scholarly journals Trends in Incidence, Primary Treatment and Survival of Chronic Myelomonocytic Leukemia: A Nationwide Population-Based Study Among 1,359 Patients Diagnosed in the Netherlands from 1989 to 2012

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4645-4645
Author(s):  
Avinash G Dinmohamed ◽  
Mirian Brink ◽  
Otto Visser ◽  
Pieter Sonneveld ◽  
Arjan A van de Loosdrecht ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Large Population ◽  
Age Groups ◽  
Relative Survival ◽  
Primary Treatment ◽  
Population Based ◽  
Chronic Myelomonocytic Leukemia ◽  
Population Based Study ◽  
Myelomonocytic Leukemia ◽  
Over Time

Abstract Background Chronic myelomonocytic leukemia (CMML) is a rare hematological malignancy with features of both myelodysplastic syndromes (MDS) and myeloproliferative neoplasms. Most data on CMML arrive from the few available clinical and epidemiological studies where CMML was often combined with MDS. So far, phase 3 clinical trials and large population-based studies specifically addressing CMML are lacking. We conducted a large nationwide population-based study to assess trends in incidence, primary treatment and survival among CMML patients in the Netherlands from 1989-2012. Methods We selected all patients diagnosed with CMML in 1989-2012 (N = 1,359; median age 75 years; age range 22-95 years; 63% males) from the nationwide population-based Netherlands Cancer Registry (NCR). Patients with juvenile myelomonocytic leukemia were excluded. Despite changes in classification, separate morphology codes for CMML were available in all editions of the International Classification of Diseases for Oncology (ICD-O; 9893, 9868 and 9945 in the first, second and third edition, respectively) and could therefore be identified in the NCR throughout the whole study period. The ICD-O does not have separate codes for CMML-1 or 2. Data on primary treatment, that is, no therapy or only supportive care (NT/SC), chemotherapy (CT) and CT followed by a stem cell transplantation (CT + SCT), were retrieved from the NCR. Patients were categorized into three calendar periods (1989-2000, 2001-2006 and 2007-2012) and four age groups (18-59, 60-69, 70-79 and ≥80 years), unless otherwise stated. Incidence rates were age-standardized to the European standard population and calculated per 100,000 person-years. Relative survival rates (RSRs) were computed as a measure of disease-specific survival. Results The overall age-standardized incidence rate (ASR) of CMML increased from 0.23 per 100,000 in 1989-2000, 0.31 in 2001-2006 to 0.38 in 2007-2012. The annual ASR became stable at around 0.4 per 100,000 since 2008 (Fig 1A). The proportion of patients diagnosed in individuals aged ≥70 years was 70%. The incidence of CMML was higher in men than in women, which was ascribed to the higher incidence among the 70-year-old men compared with the equivalent female group (Fig 1B). The primary treatment of CMML patients remained unchanged during the entire study period. In the overall series, 975 (72%), 365 (27%) and 19 (1%) CMML patients received NT/SC, CT and CT + SCT, respectively. The use of CT + SCT was mainly restricted to patients 18-59 (n = 13) and 60-69 (n = 6) years of age. Survival of CMML patients was poor and did not improve over time as the 5-year RSRs (with 95% confidence interval) were 16% (12%-20%), 20% (15%-25%) and 20% (15%-25%) in the three calendar periods, respectively. As shown in Figure 2, the overall 5-year RSRs for patients in the four age groups were 21% (13%-29%), 23% (18%-29%), 20% (16%-24%) and 12% (7%-18%), respectively. With the limitation of small numbers (n = 19), the overall 5- and 10-year RSRs were 29% (10%-52%) and 30% (10%-53%) for patients undergoing CT + SCT as primary treatment. In other words, the RSR reached a plateau after 5 years since diagnosis. In the most recent period, the 5-year RSR was 73% (25%-95%) for patients undergoing CT + SCT (n = 7). Conclusions In this first large population-based study including almost 1400 CMML patients, we found that the incidence of CMML increased over time until the year 2007. This rise is probably explained by improved case ascertainment and augmented disease awareness, rather than by changes in etiologic factors. Primary treatment remained conservative throughout the study period as treatment options for CMML, which primarily affects the elderly, are very limited. As a consequence, relative survival remained poor and essentially unchanged in both younger and older patients over the past two decades. Therefore, CMML-specific prognostic models should be applied in the diagnostic work-up to evaluate prognosis and plan risk-adapted treatment, and assist in designing clinical trials that specifically assess therapeutic options in CMML patients in order to improve their survival. Disclosures No relevant conflicts of interest to declare.

