Epidemiology of the Myeloproliferative Disorders Polycythemia Vera and Essential Thrombocythemia

ABSTRACT

The annual incidence of polycythemia vera (PV) and essential thrombocythemia (ET) is ~2 and 1.5 per 100,000 inhabitants, respectively, if based on large population surveys and adjusted to a standard population. Survival for ET patients does not differ from that of the general population. However, it appears that PV patients have a significantly higher mortality, at least for patients diagnosed before 1990.

Polycythemia vera - essential thrombocythemia - incidence - mortality - survival

REFERENCES

• 1 Adamson J W, Fialkow P J, Murphy S, Prchal J F, Steinmann L. Polycythemia vera: stem-cell and probable clonal origin of the disease.  N Engl J Med. 1976;  295 913-916
• 2 Fialkow P J, Faguet G B, Jacobson R J, Vaidya K, Murphy S. Evidence that essential thrombocythemia is a clonal disorder with origin in a multipotent stem cell.  Blood. 1981;  58 9 , (abst)
• 3 Cario H. Childhood polycythemias/erythrocytoses: classification, diagnosis, clinical presentation, and treatment.  Ann Hematol. 2005;  84 137-145
• 4 Modan B. An epidemiological study of polycythemia vera.  Blood. 1965;  26 657-667
• 5 Modan B, Kallner H, Zemer D, Yoran C. A note on the increased risk of polycythemia vera in Jews.  Blood. 1971;  37 172-176
• 6 Silverstein M N, Lanier A P. Polycythemia vera, 1935-1969: an epidemiological survey in Rochester, Minnesota.  Mayo Clin Proc. 1971;  46 751-753
• 7 Kurita S. Epidemiological studies of polycythemia vera in Japan.  Nippon Ketsueki Gakkai Zaski. 1974;  37 793-795
• 8 Prochazka A V, Markowe H L. The epidemiology of polcythaemia rubra vera in England and Wales 1968-1992.  Br J Cancer. 1986;  53 59-64
• 9 Berglund S, Zettervall O. Incidence of polycythemia vera in a defined population.  Eur J Haematol. 1992;  48 20-26
• 10 Chaiter Y, Brenner B, Aghai E, Tatarsky I. High incidence of myeloproliferative disorders in Ashkenazi Jews in northern Israel.  Leuk Lymphoma. 1992;  7 251-255
• 11 Ania B J, Suman V J, Sobell J L, Codd M B, Silverstein M N, Melton III L J. Trends in the incidence of polycythemia vera among Olmsted County, Minnesota residents, 1935-1989.  Am J Hematol. 1994;  47 89-93
• 12 Carli P M. Epidemilogy of polycythemia vera in Côte d'Ór (Burgundy).  Nouv Rev Fr Hematol. 1994;  36 147-149
• 13 Ridell B, Carneskog J, Wedel H et al.. Incidence of chronic myeloproliferative disorders in the city of Göteborg, Sweden 1983-1992.  Eur J Haematol. 2000;  65 267-271
• 14 Johansson P, Kutti J, Andreasson B et al.. Trends in the incidence of chronic Philadelphia chromosome negative (Ph-) myeloproliferative disorders in the city of Göteborg, Sweden, during 1983-99.  J Intern Med. 2004;  256 161-165
• 15 Girodon F, Jooste V, Maynadié M, Favre B, Schaeffer C, Carli P M. Incidence of chronic Philadelphia chromosome negative (Ph-) myeloproliferative disorders in the Côte d'Ór area, France, during 1980-99.  J Intern Med. 2005;  258 90-91
• 16 Johansson P, Safai-Kutti S, Rhedin C, Vilén L, Väärt J, Kutti J. On the diagnosis of polycythaemia vera as assessed in the health and medical care in the Västra Götaland region, Sweden.  J Intern Med. 2002;  251 348-354
• 17 Streiff M B, Spivak J L. The diagnosis and management of polycythemia vera in the era since the Polycythemia Vera Study Group: a survey of American Society of Hematology member's practice patterns.  Blood. 2002;  99 1144-1149
• 18 Andréasson B, Löfvenberg E, Westin J. Management of patients with polycythaemia vera: results of a survey among Swedish haematologists.  Eur J Haematol. 2005;  74 489-495
• 19 Passamonti F, Rumi E, Pungolino E et al.. Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia.  Am J Med. 2004;  117 755-761
• 20 Kiladjian J J, Gardin C, Renoux M, Bruno F, Bernard J F. Long-term outcomes of polycythemia vera patients treated with pipobroman as initial therapy.  Hematol J. 2003;  4 198-207
• 21 Kiladjian J J, Bernard J F, Fenaux P. Is life expectancy of polycythemia vera patients clearly different from that of the general population?.  Am J Med. 2005;  118 565-566
• 22 Mesa R A, Silverstein M N, Jacobsen S J, Wollan P C, Tefferi A. Population-based incidence and survival figures in essential thrombocythemia and agnogenic myeloid metaplasia: an Olmsted County Study, 1976-1995.  Am J Hematol. 1999;  61 10-15
• 23 Jensen M K, de Nully Brown P, Nielsen O J, Hasselbalch H C. Incidence, clinical features and outcome of essential thrombocythaemia in a well defined geographical area.  Eur J Haematol. 2000;  65 132-139