African-American Multiple Myeloma Patients Have a Better Survival Than Caucasian Patients: a Population-Based Study Including 28,636 Patients.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1832-1832 ◽  
Author(s):  
Ola Landgren ◽  
Susan Devesa ◽  
Pamela Mink ◽  
William F Anderson ◽  
Brendan Weiss ◽  
...  

Abstract Abstract 1832 Poster Board I-858 Background Multiple myeloma is the most common hematologic malignancy in African-Americans with twice the incidence of Caucasians. Prior single center studies have reported poorer survival among African-American multiple myeloma patients. In contrast, recent data based on multiple myeloma patients who received autologous transplantation in an equal access health care system, showed comparable survival between African-Americans and Caucasians, suggesting that the reportedly poorer outcome for African-Americans may be due to inequalities in access to care. Also, based on publicly available cancer registry data, several publications have reported excess mortality rates for African-American multiple myeloma patients. We conducted a large population-based study including almost 30,000 multiple myeloma patients to evaluate survival patterns by race. Methods. Patient information was obtained from the Surveillance, Epidemiology and End Results (SEER) cancer registries of the National Cancer Institute. Data were drawn from the original nine SEER registries (Atlanta, Connecticut, Detroit, Hawaii, Iowa, New Mexico, San Francisco-Oakland, Seattle-Puget Sound, and Utah), which collectively cover approximately 10% of the U.S. population. Using the SEER*Stat statistical software package, we calculated 1-, 5-, and 10-year relative survival rates (RSR) of multiple myeloma patients diagnosed 1973-2004. We applied four calendar periods (1973-1979, 1980-1986, 1987-1993, and 1994-2004), follow-up through 2005 was included, and age at diagnosis was grouped into three strata (<50, 50-64, and 65+ years). Results We identified a total of 28,636 multiple myeloma patients (4,855 African-American; 23,781 Caucasian); 64% were 65 years or greater at diagnosis. When we included all patients, a comparison of survival rates in African-American and Caucasian males showed that African-American males had significantly better 5-year (32.2% vs. 28.7%) and 10-year (16.4% vs. 11.5%) RSRs (p<0.05). Similarly, African-American females had significantly better 1-year (73.3% vs. 70.2%) and 5-year (31.1% vs. 27.7%) RSRs than Caucasian females (p<0.05). When we examined survival patterns by calendar period (between 1973-1979 and 1994-2004), we found Caucasian patients to have significant improvements in the 1-year (from 67.1 to 72.4%), 5-year (from 24.1% to 31.7%), and 10-year RSR (from 9.9% to 14.9%). African-Americans also showed significantly improved 1-year RSRs (from 68.5% in 1973-1979 to 74.9% in 1994-2004) but the improvements in 5- and 10-year RSRs were not significant. When we examined survival patterns by age group, in the youngest age group (<50 years), both African-Americans and Caucasians improved 5- and 10-year RSRs, while 1-year RSR was improved for Caucasians only (from 80.9% to 84.9%). For patients 50-64 years, Caucasians had significantly improved 1-, 5-, and 10-year RSR; for African-Americans only 1-year RSR improvement was significant. Among patients 65+, there was no significant improved survival for African-Americans while Caucasians had improved 1-year (from 61.4% to 66.9%) and 5-year (from 19.6% to 24.7%) RSRs. Conclusions This large study of almost 30,000 patients found African-American multiple myeloma patients to have a significantly better prognosis than Caucasian patients, suggesting there is disease heterogeneity by race. After the introduction of newer therapies (autologous transplant, IMiDs, and bortezomib), Caucasian multiple myeloma patients showed a more pronounced survival benefit which might reflect inequalities in access to modern care; however, still African-Americans showed similar/better survival compared to Caucasians. Clinicians should be aware that the excess mortality rates for multiple myeloma among African-Americans, to a major degree, is a reflection of the fact that multiple myeloma is 2- to 3-fold more common among African-Americans. Future studies are needed to improve our understanding of the molecular basis for racial disparity patterns in multiple myeloma. Ultimately, such efforts will facilitate an improved understanding regarding disease subtype-specific benefits for individual agents, as well as mechanistic insights into drug sensitivity and resistance. Disclosures Weiss: The Binding Site, Inc.: Research Funding.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 2408-2408
Author(s):  
Sigurdur Y. Kristinsson ◽  
Ola Landgren ◽  
Paul Dickman ◽  
Asa Derolf ◽  
Magnus Bjorkholm