scholarly journals Patterns of Primary Treatment, Trial Participation and Survival in Adult Acute Myeloid Leukemia: A Nationwide Population-Based Study Among Patients Diagnosed in the Netherlands from 1989 to 2012

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 2631-2631
Author(s):  
Avinash G Dinmohamed ◽  
Otto Visser ◽  
Yvette van Norden ◽  
Nicole MA Blijlevens ◽  
Jan J. Cornelissen ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Myeloid Leukemia ◽  
Age Groups ◽  
Primary Treatment ◽  
Population Based ◽  
Trial Participation ◽  
Treatment Trial ◽  
Population Based Study ◽  
Inclusion Rate ◽  
Over Time

Abstract Background Large representative population-based studies in acute myeloid leukemia (AML) are scarce and restricted to reports from Sweden including cohorts until the year 2006 (Derolf, Blood 2009 & Juliusson, Blood 2009). So far, long-term data on primary treatment and trial participation in an unselected AML population are lacking. We conducted a nationwide population-based study to assess patterns of primary treatment, trial participation and survival among adult patients diagnosed with AML in the Netherlands from 1989 to 2012. Methods We selected all adult patients (≥18 years) diagnosed between 1989 and 2012 with acute promyelocytic leukemia (APL; N = 585; median age, 52 years; 47% males) and non-APL AML (N = 12,032; median age, 66 years; 54% males) from the nationwide population-based Netherlands Cancer Registry (NCR). Data on primary treatment for individual patients, that is, supportive care only, chemotherapy (CT) and stem cell transplantation (SCT), were retrieved from the NCR. We obtained data on the type of SCT, that is, autologous (autoSCT) or allogeneic SCT (alloSCT), from the HOVON SCT working group registry, and data on trial participation from the HOVON and EORTC cooperative trial groups. Trial participation of APL patients was outside the scope of this study. Patients were categorized into four calendar periods (1989-1994, 1995-2000, 2001-2006 and 2007-2012) and five age groups (18-40, 41-60, 61-70, 71-80 and >80 years), unless otherwise stated. We calculated relative survival rates (RSRs) as a measure of disease-specific survival. Results The overall age-standardized incidence of non-APL AML (3.0 per 100,000) and APL (0.15 per 100,000) remained stable over time. The proportion of patients with non-APL AML and APL diagnosed in individuals >60 years of age were 65% and 36%, respectively. In the overall series, 40%, 52% and 64% of the patients with non-APL AML in the first three age groups received CT, 15%, 10% and <1% received autoSCT and 38%, 27% and 7% received alloSCT, respectively. The use of alloSCT for non-APL AML increased over time among patients up to 70 years of age and were gradually applied in patients 61-70 years of age since the early 2000s (Fig 1A). Among patients with non-APL AML over 70 years of age, treatment remained conservative over time (Fig 1A). Overall, 77%, 78%, 75% and 52% of patients with APL aged 18-40, 41-60, 61-70 and >70 years received CT. The use of CT for APL increased over time in all age groups (Fig 1B). When a clinical trial was open for accrual, the inclusion rate was 68%, 57%, 30%, and 11% among patients with non-APL AML in the first four age groups, respectively. Of the patients that were not entered into a clinical trial and survived at least 30 days after diagnosis, 90%, 85%, 73% and 41% in the first four age groups received intensive therapy (CT, auto- and alloSCT) off-study, respectively. There was an overall improvement in RSRs over the study period among patients with non-APL AML and APL. The 5-year RSRs (with 95% confidence interval) increased from 12% (11%-14%), to 20% (18%-21%) for non-APL AML and from 45% (35%-54%) to 66% (58%-74%) for APL in the first and last calendar period, respectively. This improvement was mainly confined to patients with non-APL AML (Fig 2A) and APL (Fig 2B) up to 70 years of age. In addition, the RSRs also increased over time in elderly patients with APL above 70 years of age (Fig 2B). Conclusions In this comprehensive population-based study, we found that survival over the past two decades increased among patients with non-APL AML up to 70 years of age and among patients with APL in all age groups. This may be explained by the increased use of intensive and potentially curative treatment over time. The inclusion rate in clinical trials that employ intensive treatment approaches decreased with age, with a particular low accrual in patients above the age of 60. Survival remained poor and unchanged among patients with non-APL AML over 70 years of age, possibly due to the lack of age-adapted treatment approaches and the lack of clinical trials addressing the issue of age and frailty. Therefore, there is a need to design specific trials with innovative treatment strategies in elderly, frail patients who are not eligible for current clinical trials. Figure 1 Figure 1. Figure 2 Figure 2. Disclosures No relevant conflicts of interest to declare.

