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Blood, 1965, Vol. 26, No. 3, pp. 243-256.
© 1965 American Society of Hematology, Inc.
Polycythemia Vera: Age Relationships and Survival
EDWIN E. OSGOOD 1
1 Division of Experimental Medicine, University of Oregon Medical School,
Portland, Oregon.
Study of our first 201 cases of polycythemia vera and of cases reported in
the literature shows a remarkable age distribution for this disease. The age
at onset, age at diagnosis, age at first treatment, and age at death, as well as
survival times, each fits a normal distribution.
In our cases, the median age at onset is 57 years with a standard deviation
of 13 years, an entirely different distribution from that for the population
of Oregon. This requires a logarithmic increase in relative age specific incidence from age 20 to 40, when there is little difference in the number of
persons alive at each age, with a doubling of the proportion occurring in
each 5-year interval every 7.5 years. After age 55, the proportion of new
cases developing follows the slope of the number alive in the population.
This means that the incidence remains almost constant after the peak age
incidence is reached.
Unfortunately, no data exist to transform these figures to absolute values,
but if enough of the population could be studied to give absolute values at
any one point, all other points could be determined. The implication is that
polycythemia vera is due to a single cause which is highly correlated with
age.
The normal distribution of survival times means that polycythemia vera
is not a malignant process since survival times in all malignancies studied
fit a log normal distribution. The constant value of age at death means that
age at first treatment is a most important prognostic factor.
The mean age at death of patients with polycythemia vera, treated with
P32, is 69 years ± 1, and treated without radiation therapy is 65 years ± 1.
Apparently, if a patient lives to be treated with P32 previous treatment by
other modalities or no treatment prior to that, will not affect the years to
be gained by P32 treatment. However, the total survival time is highly correlated with age and the sooner after onset that the patient can be treated
the more likely it is he will benefit from P32 therapy.
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