Abstract Background: Over the last decades there have been advances in the treatment of patients with multiple myeloma (MM) and prognosis has improved with the introduction of new treatment strategies. However, few studies have addressed the issue which patients benefit most from these therapeutic changes over the years. Aims: To evaluate relative survival in all diagnosed MM patients in Sweden 1973–2001 and relate the changes to age, sex and type of hospital where diagnosis was made. Methods: All patients with MM notified to the Swedish Cancer Register in 1973–2001 were followed up by record linkage to the nationwide Cause of Death Register. Survival analyses were performed by obtaining relative survival (RS) defined as the ratio of observed versus expected survival. The study period was divided arbitrarily to four calendar periods: 1973–1979, 1980–1986, 1987–1993, and 1994–2002. Patients were grouped according to age at diagnosis (0–40, 41–50, 51–60, 61–70, 71–80, and 80+), sex, and hospital category. RS was estimated using SAS (Cary, NC, USA) and excess mortality modelled using Poisson regression. Results: A total of 13,376 patients (7,114 males and 6,262 females, mean age 69.8 years, and 32% diagnosed at a university hospital) were diagnosed with MM in Sweden between January 1st 1973 and December 31st 2001. The overall one-year RS estimates were 73%, 78%, 80%, and 81%, respectively, for the four calendar periods. The overall five-year RS was 31%, 32%, 34%, and 36% and the ten-year RS remained stable at 12%, 11% 13% in the first three periods; ten-year RS could not be calculated for the last calendar period. The increase in one-year RS was observed in all age categories over the four calendar periods, while the increase in five-year RS was restricted to patients <70 years. Younger age at onset was associated with a superior survival in all calendar periods. Differences in survival by age at diagnosis and calendar period were highly statistically significant (p<0.0001). Females had a superior 1- (p=0.002), 5- (p=0.024), and 10-year RS (p=0.019) compared to males, after adjusting for age and period. Patients diagnosed at university hospitals had superior 5- and 10-year RS (p=0.007) but not 1-year RS. Summary/conclusions: The present study shows an improved prognosis over time in a population-based study including > 13,000 MM patients diagnosed during a 29-year period. Of interest is that even one-year RS has improved in all age groups over the whole study period. Increase in five-year RS was only observed in patients aged <70 years. The ten-year RS did not improve over the first 20 years and could not be estimated for patients diagnosed in the last period. Younger age at diagnosis was associated with superior one-, five- and ten-year RS in all calendar periods. Females had a significantly better survival than males. A significant difference in survival was seen according to type of hospital, with patients diagnosed at a university hospital surviving longer. In conclusion, the results show that survival of MM patients has improved during the study period. However, long-term survival has not improved significantly. Males, elderly patients and patients diagnosed during early calendar periods experienced higher excess mortality.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 2639-2639
Author(s):  
Jayadev Manikkam Umakanthan ◽  
Dipesh Uprety ◽  
Vineela Kasireddy

