Chapter 15 Multiple myeloma

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1 Chapter 15 Multiple myeloma Peter Adamson Summary In the UK and in the 199s, multiple myeloma accounted for around 1 in 8 diagnosed cases of cancer and 1 in 7 deaths from cancer. There was relatively little geographical variation, although what patterns there were appeared to be similar for incidence and mortality. Incidence and mortality were noticeably higher than average in Northern and in males, and slightly lower than average in the North in both sexes. There is no apparent link between the observed geographical variations in incidence and any known or suspected risk factor for the disease. The geographical variations in incidence also appear not to be related to deprivation, and hence to any factors, including lifestyle, for which deprivation may be a marker. Introduction Multiple myeloma is a cancer which affects plasma cells in the bone marrow. In myeloma a single cell becomes malignant and produces a very large number of identical copies. Plasma cells produce antibodies which the body needs to fight infection. In myeloma normal antibody levels are reduced and this can lead to a susceptibility to life-threatening infections. It has some similarities with chronic lymphocytic leukaemia (CLL), but in myeloma, malignant cells rarely move from the bone marrow to enter the bloodstream. Incidence and mortality In the 199s there were about 1,8 newly diagnosed cases of multiple myeloma each year in males, and about 1,7 in females in the UK and. Myeloma accounted for 1.3 per cent of all cases of cancer per year in males and 1. per cent in females. Overall, the age-standardised incidence rates were 5.5 and 3.7 per 1, in males and females, respectively, a male-to-female ratio of 1.5:1. Myeloma is a disease of the elderly and rarely affects people under the age of. Incidence increased with age, with the highest rates in people in their 7s and 8s. Above the age of, all age-specific rates were markedly higher in men than in women. Multiple myeloma accounted for about 1,3 deaths in both males and females each year in the UK and in the 199s. Of all deaths from cancer, in males 1.5 per cent were due to myeloma, and in females 1.7 per cent. The agestandardised mortality rates were 3.7 per 1, in males and. in females, giving a male-to-female ratio of 1.:1 similar to that for incidence rates. Age-specific mortality followed the same pattern as for incidence, with the highest rates in those aged over 85, and higher rates in men than women. The average mortality-to-incidence ratio for the UK and was about.7 for both sexes. Incidence and mortality trends From 1971 to the mid-198s, incidence increased steeply in both sexes; the rate of increase slowed down in males and rates levelled off in females in the 199s. In the mid-199s, incidence rates were about 8 per cent higher in males and about 7 per cent higher in females than in the early 197s. This rise was mainly due to large increases in incidence in those aged 75 and over. 1 The pattern for mortality was similar, with steep increases in rates, particularly in the elderly, up to the 198s, followed by a slower increase or levelling off. 1 The increases in incidence and mortality rates were markedly higher in males than females over the period. The increases in mortality in the elderly can be partially explained by better diagnostic practice (which may also account for some of the increase in incidence) and improvements in the accuracy of death certification. Survival Survival from myeloma is remarkable in that there have been improvements in one-year, five-year and median survival but little or no improvement in long-term survival. Relative survival for patients diagnosed in England and Wales in was around and 3 per cent, at one and five years respectively, 3 compared with and 13 per cent for patients diagnosed in Ten-year survival has changed little from the 197s, increasing from about 5 per cent to 7 per cent at the end of Median survival improved from around two years in the 198s and early 199s to around four years in the late 199s. The most modern treatments can induce a complete response in patients, but remissions are not durable and the cure rate is low. 13

