Polycythemia Vera: An Evidence-Based Examination From a Nursing Perspective
Heather Greenberg MSN, RN, CNS

$7.95 		Critical Care Nursing Quarterly
June 2013 
Volume 36  Number 2
Pages 228 - 232
 
  PDF Version Available!

ABSTRACT
MYELOPROLIFERATIVE disorders describe a group of 3 disorders with similar biologic components. These include polycythemia vera (PV), essential thrombocytopenia, and primary myelofibrosis.1 Each of these disorders is characterized by increased hematopoiesis, overproduction of blood elements, and the potential for conversion to acute leukemia.1 Polycythemia vera is distinguished by excessive red blood cell production.Primary polycythemia, or PV, is not triggered by another illness, but is acquired and is a progressive disorder of bone marrow, growing over a period of 10 to 20 years. Typically, the affected people are unaware of the disease until they develop a complication or are diagnosed because of other unrelated medical problems. Polycythemia vera affects one person in 200 000, usually more men than women, and occurs mostly among people aged 50 to 70 years.2 If the patient undergoes treatment after diagnosis, their life expectancy is about 10 to 15 years; if not treated, they usually only live about 18 months after diagnosis.3 Most patients will die from complications of thrombosis, heart failure, leukemia, or hemorrhage.4 The last phase, or spent phase, of PV occurs in approximately 20% of patients and includes myeloid metaplasia with myelofibrosis, splenomegaly, and cytopenia.5In the late 1960s and early 1970s, a PV study group was formed to develop diagnostic criteria and recommendations for treatment. Several studies performed after the group's recommendations narrowed the cause of PV to a clonal transformation of a single hematopoietic stem cell. In 2001, The World Health Organization further refined the diagnostic criteria using evidence-based information from ongoing studies.2Polycythemia vera is caused by an increased cell growth and decreased cell death of erythroid progenitors. Polycythemia vera can develop from a proliferative phase to a metastatic and then spent or malignant phase that can develop into acute leukemia.4 Polycythemia vera is acquired from

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