Abstract Background: Bortezomib is an antineoplastic agent that acts through protease inhibition. Since, its approval in 2003 by U.S. Food and Drug administration, it has been used religiously for multiple myeloma. There is no current population based study that assesses the survival benefit in multiple myeloma from bortezomib. We conducted a population based study to evaluate the relative survival rates in multiple myeloma patients in pre and post-Bortezomib era in the United States. Methods: We analyzed the Surveillance, Epidemiology, and End Results (SEER) 18 registry database to compare five-year relative survival rates (RS) among multiple myeloma patients. The study arms were categorized by gender (male and female), race (Caucasians and African-Americans) and age (20-59, ≥60 years). The RS were compared between pre-bortezomib era (1991-2002) and post-bortezomib era (2004-2011). The survival rate accompanied standard errors and the statistical significance was defined as p value <0.05. Results: The database comprised of 57,328 patients. The RS have improved significantly during post-bortezomib era in all cohorts under consideration. There was no significant difference in survival rate between male and female and between the African American and Caucasian. However, the relative survival was much better with the young adult (20-59 years) as compared to adult ≥60 years. The table detailing the relative survival is given below. Conclusion: The Primary treatment protocols for Multiple Myeloma have changed dramatically since the approval of Bortezomib in 2003. Other novel agents introduced in the last decade include thalidomide, Lenalidomide and liposomal doxorubicin that are used in conjunction with Bortezomib. Survival trends continue to improve and we believe this modest improvement in survival rate is mainly due to the introduction of Bortezomib and other novel agents. The next challenge is to find new drugs which would prevent relapses and further prolong survival. Abstract 2639. Table.Study armSubclass of cohortsPre-bortezomib era (1991-2002)Post- bortezomib era (2004-2011)Z scoreP ValueNRS (%)Relative Standard Error (SE) (%)NRS (%)Relative Standard Error (SE) (%) GenderMale13,06634.60.517,76546.10.615.904<0.0001Female11,58032.10.514,91743.70.614.765<0.0001 RaceCaucasian18,83632.90.423,98145.00.519.259<0.0001African American4,27434.70.86,39145.10.98.488<0.0001 Age group20-596,35848.10.69,43960.20.712.920<0.0001>=6018,28827.80.423,24338.40.516.606<0.0001 Total: male and female: 1991-2002; 24,646, RS 33.4%; SE 0.3%; 2004-2011; 32,682, RS 45.0%, SE 0.4%; Z score 21.766, P value <0.0001. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2010 ◽  
Vol 116 (25) ◽  
pp. 5501-5506 ◽  
Author(s):  
Adam J. Waxman ◽  
Pamela J. Mink ◽  
Susan S. Devesa ◽  
William F. Anderson ◽  
Brendan M. Weiss ◽  
...  

Abstract Multiple myeloma (MM) is the most common hematologic malignancy in blacks. Some prior studies suggest inferior survival in blacks; others suggest similar survival. Using the original 9 Surveillance, Epidemiology, and End Results registries, we conducted a large-scale population-based study including 5798 black and 28 939 white MM patients diagnosed 1973-2005, followed through 2006. Age-adjusted incidence rates, disease-specific survival, and relative survival rates were calculated by race, age, and time period of diagnosis. Mean age at diagnosis was 65.8 and 69.8 years for blacks and whites, respectively (P < .001). Incidence among blacks was m twice that among whites; this disparity was greater among patients < 50 years (P = .002). Over the entire study period, disease-specific and relative survival rates were higher in blacks than whites (P < .001). For whites, 5-year relative survival rates increased significantly 1973-1993 to 1994-1998 (26.3% to 30.8%; P < .001) and 1994-1998 to 1999-2005 (30.8% to 35.0%; P = .004). Survival improvements among blacks were smaller and nonsignificant (1973-1993 to 1999-2005: 31.0% to 34.1%; P = .07). We found (1) a younger age of onset among blacks; (2) better survival in blacks 1973-2005; and (3) significant survival improvement among whites over time, with smaller, nonsignificant change seen among blacks, possibly due to unequal access to and/or disparate responsiveness to novel therapies.


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2017-2017
Author(s):  
Sairah Ahmed ◽  
Yvonne T Dinh ◽  
Sofia Qureshi ◽  
Gabriela Rondon ◽  
Qaiser Bashir ◽  
...  

