The epidemiology of polycythaemia rubra vera in England and Wales 1968-1982

Abstract

Summary The epidemiology of polycythaemia rubra vera (PV) has not been studied extensively in the past. In 1968 PV became subject to cancer registration in England and Wales. The mortality rates and registration rates for PV were abstracted for 1968-1982. The average annual mortality rates were 3.0/million/y (men, 1068 cases) and 2.3/million/y (women, 886 cases), there being no significant increase over the time period. The average annual registration rates were 10.7/million/y (men, 3321 cases) and 6.7/million/y (women, 2207 cases) and showed a large increase from 1968 to 1974 with a stable rate subsequently. This increase was concentrated in the 65 + age groups. The median age of registration was 60-64 y with a peak of mortality and incidence between ages 75 and 84y. The data suggest some degree of overdiagnosis for PV registrations, however the rates are comparable with those seen in other studies in developed countries. The routine data sources require further validation, but they appear to provide useful information for the study of the epidemiology of PV.

Trends in the incidence of polycythemia vera among olmsted county, Minnesota residents, 1935–1989

“…For every case of PV in a female we found 1.6 cases in males, a ratio similar to that found in England and Wales, where 2,207 women and 3,321 men with PV were reported from 1968 to 1980 [9]. Because the incidence of PV rises with age and there are more elderly women than men, the excess risk among men is better assessed by the ratio of age-adjusted incidence rates, which in this study was 2.2: 1.…”

“…We did not find a significant increase in the incidence of polycythemia Vera among Olmsted County residents in the last 20 years relative to an earlier period of observation [2]. Prochazka and Markowe reported an increase in the standardized registration rate for PV in England and Wales from 1968 to 1974, with a subsequent stabilization until the end of their study period in 1982 [9]. In contrast, Berglund and Zettervall found a monotonically rising incidence from 1950 to 1984 in Malmo, Sweden, with age-adjusted rates of 1.0, 1.6, 2.2, and 2.6 per 100,000 per year for 1950-1959, 1960-1969, 1970-1979, and 1980-1984, respectively [4].…”

How common are myeloproliferative neoplasms? A systematic review and meta‐analysis

“…No interaction by geographical location for PV was identified, P = 0.813 (Table ). Crude annual incidence did not significantly differ between males (0.64 per 100,000, 95% CI: 0.28–1.45) and females (0.51 per 100,000, 95% CI: 0.21–1.23) within the studies ( n = 10 ) reporting incidence by gender. Excluding Kurita's study, crude annual incidence did not significantly differ between males (0.87 per 100,000, 95% CI: 0.58–1.30) and females (0.73 per 100,000, 95% CI: 0.46–1.15), P = 0.634 (Table ).…”

“…To obtain additional, relevant incidence and prevalence data that had not been included in reports, corresponding authors ( n = 12) were contacted to provide additional data. Communication was successful for 6 reports, contact details were no longer applicable for 1 corresponding author, 1 author had retired and 4 were unresponsive.…”

“…More recently, new mutations in the endoplasmic reticulum chaperone, CALR have been discovered in patients who are both JAK2 and MPL negative . Additional mutations have been identified that contribute to disease pathogenesis, progression and prognosis including JAK2 exon 12, LNK, TET2, MPL, CBL, and EZH2 .…”