scholarly journals Patterns of Survival Among Patients With Myeloproliferative Neoplasms Diagnosed in Sweden From 1973 to 2008: A Population-Based Study

2012 ◽  
Vol 30 (24) ◽  
pp. 2995-3001 ◽  
Author(s):  
Malin Hultcrantz ◽  
Sigurdur Yngvi Kristinsson ◽  
Therese M.-L. Andersson ◽  
Ola Landgren ◽  
Sandra Eloranta ◽  
...  
Keyword(s):  
General Population ◽  
Life Expectancy ◽  
Excess Mortality ◽  
Myeloproliferative Neoplasms ◽  
Treatment Options ◽  
Large Population ◽  
Relative Survival ◽  
Population Based ◽  
Population Based Study ◽  
Over Time

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.

scholarly journals Success Story of Targeted Therapy in Chronic Myeloid Leukemia: A Population-Based Study of Patients Diagnosed in Sweden From 1973 to 2008

2011 ◽  
Vol 29 (18) ◽  
pp. 2514-2520 ◽  
Author(s):  
Magnus Björkholm ◽  
Lotta Ohm ◽  
Sandra Eloranta ◽  
Åsa Derolf ◽  
Malin Hultcrantz ◽  
...  
Keyword(s):  
Chronic Myeloid Leukemia ◽  
Myeloid Leukemia ◽  
Kinase Inhibitor ◽  
Large Population ◽  
Age Groups ◽  
Relative Survival ◽  
The Elderly ◽  
Population Based ◽  
Population Based Study ◽  
First Line Therapy

Purpose Chronic myeloid leukemia (CML) management changed dramatically with the development of imatinib mesylate (IM), the first tyrosine kinase inhibitor targeting the BCR-ABL1 oncoprotein. In Sweden, the drug was approved in November 2001. We report relative survival (RS) of patients with CML diagnosed during a 36-year period. Patients and Methods Using data from the population-based Swedish Cancer Registry and population life tables, we estimated RS for all patients diagnosed with CML from 1973 to 2008 (n = 3,173; 1,796 males and 1,377 females; median age, 62 years). Patients were categorized into five age groups and five calendar periods, the last being 2001 to 2008. Information on use of upfront IM was collected from the Swedish CML registry. Results Relative survival improved with each calendar period, with the greatest improvement between 1994-2000 and 2001-2008. Five-year cumulative relative survival ratios (95% Cls) were 0.21 (0.17 to 0.24) for patients diagnosed 1973-1979, 0.54 (0.50 to 0.58) for 1994-2000, and 0.80 (0.75 to 0.83) for 2001-2008. This improvement was confined to patients younger than 79 years of age. Five-year RSRs for patients diagnosed from 2001 to 2008 were 0.91 (95% CI, 0.85 to 0.94) and 0.25 (95% CI, 0.10 to 0.47) for patients younger than 50 and older than 79 years, respectively. Men had inferior outcome. Upfront overall use of IM increased from 40% (2002) to 84% (2006). Only 18% of patients older than 80 years of age received IM as first-line therapy. Conclusion This large population-based study shows a major improvement in outcome of patients with CML up to 79 years of age diagnosed from 2001 to 2008, mainly caused by an increasing use of IM. The elderly still have poorer outcome, partly because of a limited use of IM.

scholarly journals 224. Evaluating the Epidemiology of Bloodstream Infections: A Population-Based Study

2021 ◽  
Vol 8 (Supplement_1) ◽  
pp. S219-S220
Author(s):  
Elaha Niazi ◽  
Kwadwo Mponponsuo ◽  
Ranjani Somayaji ◽  
Elissa Rennert-May ◽  
John Conly ◽  
...  
Keyword(s):  
Large Population ◽  
Age Groups ◽  
Bloodstream Infections ◽  
Population Based ◽  
Incidence Rates ◽  
Population Based Study ◽  
Health Region ◽  
Canadian Health ◽  
Over Time ◽  
Age And Sex