Abstract Abstract 2017 Background: Multiple myeloma (MM) remains an incurable disease and is the most common hematologic malignancy among African-Americans. In the United States, MM and its precursor monoclonal gammopathy of undetermined significance (MGUS) are twice as common in African Americans (Hari et al 2010). Analysis of the Surveillance Epidemiology and End Results (SEER) database from 1969 to 2003 demonstrated African-Americans have twice the mortality from MM compared to Caucasians. However this may be a function of the considerable difference in incidence of MM between Caucasian and African-American populations. Retrospective data from Southwest Oncology Group showed comparable outcomes between groups before the advent of autologous stem cell transplantation (auto-HCT). Recently Hari et al. determined that African-American and Caucasians have similar outcomes after auto-HCT for MM. In the age of novel therapy, Waxman et al addressed racial disparity in population based query of SEER and found disease specific survival was greater for African-Americans than Caucasians; and over time, survival improvement was much less pronounced among African-Americans than Caucasians. Nevertheless transplant specific data is sparse in the contemporary era with novel treatment options. Methods: We performed a retrospective review of 196 African-American multiple myeloma patients (pts) and 806 Caucasian patients initially seen at the M.D. Anderson Cancer Center from 1/1/2002 to 12/31/2010 who underwent autologous transplantation after high dose chemotherapy. The year 2002 was used to incorporate patients who had been exposed to novel agents. Results: A total of 1002 patients were analyzed, 196 African American and 806 Caucasian pts with multiple myeloma who underwent an autologous transplant. Median age at diagnosis was 59 years for both cohorts. Initial response prior to transplant was fairly evenly matched between groups (TABLE 1) as well as final response after transplant. 25% of Caucasian pts and 21% of African-American pts achieved a very good partial response (VGPR) while 28% of Caucasian and 21% of African-American pts achieved a complete response (CR). For evaluable patients, the International Staging System (ISS) at diagnosis was determined. The percentage of stage I, II and III patients in the African-American group was 27%, 20% and 17% respectively. The percentage of stage I, II and III patients in the Caucasian group was 26%, 19% and 17% respectively. Importantly 133/806 of Caucasians and 33/196 of African Americans were diagnosed at ISS stage III (p value=0.91). There was no measurable difference in progression free survival (figure 1) or overall survival (figure 1figure 2) with a maximum follow-up of >100 months. Conclusion: In this retrospective single-center study we demonstrated no difference in progression free survival or overall survival between African-American and Caucasian patients with MM treated in the era of novel agents and autologous stem cell transplantation. These findings concur with previous studies showing no difference in response to treatment between racial groups. In light of older SEER data this may be an effect of novel agents, improved access of care for African Americans or a combination of both. Disclosures: No relevant conflicts of interest to declare.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4002-4002 ◽  
Author(s):  
Wendy Cozen ◽  
Amie E. Hwang ◽  
Sikander Ailawadhi ◽  
Seema Singhal ◽  
Carol Ann Huff ◽  
...  

