnification shows a giant platelet and an eosinophil. Erythrocytes show signs of hypochromia as a result of repeated phlebotomies. Courtesy of U. Woermann, MD, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland.
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Further Inpatient Care

The optimum management of polycythemia vera (PV) remains elusive despite the findings of the Polycythemia Vera Study Group (PVSG). However, certain diseases, such as familial erythrocythemia, secondary polycythemia, and relative polycythemia (a benign condition), should be differentiated, and the exact diagnosis of PV should be established.

General principles in the management of PV include the following:

  • Tailor therapy to suit the clinical needs of the patient; consider the status of the formed elements of the blood, bone marrow, and organomegaly
  • Normalize red blood cell mass with phlebotomy as rapidly as clinically possible (250-500 mL every other day); patients who are elderly or have cardiovascular compromise should be phlebotomized cautiously, and smaller amounts should be removed
  • Suppress myeloproliferative activity with chemotherapy (hydroxyurea) in all patients older than 50 years
  • In general, phosphorus-32 ( 32 P) should be reserved for patients older than 80 years or patients with comorbid conditions in whom life expectancy is less than 5-10 years and the convenience of 32 P dosing outweighs the substantial risks of developing acute leukemia 5-15 years after 32 P administration
  • Patients with thrombotic tendencies or those who develop thrombocytosis following phlebotomy should be treated with marrow suppression; consider anagrelide in younger patients (aged 50-70 y)
  • Maintain blood values at reference range levels by regular examination and treatment
  • Avoid overtreatment and toxicity by careful and judicious use of chemotherapy and radiation; supplemental phlebotomy is preferred over excess marrow suppression
  • Postpone elective surgery until long-term control of the disease is established
  • Women of childbearing age should be treated with phlebotomy only
  • In young males, myelosuppressive therapy can lead to aspermia; thus, evaluate treatment carefully before using any chemotherapy or radiotherapy
  • The PVSG no longer recommends the use of alkylating agents because of the associated increased incidence of leukemia and certain types of cancer
  • Treat hyperuricemia with allopurinol (100-300 mg/d) until remission has been attained; for acute gouty attacks, colchicine or other anti-inflammatory agents are indicated (see Gout and Pseudogout)
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Complications

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  • Bleeding complications (1%) in patients with polycythemia vera (PV) include epistaxis, gum bleeding, ecchymoses, and GI bleeding.
  • Thrombotic complications (1%) include venous thrombosis or thromboembolism and an increased prevalence of stroke and other arterial thromboses.
  • In young patients and during pregnancy, interferon alfa may be useful when HU is unsuitable. Anagrelide may be useful when interferon alfa is not tolerated.
  • In the very elderly patients for whom regular clinic attendance is impractical, 32 P or intermittent busulfan may still be used.
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Prognosis

Polycythemia vera (PV) is a chronic disease, and its natural history of 1.5-3 years of median survival in the absence of therapy has been extended to at least 10-20 years because of new therapeutic tools. According to a study of PV patients from Surveillance, Epidemiology and End Results (SEER) data, although the median survival  is 14 years, mortality is higher than in an age- and sex-matched population. 5 year survival in the overall cohort was 79.5% but patients are at a high risk of second primary malignancies and leukemic transformation, which may compromise long-term survival. [30]

The major causes of morbidity and mortality are as follows:

  • Thrombosis has been reported in 15-60% of patients, depending on the control of their disease. It is the major cause of death in 10-40% of patients. Venous and arterial thromboses have resulted in pulmonary emboli, renal failure from renal vein or artery thrombosis, intestinal ischemia from mesenteric vein thromboses, or peripheral arterial emboli.
  • Hemorrhagic complications occur in 15-35% of patients and lead to death in 6-30% of these patients. Bleeding is usually the consequence of vascular compromise resulting from ischemic changes from thrombosis or hyperviscosity.
  • Peptic ulcer disease is reported to be associated with polycythemia vera (PV) at a 3- to 5-fold higher rate than that of the general population. This has been attributed to increased histamine serum levels.
  • Myelofibrosis and pancytopenia occur in 3-10% of patients, usually late in the disease, which is considered the spent phase of polycythemia vera (PV). In these patients, infections and bleeding complications may be the most serious health threats, and red blood cell transfusions may be required to maintain adequate red blood cell counts and to improve fatigue and other anemia-related symptoms.
  • Acute leukemia or a myelodysplastic syndrome develops in 1.5% of patients treated with phlebotomy alone. The transformation risks increase to 13.5% within 5 years with treatment using chlorambucil and to 10.2% within 6-10 years in patients treated with 32 P. At 15 years, the transformation risk for HU is 5.9%, which, although not statistically significant, is a worrisome trend.
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