Abstract Background Bloodstream infections (BSI) are a major cause of morbidity, mortality, and health care costs worldwide. Population-based studies are key to assess BSI epidemiology over time while minimizing selection bias but remain limited. Therefore, we aimed to assess the incidence of BSI in a large Canadian health region in a contemporary period. We hypothesized that there would be significant age and sex-based differences including over time. Methods We conducted a retrospective cohort study from 2011 through 2018 using a population-based microbiology database to determine the annual age- and sex-specific BSI testing and case rates with the census as the population reference. BSI was defined as a positive blood culture for a pathogen. Episodes &gt; 30 days apart were included for analysis. Incidence rate ratios (IRR) for testing and case rates including by sex were calculated to assess changes over time. All analyses were run at a two-sided α of 0.05 and were conducted with R 4.0.4. Results A total of 154,147 distinct individuals (49.9% male) were analyzed and 22,869 (14.8%) had a BSI at the first encounter in the study period. Overall BSI testing incidence ranged from 1529 to 1707 per 100,000 person-years and case incidence ranged from 180 to 292 per 100,000 person-years. Testing and case incidence for BSI was greatest in the 0-4 and 75+ years age groups (p &lt; 0.01). Males compared to females had greater testing and case incidence rates in young and old age groups, but females had greater rates in the 15-44 years groups (p &lt; 0.01). Overall IRR for cases comparing 2018 to 2011 was 0.62 (95% CI 0.59-0.65) reflecting a significant decrease over time. Testing also decreased over the study period with an IRR of 0.90 (95% CI 0.88-0.91). Testing and case IRRs were not significantly different stratified by sex. Incidence rates (per 100,000 person-years) of BSI testing and cases by sex from 2011 through 2018 in a Canadian health region Conclusion In our large population-based study of BSI, we identified that BSI remain frequent and the youngest and oldest age groups as well as males in these age groups have the greatest BSI incidence rates which may reflect both biological sex and gender-based differences. Encouragingly, BSI incidence rates have decreased over time at a greater increment relative to testing rates. Future studies of BSI should focus on pathogen and outcome-based evaluations. Disclosures All Authors: No reported disclosures

scholarly journals OP0248 PREMATURE MORTALITY BURDEN FOR SYSTEMIC SCLEROSIS: NATIONWIDE POPULATION-BASED STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 156.1-156
Author(s):  
E. Yen ◽  
D. Singh ◽  
M. Wu ◽  
R. Singh
Keyword(s):  
Rheumatoid Arthritis ◽  
United States ◽  
Disease Burden ◽  
Age Groups ◽  
Premature Mortality ◽  
Population Based ◽  
Age Group ◽  
Population Based Study ◽  
Mortality Burden ◽  
Over Time

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

Progress in Hodgkin lymphoma: a population-based study on patients diagnosed in Sweden from 1973-2009

Blood ◽  
2012 ◽  
Vol 119 (4) ◽  
pp. 990-996 ◽  
Author(s):  
Jan Sjöberg ◽  
Cat Halthur ◽  
Sigurdur Y. Kristinsson ◽  
Ola Landgren ◽  
Ulla Axdorph Nygell ◽  
...  
Keyword(s):  
Hodgkin Lymphoma ◽  
Treatment Options ◽  
Age Groups ◽  
Relative Survival ◽  
Population Based ◽  
Term Treatment ◽  
Cure Rate ◽  
Population Based Study ◽  
Long Term Treatment