Abstract Abstract 4002 African-Americans (AA) have a 2–3-fold higher risk of multiple myeloma (MM) relative to Whites (Gebregziabher, 2006). We have formed a consortium and are conducting a multi-center study with 9 clinical centers and 4 NCI Surveillance, Epidemiology and End-Results (SEER) Program population-based cancer registries to determine the causes of the disease in this population and explain the excess risk (Myeloma in African-American Patients, MAP). Participation involves providing a blood or saliva specimen for DNA and answering a lifestyle and medical history questionnaire. At the end of the data collection period, a genome-wide scan will be performed and our results compared to those from 2,000 African-American controls participating in cohort studies. Patients with African ancestry (predominantly African-Americans) are identified from outpatient clinic rosters or from population-based cancer registries. For patients recruited at clinics, information on subtype, cytogenetics, FISH and lytic bone lesions is abstracted from medical records. To date, 601 patients have agreed to be in the study and we have received DNA samples from 592 patients; 54.6% are female and 45.4% are male. The mean age at diagnosis is 57 years (SD =11.2) with a median age at diagnosis of 58 years (range 27 to 90 years of age). Of the 514 subjects who completed a questionnaire, 7.8% were obese at age 20 (body mass index > 30) and 39% were obese 5 years prior to diagnosis. A first-degree relative with MM was reported by 17 cases (3%), 74% higher than the lifetime risk of 1.7% in the general population based on SEER data. In addition, cases reported 21 first-degree relatives with leukemia (4%), 7 with non-Hodgkin lymphoma (1%) and 14 with Hodgkin lymphoma (3%). To date, clinical information has been abstracted for 351 patients. Of these, 207 (58%) have active disease with the following distribution: stage I (30%), stage II (27%) and stage III (43%). The remainder have relapsed (13%), refractory (1%), relapsed and refractory (4%), or smoldering myeloma (6%), or are in remission (18%). The subtype distribution is: IgG (74%), IgA (11.4%), IgD (0.9%) and IgM (0.3%), and light chain only (13.5%); a distribution significantly different from that observed in a predominantly White population (P <0.007) (Kyle, 2003), (Table 1). Lytic bone lesions were present in 67% of patients, similar to the prevalence observed in other series. FISH and cytogenetics data on hyperdiploidy, deletions in chromosome 13 and 17p, and IGH translocations are being collected on this large cohort of African-Americans patients and will be presented at the ASH meeting. Disease characteristics in AA patients appear to be different than those previously reported in predominantly White populations. Table 1. Type of multiple myeloma and presence of bony lesions in 351 African-American patients. African-American Patients Mayo Clinic1 n % % Type     IgA 38 11.4 20     IgD 3 0.9 <10     IgG 247 74 50     IgM 1 0.3 <10     Light Chain only 45 13.5 20     Total 334     Not Available 17 Lytic Bone Lesions     Present 160 67 70     Absent 79 33     Total 239     Not Available 112 1 Kyle RA, Gertz MA, Witzig TE, Lutz JA, Lacy MQ, Dispenzieri A, Fonseca R, Rajkumar SV, Offord JR, Larson DR, Plevak ME, Themeau TM, Gregg PR, Review of 1,027 patients with newly diagnosed multiple myeloma. Mayo Clin Proc, 78: 21–33, 2003. Disclosures: No relevant conflicts of interest to declare.


Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4380-4380 ◽  
Author(s):  
Øystein Langseth ◽  
Tor Åge Myklebust ◽  
Tom Børge Johannesen ◽  
Øyvind Hjertner ◽  
Anders Waage