2 Chapter 15: Multiple myeloma Cancer Atlas of the UK and Geographical patterns in incidence As myeloma is an uncommon malignancy and the average numbers of cases and deaths per year are small (Table B15.), the variations in incidence and mortality, particularly at health authority level, should be interpreted with caution. Within the countries of the UK and, the highest incidence rates in males were in Northern and (Figure 15.1), where the rates were 15- per cent higher than the average for males. Incidence was slightly above average in the South region of England. In females, incidence rates appeared to be slightly higher than average in Scotland, Northern, and the South region of England. Rates in the North of England were below average for both sexes. Within countries and regions there was relatively little variation in incidence at the health authority level, although the range of values was wider in males than in females (Table B15.1). The maps for incidence (Map 15.1) do not show any particularly obvious geographical patterns apart from those already described at the country and regional level. Incidence was generally higher than average for both sexes in Northern, (except for the South Eastern area), southern Scotland and some of the health authorities in the northern part of the South East region of England. In much of the North and North East, and the eastern part of the South East region incidence was generally below average. Geographical patterns in mortality At the country and regional level, the pattern of mortality rates was similar to that for incidence (Figure 15.). Rates were noticeably higher than average in males in Northern and both sexes in, and slightly lower than average in both sexes in the North of England. In all other countries and regions, rates were close to the average. At the health authority level, rates showed a marked difference from the average in very few areas (Figure 15.), with no clusters of relatively high or low rates except in males in Northern and a similar pattern to that in incidence. As with incidence, there was otherwise no obvious geographical pattern in the variation of mortality rates (Map 15.), and there was less correlation in mortality between the sexes than for incidence, with the exception of. regular follow-up checks. Each year about per cent of people with MGUS will go on to develop myeloma or a related condition. 5 Additionally, autoimmune disorders, chronic immune stimulation, exposure to ionising radiation, occupational exposures, exposure to hair-colouring products, consumption of alcohol and tobacco, and a family history of myeloma and other diseases have been examined as possible risk factors for multiple myeloma. 5 Evidence from epidemiological studies supports a causal role for autoimmune disorders, exposure to ionising radiation, and occupational exposure to benzene and pesticides in the development of myeloma, 5 although these risk factors would affect very few people and are therefore unlikely to explain the observed geographical variations in incidence and mortality. Socio-economic deprivation In England and Wales in the early 199s there was no relationship between either the incidence of, or mortality from, multiple myeloma and deprivation, measured using the Carstairs index. 1 For patients diagnosed in the 197s and 198s, there was virtually no difference in survival between affluent and deprived groups, but for patients diagnosed in the late 199s, five-year relative survival was higher in the most affluent group by about 5 percentage points in men and 8 in women (for women this deprivation gap was statistically significant). The observed geographical variations in both incidence and mortality are not related to any factor for which deprivation may be a relevant marker. References 1. Quinn MJ, Babb PJ, Brock A, Kirby L et al. Cancer Trends in England and Wales Studies on Medical and Population Subjects No.. London: The Stationery Office, 1.. Coleman MP, Babb P, Damiecki P, Grosclaude P et al. Cancer Survival Trends in England and Wales, : Deprivation and NHS Region. Studies on Medical and Population Subjects No. 1. London: The Stationery Office, ONS. Cancer Survival: England and Wales, March. Available at ssdataset.asp?vlnk= Stewart BW, Kleihues P. World Cancer Report. Lyon: IARC Press, Herrinton LJ, Weiss NS, Olshan AF. Multiple myeloma. In: Schottenfeld D, Fraumeni Jnr JF (eds) Cancer Epidemiology and Prevention, second edition. New York: Oxford University Press, Coleman MP, Rachet B, Woods LM, Mitry E et al. Trends and socioeconomic inequalities in cancer survival in England and Wales up to 1. British Journal of Cancer ; 9: Risk factors and aetiology People with monoclonal gammopathy of unknown significance (MGUS) are predisposed to developing multiple myeloma. MGUS is a disorder related to myeloma. The majority of people diagnosed with MGUS never develop symptoms but require 1

3 Cancer Atlas of the UK and Chapter 15: Multiple myeloma Figure 15.1 Multiple myeloma: incidence by sex, country, and region of England UK and Males Females UK and males UK and females Northern and North Eastern London South East South 1 Northern , Age standardised using the European standard population, with 95% confidence interval England Wales Scotland Northern Figure 15. Multiple myeloma: mortality by sex, country, and region of England UK and Males Females UK and males UK and females 1 Northern and North Eastern London South East South 1 Scotland , 199- Age standardised using the European standard population, with 95% confidence interval England Wales Scotland Northern 15

4 Chapter 15: Multiple myeloma Cancer Atlas of the UK and Figure 15.3a Multiple myeloma: incidence by health authority within country, and region of England Males, UK and UK and Northern and North Eastern London South East South 1 Northern , Age standardised using the European standard population, with 95% confidence interval Wales Scotland Northern Figure 15.3b Multiple myeloma: incidence by health authority within country, and region of England Females, UK and UK and Northern and North Eastern London South East South 1 Northern , Age standardised using the European standard population, with 95% confidence interval Wales Scotland Northern 1

5 Cancer Atlas of the UK and Chapter 15: Multiple myeloma Figure 15.a Multiple myeloma: mortality by health authority within country, and region of England Males, UK and UK and Northern and North Eastern London South East South Wales Scotland Northern 1 Scotland , 199- Age standardised using the European standard population, with 95% confidence interval Figure 15.b Multiple myeloma: mortality by health authority within country, and region of England Females, UK and UK and Northern and North Eastern London South East South Wales Scotland Northern 1 Scotland , 199- Age standardised using the European standard population, with 95% confidence interval 17

6 Chapter 15: Multiple myeloma Cancer Atlas of the UK and Map 15.1a Multiple myeloma: incidence* by health authority Males, UK and Ratio* 1.5 and over 1.33 to to to to.91.7 to.75 Under.7 *Ratio of directly age-standardised rate in health authority to UK and average 18

7 Cancer Atlas of the UK and Chapter 15: Multiple myeloma Map 15.1b Multiple myeloma: incidence* by health authority Females, UK and Ratio* 1.5 and over 1.33 to to to to.91.7 to.75 Under.7 *Ratio of directly age-standardised rate in health authority to UK and average 19

8 Chapter 15: Multiple myeloma Cancer Atlas of the UK and Map 15.a Multiple myeloma: mortality* by health authority Males, UK and Ratio* 1.5 and over 1.33 to to to to.91.7 to.75 Under.7 *Ratio of directly age-standardised rate in health authority to UK and average 17

9 Cancer Atlas of the UK and Chapter 15: Multiple myeloma Map 15.b Multiple myeloma: mortality* by health authority Females, UK and Ratio* 1.5 and over 1.33 to to to to.91.7 to.75 Under.7 *Ratio of directly age-standardised rate in health authority to UK and average 171

10 Chapter 15: Multiple myeloma Cancer Atlas of the UK and 17