scholarly journals Incidence and Outcome in Aplastic Anemia Diagnosed 2000-2011 - a Nationwide Swedish Registry Study

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 3905-3905
Author(s):  
Krista Vaht ◽  
Magnus Göransson ◽  
Kristina Carlson ◽  
Cecilia Isaksson ◽  
Stig Lenhoff ◽  
...  

Abstract Introduction: Aplastic anemia (AA) is a rare life-threatening disease but since the introduction of immunosuppressive therapy (IST) and allogeneic stem cell transplantation (alloHCT), the outcome in selected patient cohorts has improved considerably with a 5-year overall survival of 70-80 %. However, there is a lack of contemporary population-based data on the incidence and survival of AA. Aims: To determine incidence, treatment and survival in AA patients (pts) diagnosed in Sweden during 2000-2011. Patients and Methods: In a retrospective study, the Swedish National Patient Registry was utilized to identify pts diagnosed with AA. After careful revision of each patient's chart, 257 cases proved to fulfill the diagnostic Camitta criteria. Incidence and confidence intervals were calculated according to Rothmann, rates and proportions with Pearson's chi-square test, survival statistics using the Kaplan-Meier and log-rank method and the relative survival analysis used the Ederer II method. Results: The overall incidence of AA was 2.35 (95% CI 2.06-2.64) cases per million inhabitants per year. A biphasic age distribution was seen; one peak in pts aged 15-20 (CI 2.87; 1.72-4.03), and one in pts >60 years (4.36; CI 3.55-5.18). Median age at diagnosis was 60 (2-92) years (yrs), and median follow-up 76 (range 0-193) months. The disease severity grades were non-severe AA 38%, severe AA 38%, and very severe AA 24%, with no age-related differences. The primary treatment was IST (63%), alloHCT (10%), or palliation (27%). Distribution of initial treatment in different age groups are given in Table 1. Five-year survival of AA patients was 61%, and median survival 150 months. The 5-year survival, irrespective of treatment modality, varied in different age groups and was significantly lower in pts aged 40-59 and >60 yrs compared to young pts (p<0.05 and p<0.001, respectively); 91% in pts aged 0-18,, 91% 19-39, 71% 40-59 and 38% > 60 yrs. The age-related survival difference was visible early after diagnosis: pts >60 yrs had a 3-month survival of 84% compared to 98% for pts 0-18, 98% 19-39 and 93% for 40-59 yrs (p=0.021, 0.023 and 0.151, respectively). For all pts, type of primary treatment was significantly associated with survival; alloHCT pts had a 5-year survival rate of 96%, the IST group 69%, and palliative pts 30% (p=0.009 and p<0.0001). For patients 0-39 yrs, there was no statistical difference in survival comparing primary treatment modalities (IST or alloHCT); 0-18 yrs 86% vs 100% (p=0.158), and 19-39 yrs 90% vs 100% (p=0.395); also when grouping these pts together (88 % vs 100 %, p=0.103). In patients treated with primary IST (n=161) there was no difference in 5-year survival between the age groups 0-18, 19-39 and 40-59 yrs, whereas patients above 60 yrs did significantly worse than all other age groups; 86%, 90%, 70% and 52%, respectively (p<0.005). Forty-three (27%) pts in the entire IST group were allografted after not responding to/relapsing after 1 or 2 cycles of IST, where only 2 pts (3%) >60 yrs underwent HCT compared to 14 pts (48%), 17 pts (57%) and 10 pts (30%) in the other age groups. The relative 5-year survival (i.e. the excess mortality) for all patients was 66% (95% CI 59-72). When dividing patients by the median age at diagnosis, relative 5-year survival was 85% (CI 77-90) in patients <60 yrs, while pts >60 did significantly worse, 47.0% (CI 37-57). When splitting pts into two time-periods (2000-2005 or 2006-2011), we found no difference in 5-year survival, neither for all patients nor for the different age groups. Conclusions: Younger AA patients, regardless of initial therapy, experience a very good long-term survival. Also middle-aged patients do reasonably well but for patients above the median age at diagnosis (>60 yrs), the excess mortality is still substantial. Apparently, the challenge today is how to improve the management of elderly AA patients and prospective studies to address this medical need are warranted. Disclosures Brune: Meda Pharma: Consultancy.