Abstract In recent decades, attention has focused on reducing long-term, treatment-related morbidity and mortality in Hodgkin lymphoma (HL). In the present study, we looked for trends in relative survival for all patients diagnosed with HL in Sweden from 1973-2009 (N = 6949; 3985 men and 2964 women; median age, 45 years) and followed up for death until the end of 2010. Patients were categorized into 6 age groups and 5 calendar periods (1973-1979, 1980-1986, 1987-1994, 1994-2000, and 2001-2009). Relative survival improved in all age groups, with the greatest improvement in patients 51-65 years of age (P < .0005). A plateau in relative survival was observed in patients below 65 years of age during the last calendar period, suggesting a reduced long-term, treatment-related mortality. The 10-year relative survival for patients diagnosed in 2000-2009 was 0.95, 0.96, 0.93, 0.80, and 0.44 for the age groups 0-18, 19-35, 36-50, 51-65, and 66-80, respectively. Therefore, despite progress, age at diagnosis remains an important prognostic factor (P < .0005). Advances in therapy for patients with limited and advanced-stage HL have contributed to an increasing cure rate. In addition, our findings support that long-term mortality of HL therapy has decreased. Elderly HL patients still do poorly, and targeted treatment options associated with fewer side effects will advance the clinical HL field.

Patterns of Survival and Causes of Death In 9,384 Patients with Myeloproliferative Neoplasms Diagnosed In Sweden Between 1973 and 2008

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 3071-3071
Author(s):  
Malin Hultcrantz ◽  
Sigurdur Y. Kristinsson ◽  
Therese M-L Andersson ◽  
Sandra Eloranta ◽  
Åsa Rangert Derolf ◽  
...  
Keyword(s):  
General Population ◽  
Cardiac Disease ◽  
Causes Of Death ◽  
Myeloproliferative Neoplasms ◽  
Relative Survival ◽  
Relative Number ◽  
Population Based ◽  
Calendar Period ◽  
Population Based Study ◽  
Over Time

Abstract Abstract 3071 Background: Available data on survival patterns among patients diagnosed with myeloproliferative neoplasms (MPN) show a great diversity. For instance, in essential thrombocythemia (ET) there are reports stating that survival is not affected by the disease while other investigators consider ET to be a serious disease that significantly reduces life expectancy. Patients with primary myelofibrosis (PMF) are consistently reported to have a shortened life span while polycythemia vera (PV) is associated with a reduced survival in many, but not all, studies. We conducted a comprehensive, population-based study to assess survival and to define causes of death MPN patients, and to compare patterns to the general population. Patients and Methods: The nationwide Swedish Cancer Registry was used to identify all cases of MPN between 1973 and 2008 with follow-up to 2009. Relative survival ratios (RSRs) and excess mortality rate ratios (EMRRs) were computed as measures of survival. The Cause of Death Registry was used to obtain information on causes of death both in the patient and the general population. Results: A total of 9,384 MPN patients were identified (PV n=4,389, ET n=2,559, PMF n=1,048 and MPN not otherwise specified (MPN NOS) n=1,288); 47% were males and the median age at diagnosis was 71. The reporting rate to the Cancer Registry increased over time being well above 95% during the most recent calendar period. There was a significant overall excess mortality in all subtypes of MPN, reflected in 5-year and 10-year RSRs of 0.83 (95% CI 0.81–0.84) and 0.64 (0.62-0.67) for PV, 0.80 (0.78-0.82) and 0.68 (0.64-0.71) for ET and 0.39 (0.35-0.43) and 0.21 (0.18-0.25) for PMF, respectively. Higher age at MPN diagnosis was associated with a poorer survival. For example, the 10-year RSR for patients <50 years was 0.86 (0.83-0.88) as compared to 0.35 (0.29-0.43; p<0.001) in those >80 years. Females had a superior survival, EMRR 0.72 (0.66-0.78), compared to males (reference 1.00). Survival of patients with MPN improved significantly over time with an EMRR of 0.60 (0.53-0.67) in 1983–1992, 0.29 (0.25-0.34) in 1993–2000 and 0.23 (0.19-0.27) 2001–2008 using the calendar period 1973–1982 as a reference (1.00). However, MPN patients of all subtypes including PV and ET had an inferior survival compared to the general population during all calendar periods indicating that these patients continue to experience higher mortality. The 10-year RSRs for patients diagnosed 1993–2008 were 0.72 (0.67-0.76) for PV and 0.83 (0.79-0.88) for ET (Figure). The most common causes of death in MPN patients were, in order, hematological malignancy 27.2%, cardiac disease 27.1%, solid tumors 12.4% and vascular events including thromboembolism and bleeding, 9.2%. Four per cent of patients in this cohort were reported to have died due to acute myeloid leukemia. Over time, the proportion of deaths due to cardiac disease and thromboembolic events has decreased. On the other hand, we observed an increasing relative number of deaths due to hematological malignancies during the more recent calendar periods. The relative risks of dying from these causes in relation to the general population will be presented. Summary/conclusion: In this large population based study including over 9,000 MPN patients, we found all MPN subtypes to have a significantly lower life expectancy compared to the general population. Survival improved over time, however patients of all subtypes including ET had an inferior relative survival even in the most recent calendar period. Especially during earlier years, a certain misclassification and under reporting of ET may have contributed to a reduction in survival rates in the ET group. The relative number of deaths due to cardiac disease and thromboembolic events decreased during more recent calendar periods. This, and the improvement in survival might reflect the introduction of better treatment strategies for both the disease itself and for the prevention and treatment of thromboembolic complications of MPNs. Disclosures: No relevant conflicts of interest to declare.

