Myeloproliferative neoplasms (MPN) are a related group of blood cancers. In these disorders, the bone marrow cells that produce blood cells develop and function abnormally. The three main types of MPN are polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). In addition to developing on its own, myelofibrosis may also develop as a result of ET or PMF.
PV and ET each result from the overproduction of certain types of blood cells. PMF involves scarring of the bone marrow and changes in blood cell production. The prognosis of these conditions varies greatly across people and by type of MPN. PV and ET both tend to be fairly slow-growing and may have only a modest effect on life expectancy. PMF tends to progress more rapidly, but the course of the disease can vary. Recently treatment options have become available and treatment has been shown to provide improvement of symptoms.1,2 In rare cases, MPN can transform into a fast-growing type of blood cancer known as acute myeloid leukemia.
Myeloproliferative neoplasms are most common in older adults. Out of every 100,000 people in the United States, an estimated 44 to 57 people have PV, and a similar number have ET. Myelofibrosis is less common, affecting 4 to 6 people per 100,000.3
An important advance in our understanding of MPN is the identification of certain gene mutations that contribute to these conditions.1 In the case of myelofibrosis, this discovery has contributed to the development of new drugs to treat the condition.4
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Polycythemia vera: results from the overproduction of red blood cells. White blood cells and platelets may also be overproduced.
Essential thrombocythemia: results from the overproduction of platelets. Red blood cells and white blood cells may also be overproduced.
Myelofibrosis: results from the formation of scar tissue in the bone marrow, which interferes with the normal production of blood cells (red blood cells, white blood cells, and platelets).
References
1 annucchi AM, Guglielmelli P, Tefferi A. Advances in understanding and management of myeloproliferative neoplasms. CA Cancer J Clin. 2009;59:171-191.
2 Hultcrantz M, Kristinsson SY, Andersson TM, et al. Patterns of survival among patients with myeloproliferative neoplasms diagnosed in Sweden from 1973 to 2008: a population-based study. Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 2012;30:2995-3001.
3 Mehta J, Wang H, Iqbal SU, Mesa R. Epidemiology of myeloproliferative neoplasms in the United States. Leukemia & lymphoma. 2013.
4 Keohane C, Radia DH, Harrison CN. Treatment and management of myelofibrosis in the era of JAK inhibitors. Biologics : targets & therapy. 2013;7:189-198.
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