Background: Several population-based studies on multiple myeloma (MM) have shown an improvement in relative survival (RS) for patients aged 65 years or younger at the time of diagnosis. In patients aged 75 years or older, the improvement in long-term RS is absent in most reports or much less pronounced. To our knowledge, only 2 studies based on the Surveillance, Epidemiology and End Results (SEER) database have demonstrated a significant increase in RS for this age-group. (Pulte et al. 2011, Costa et al. 2017) We performed a population-based study on Norwegian MM-patients to provide up-to-date estimates on changes in relative survival during the past 3 decades. Methods: The Cancer Registry of Norway (CRN) was established in 1951 and provides high-quality nationwide cancer statistics. For MM, the reported completeness of case-ascertainment is above 95%. Incidence data and all reported cases of MM diagnosed between January 1, 1982 and December 31, 2017 were retrieved from the CRN (n=10 961). Nationwide drug consumption statistics for drugs used in myeloma treatment were retrieved from the Norwegian Drug Wholesales Statistics and the Norwegian Prescriptions Database, Norwegian Institute of Public Health. Follow-up ended December 31, 2017. The time of MM-diagnosis was divided into 7 categories: 1982-1987, 1988-1992 (melphalan-prednisone), 1993-1997(early high-dose melphalan with autologous stem-cell transplant (HDM-ASCT)), 1998-2002 (introduction of thalidomide), 2003-2007 (early thalidomide upfront, introduction of bortezomib), 2008-2012 (thalidomide and bortezomib upfront, introduction of lenalidomide), 2013-2017 (lenalidomide upfront, early pomalidomide, daratumumab, panobinostat and carfilzomib). Age at diagnosis was divided into 3 categories; <65 years, 65-79 years and 80 years or older. We estimated relative survival ratios (RSR) with 95% confidence intervals 5 and 10 years after the time of diagnosis by the Ederer-II method. The analysis was stratified by age-group and calendar period of diagnosis. Cohort analysis was applied to calendar periods with complete follow-up, and period analysis was applied where complete-follow up was not available (2013-2017 and 10-year estimates for 2008-2012). Non-overlapping confidence intervals were considered statistically significant. Results: The age-standardized incidence rate was stable until approximately year 2000, followed by an increasing tendency reaching 8.4 cases per 100 000 persons in 2017. Figure 1. Patients diagnosed before the age of 65 had a steady increase in both 5 and 10-year RSR across all calendar periods. For patients aged 65-79 years, the 5- and 10-year RSR's were stable at approximately 0.3 and 0.1, respectively, until the calendar period 1998-2002. In the following years, an improvement in both 5- and 10-year RSR was observed. The 5-year RSR improved significantly from 0.31 (95% CI; 0.27-0.35) in the first calendar period to 0.43 (95% CI;0.39- 0.47) during 2008-2012. The predicted 5-year RSR for 2013-2017 was 0.48 (95% CI; 0.44-0.52). There were also signs of improved 10-year RSR, predicted to 0.23 (95% CI; 0.18-0.27) during 2013-2017 compared to 0.11 (95% CI; 0.08-0.14) during 1982-1987. The 5-year RSR for patients aged 80+ years was 0.11 (95% CI; 0.01-0.17) during 1982-1987 and the 10-year RSR was 0.03 (95% CI; 0.01-0.11). In the following 4 calendar periods the RSR-estimates fluctuated before a rising tendency during the last 2 periods. The 5-year RSR improved significantly to 0.26 (95% CI; 0.20-0.32) during 2008-2012 and further rising to a predicted value of 0.32 (95% CI; 0.25-0.38) during 2013-2017. Figure 2. Additionally, complete annual prescription statistics for the oral agents thalidomide, lenalidomide and pomalidomide were obtained. Conclusions: We provide real-world observations on changes in RS in a population-based cohort of 10 961 MM-patients. We demonstrate an improvement in 5-year relative survival across all age-groups, including patients aged 80 years or older. For patients <65 years, there was a steady increase in RS since 1982 and we see no obvious impact of the implementation of HDM-ASCT. For the other age-groups, the improvement in RS coincides with the introduction of new drugs. The increase in incidence since 2000 may be due to increased testing for M-protein. We can point to the introduction of new drugs and increased incidence of indolent cases as possible reasons for increasing RS. Disclosures No relevant conflicts of interest to declare.


Author(s):  
Ingigerður S. Sverrisdóttir ◽  
Sölvi Rögnvaldsson ◽  
Sigrún Thorsteinsdottir ◽  
Gauti K. Gíslason ◽  
Thor Aspelund ◽  
...  

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 156.1-156
Author(s):  
E. Yen ◽  
D. Singh ◽  
M. Wu ◽  
R. Singh