Improving Survival in Patients with Chronic Lymphocytic Leukemia - Population Based Study

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2852-2852
Author(s):  
Marek Trneny ◽  
Petra Obrtlikova ◽  
Jiri Schwarz ◽  
Tomas Pavlik ◽  
Jan Muzik ◽  
...  
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Large Population ◽  
Relative Survival ◽  
Population Based ◽  
Lymphocytic Leukemia ◽  
Calendar Period ◽  
Population Based Study ◽  
Female Ratio ◽  
Number Of Patients ◽  
Full Picture

Abstract Abstract 2852 Background: The chronic lymphocytic leukemia is the most frequent leukemia in the western world predominantly diagnosed in older population. It is still considered to be incurable disease, but the significant proportion of patients do not require any treatment during the course of disease. The outcome is influenced by many factors including leukemia biology, patient's status, complications based on immune dysfunction, treatment choice, supportive care and other factors. Clinical trials are focused on highly selected population in need of treatment. Population based data providing the full picture is extremely rare, especially with the treatment data. The presented population-based study provides the full picture of CLL population including the untreated as well treated cohorts in different age and gender subgroups. Methods: Using data from the nationwide, population-based Czech Cancer Registry (CCR) and Czech population life-tables provided by the Czech Statistical Office to the Human Mortality Database stratified by age, sex, and calendar time we characterized trends in incidence, mortality and prevalence for all pts diagnosed with CLL in Czech Republic 1979–2008 and relative survival for patients diagnosed in five calendar periods (1980–1984, 1985–1989, 1990–1994, 1995–1999, 2000–2003). All computations were performed using Stata 10.1™ software. Results: Altogether 13, 162 pts with CLL diagnosis have been reported to the CCR from 1979 till 2008. Median age 70 years at diagnosis remains unchanged during the whole period, the male/female ratio was 1.5: 1. The CLL incidence increased from 3.3 in 1979 to 5.6 per 100 thousands inhabitants in 2008. The therapy was administered in 54% of all patients, with the significant trend to decrease with calendar period from 60.4% for pts dg in 1980–84 period to 42.3% in 200019403 period. The treatment was administered in 70.2% of pts younger than 60 y compared to 50.1% of older than 60y with the trend to decreased number of treated pts during the time in both cohorts (from 78.5% to 55.3% for younger and from 55.4% to 38.7% for older cohort). The improvement in five years relative survival ratios (RSRs; 95% confidence interval) with calendar period was observed for all patients starting at 0.46 (0.39–0.53), 0.48 (0.42–0.55), 0.52 (0.46–0.59), 0.60 (0.54–0.66) to 0.69 (0.62–0.74). The 5 y RSRs was better for untreated patients compared to treated pts resp. with improvement in both cohorts from 0.53 (0.44–0–62) and 0.34 (0.25–0.45) resp. in 1980–4 period to 0.77 (0.70–0.84) and 0.51 (0.42–0.60) resp. in 2000–3 period. The same trend was observed for young and old, untreated and treated pts. The females have significant better outcome compared to males in all cohorts, young as well old, untreated as well treated. Conclusions: In this large population-based study with more than 13, 000 patients we confirmed the increased CLL incidence together with decreased need of treatment which can be explained by the higher number of patients diagnosed in early stages in the recent time. The patients without need of treatment have siginificantly better 5y RSRs compared to pts with need of treatment, the females have significantly better 5y RSRs compared to males consistently in all subgroups. The patient's survival was improved with calendar period for the whole CLL population as well for the younger and older, females and males, untreated as well treated cohort. Disclosures: No relevant conflicts of interest to declare.

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