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Polycythemia Vera

Polycythemia Vera (PV)    

  • Is one of a related group of blood cancers known as “myeloproliferative neoplasms” (MPNs). Too many red blood cells are made in the bone marrow and, in many cases, the numbers of white blood cells and platelets are also elevated. 
  • With careful medical supervision, PV can usually be managed effectively for many years. For some PV patients, however, the PV may progress to a more aggressive blood disease, such as myelofibrosis, acute myeloid leukemia or myelodysplastic syndromes.

What You Should Know

  • Hematologists and oncologists are specialists who treat people who have PV or other types of blood cancer.
  • PV is a chronic disease; it is not curable, but it usually can be managed effectively for very long periods.
  • Medical supervision of individuals with PV is important to prevent or treat complications.

What You Should Do

  • Talk with your doctor about your diagnostic tests and what the results mean
  • Talk with your doctor about all your treatment options, side effects, and the results you can expect from treatment.
  • Ask your doctor whether a clinical trial is a good treatment option for you.

How Does PV Develop?

The DNA (genetic material) of a developing stem cell in the bone marrow is damaged. This is called an “acquired mutation.”

  • Stem cells form blood cells (white cells, red cells and platelets).

The mutated cell leads to uncontrolled blood cell production, especially red cells.

  •  In many cases, the numbers of white blood cells and platelets are also elevated.

 With extra blood cells in the bloodstream, abnormal clots and bleeding are more likely to occur.

  • This can increase the risk of
    • Heart attack, stroke (a clot that blocks blood flow to part of the brain which can damage brain tissue).
    • Pulmonary embolism (blockage of an artery in the lungs).
    • Accumulation of extra blood cells  in the spleen, causing the spleen to swell. 

Source: Myeloproliferative Neoplasms. Reviewed by John Mascarenhas, MD.