Background:Premature mortality is an important way to quantify disease burden. Patients with systemic sclerosis (SSc) can die prematurely of disease, however, the premature mortality burden of SSc is unknown. The years of potential life lost (YPLL), in addition to age-standardized mortality rate (ASMR) in younger ages, can be used as measures of premature death.Objectives:To evaluate the premature mortality burden of SSc by calculating: 1) the proportions of SSc deaths as compared to deaths from all other causes (non-SSc) by age groups over time, 2) ASMR for SSc relative to non-SSc-ASMR by age groups over time, and 3) the YPLL for SSc relative to other autoimmune diseases.Methods:This is a population-based study using a national mortality database of all United States residents from 1968 through 2015, with SSc recorded as the underlying cause of death in 46,798 deaths. First, we calculated the proportions of deaths for SSc and non-SSc by age groups for each of 48 years and performed joinpoint regression trend analysis1to estimate annual percent change (APC) and average APC (AAPC) in the proportion of deaths by age. Second, we calculated ASMR for SSc and non-SSc causes and ratio of SSc-ASMR to non-SSc-ASMR by age groups for each of 48 years, and performed joinpoint analysis to estimate APC and AAPC for these measures (SSc-ASMR, non-SSc-ASMR, and SSc-ASMR/non-SSc-ASMR ratio) by age. Third, to calculate YPLL, each decedent’s age at death from a specific disease was subtracted from an arbitrary age limit of 75 years for years 2000 to 2015. The years of life lost were then added together to yield the total YPLL for each of 13 preselected autoimmune diseases.Results:23.4% of all SSc deaths as compared to 13.5% of non-SSc deaths occurred at <45 years age in 1968 (p<0.001, Chi-square test). In this age group, the proportion of annual deaths decreased more for SSc than for non-SSc causes: from 23.4% in 1968 to 5.7% in 2015 at an AAPC of -2.2% (95% CI, -2.4% to -2.0%) for SSc, and from 13.5% to 6.9% at an AAPC of -1.5% (95% CI, -1.9% to -1.1%) for non-SSc. Thus, in 2015, the proportion of SSc and non-SSc deaths at <45 year age was no longer significantly different. Consistently, SSc-ASMR decreased from 1.0 (95% CI, 0.8 to 1.2) in 1968 to 0.4 (95% CI, 0.3 to 0.5) per million persons in 2015, a cumulative decrease of 60% at an AAPC of -1.9% (95% CI, -2.5% to -1.2%) in <45 years old. The ratio of SSc-ASMR to non-SSc-ASMR also decreased in this age group (cumulative -20%, AAPC -0.3%). In <45 years old, the YPLL for SSc was 65.2 thousand years as compared to 43.2 thousand years for rheumatoid arthritis, 18.1 thousand years for dermatomyositis,146.8 thousand years for myocarditis, and 241 thousand years for type 1 diabetes.Conclusion:Mortality at younger ages (<45 years) has decreased at a higher pace for SSc than from all other causes in the United States over a 48-year period. However, SSc accounted for more years of potential life lost than rheumatoid arthritis and dermatomyositis combined. These data warrant further studies on SSc disease burden, which can be used to develop and prioritize public health programs, assess performance of changes in treatment, identify high-risk populations, and set research priorities and funding.References:[1]Yen EY….Singh RR. Ann Int Med 2017;167:777-785.Disclosure of Interests:None declared


2012 ◽  
Vol 30 (24) ◽  
pp. 2995-3001 ◽  
Author(s):  
Malin Hultcrantz ◽  
Sigurdur Yngvi Kristinsson ◽  
Therese M.-L. Andersson ◽  
Ola Landgren ◽  
Sandra Eloranta ◽  
...  

PurposeReported survival in patients with myeloproliferative neoplasms (MPNs) shows great variation. Patients with primary myelofibrosis (PMF) have substantially reduced life expectancy, whereas patients with polycythemia vera (PV) and essential thrombocythemia (ET) have moderately reduced survival in most, but not all, studies. We conducted a large population-based study to establish patterns of survival in more than 9,000 patients with MPNs.Patients and MethodsWe identified 9,384 patients with MPNs (from the Swedish Cancer Register) diagnosed from 1973 to 2008 (divided into four calendar periods) with follow-up to 2009. Relative survival ratios (RSRs) and excess mortality rate ratios were computed as measures of survival.ResultsPatient survival was considerably lower in all MPN subtypes compared with expected survival in the general population, reflected in 10-year RSRs of 0.64 (95% CI, 0.62 to 0.67) in patients with PV, 0.68 (95% CI, 0.64 to 0.71) in those with ET, and 0.21 (95% CI, 0.18 to 0.25) in those with PMF. Excess mortality was observed in patients with any MPN subtype during all four calendar periods (P < .001). Survival improved significantly over time (P < .001); however, the improvement was less pronounced after the year 2000 and was confined to patients with PV and ET.ConclusionWe found patients with any MPN subtype to have significantly reduced life expectancy compared with the general population. The improvement over time is most likely explained by better overall clinical management of patients with MPN. The decreased life expectancy even in the most recent calendar period emphasizes the need for new treatment options for these patients.


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