Trends in the incidence of chronic Philadelphia chromosome negative (Ph‐) myeloproliferative disorders in the city of Göteborg, Sweden, during 1983–99

Abstract

Abstract. Johansson P, Kutti J, Andréasson B, SafaiKutti S, Vilén L, Wedel H, Ridell B (Sahlgrenska University Hospital, Göteborg, Sweden). 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. Objective. In the literature the incidence rates for the chronic Philadelphia chromosome negative (Ph-) myeloproliferative disorders (MPD) are known to vary extensively; only a few studies have, however, been concerned with incidence trends over time. Therefore, the aim of the present work was to investigate possible trends as regards incidence rates over time for Ph-MPD. Design. Herein, we carried out a retrospective population-based survey on the incidence of polycythaemia vera (PV), essential thrombocythaemia (ET) and chronic idiopathic myelofibrosis (IMF), in the city of Göteborg (Sweden), covering the years 1983-99. Results. The study comprised 416 patients with Ph-MPD. There were 205 patients with PV, 153 patients with ET, 34 with IMF and 24 with unclassified MPD. The annual incidence for PV was 1.97 per 10 5 inhabitants; the corresponding figures for ET and IMF were 1.55 per 10 5 and 0.30 per 10 5 inhabitants, respectively. There was a significant increase in the annual incidence rate for ET (P ¼ 0.008); this increase was significant for male subjects (P ¼ 0.015) but did not reach significance for females (P ¼ 0.118). No such increase over time was recorded as regards PV and IMF. Conclusions. The increasing annual incidence rate for ET is most possibly explained by the more frequent use of automated platelet counts whenever a patient consults a doctor. Thereby, an increasing number of patients with overt thrombocytosis of unknown origin are discovered and will be referred to specialists within the field of haematology for a correct diagnosis.

Twenty-five years of epidemiological recording on myeloid malignancies: data from the specialized registry of hematologic malignancies of Cote d'Or (Burgundy, France)

“…20,22 The mean age at diagnosis in our series was close to that in both the Swedish and Danish populations but approximately 5 to 10 years younger than in patients from the South Thames area. 6,22 Data on long-term survival, such as over 15 or 20 years, are quite rare in the literature, especially from populationbased studies. In AML, the most remarkable finding of our series was the tapering of the survival increase observed in the 2000-2004 period.…”

The outcome of allo-HSCT for 92 patients with myelofibrosis in the Nordic countries

“…6 The disease primarily affects the elderly, with a median age, at diagnosis, of 67 years in one study 7 and 75 years in another. 8 Several prognostic scores have been published. The 'Lille score' was the most commonly used at the time of study design and includes Hb (o10 g/dL) and WBC counts (o4 or 430 Â 10 9 /L).…”

Kinase drug discovery approaches in chronic myeloproliferative disorders

“…MPD statistics are currently not measured by Surveillance, Epidemiology and End Results Program of the National Cancer Institute, an important source of information on cancer incidence and survival in the United States, even though the incidence of these diseases matches or exceeds other measured cancers such as multiple myeloma and CML. The most complete statistics to date on MPD incidence come from a Swedish study in 2001, which showed that the annual incidence/1 00 000 population for PV, ET and PMF are 2.8, 1.5 and 0.4, respectively (Johansson et al, 2004). The prevalence of PV, ET and PMF in the United States has been estimated to be 80-1 00 000 cases, with B2-5 new cases per 1 00 000 individuals per year (Kutti and Ridell, 2001;Tefferi and Vardiman, 2007).…”