2010 ◽  
Vol 28 (15) ◽  
pp. 2520-2528 ◽  
Author(s):  
Maryska L.G. Janssen-Heijnen ◽  
Adam Gondos ◽  
Freddie Bray ◽  
Timo Hakulinen ◽  
David H. Brewster ◽  
...  

Purpose When cancer survivors wish to receive accurate information on their current prognosis during follow-up, conditional 5-year relative survival may be most suitable. We have estimated conditional 5-year relative survival for 13 cancers using a large European database—European Network for Indicators on Cancer (EUNICE)—of 10 dedicated long-standing cancer registries across Europe. Patients and Methods Patients age 15 years and older diagnosed between 1985 and 2004 were included. Conditional 5-year relative survival for each age group was computed for every additional year survived up to 10 years. Period analysis with follow-up period 2000 to 2004 was used. Results All patients with cutaneous melanoma or colorectal, endometrial, or testis cancer and younger patients with stomach, glottis, cervix, ovary, or thyroid cancer or non-Hodgkin's lymphoma exhibited hardly any excess mortality (conditional 5-year relative survival > 95%) given that they were alive at a defined time point within 10 years of initial diagnosis. However, patients with supraglottis, lung, breast, and kidney cancer, as well as older patients with most cancers exhibited substantial excess mortality (conditional 5-year relative survival < 90%). Initial differences in relative survival at diagnosis between age groups largely disappeared with time since initial diagnosis for melanoma, or stomach, colorectal, corpus uteri, or testicular cancer but persisted for patients diagnosed with other tumors. Differences between stage groups became smaller over time or disappeared. Conclusion Conditional relative survival shows clinically relevant variations according to time since diagnosis, type of cancer, and age, and can help serve as a guide for cancer survivors in planning for their future and for doctors in planning schedules for surveillance.



Author(s):  
Alexander D Vardimon ◽  
Nir Shpack ◽  
Atalia Wasserstein ◽  
Marilena Skyllouriotou ◽  
Morris Strauss ◽  
...  

Background: Upper lip appearance received major attention with the introduction of diverse treatment modalities, including lip augmentation, rhinoplasty surgery, and dental treatment designed to support the upper lip. Our objectives were to define the prevalence and characteristics of the upper lip horizontal line (ULHL), which is a dynamic line appearing during a smile, in relation to gender, malocclusions, aging, and facial morphology. Methods: First, the prevalence and gender distribution of ULHL was examined from standardized en-face imaging at full smile of 643 randomly selected patients. Second, cephalometric and dental cast model analyses were made for 97 consecutive patients divided into three age groups. Results: ULHL appears in 13.8% of the population examined, and prevailed significantly more in females (78%). The prevalence of ULHL was not related to age nor to malocclusion. Patients presenting ULHL showed shorter upper lip and deeper lip sulcus. The skeletal pattern showed longer mid-face, shorter lower facial height and greater prevalence of a gummy smile. Conclusions: Female patients with short upper lip, concavity of the upper lip, and gummy smile are more likely to exhibit ULHL. The ULHL is not age-related and can be identified in children and young adults. Therefore, it should be considered when selecting diverse treatment modalities involving the upper lip.



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.



2013 ◽  
Vol 119 (1) ◽  
pp. 164-171 ◽  
Author(s):  
Anna Piippo ◽  
Aki Laakso ◽  
Karri Seppä ◽  
Jaakko Rinne ◽  
Juha E. Jääskeläinen ◽  
...  

Object The aim of this study was to assess the early and long-term excess mortality in patients with intracranial dural arteriovenous fistula (DAVF) compared with a matched general Finnish population in an unselected, population-based series. Methods The authors identified 227 patients with DAVFs admitted to 2 of the 5 Departments of Neurosurgery in Finland—Helsinki and Kuopio University Hospitals—between 1944 and 2006. All patients were followed until death or the end of 2009. Long-term excess mortality was estimated using the relative survival ratio compared with the general Finnish population matched by age, sex, and calendar year. Results The median follow-up period was 10 years (range 0–44 years). Two-thirds (67%) of the DAVFs were located in the region of transverse and sigmoid sinuses. Cortical venous drainage (CVD) was present in 28% of the DAVFs (18% transverse and sigmoid sinus, 42% others). Of the 61 deaths counted, 11 (18%) were during the first 12 months and were mainly caused by treatment complications (5 of 11, 45%). The 1-year survivors presenting with hemorrhage experienced excess mortality until 7 years from admission. However, DAVFs with CVD were associated with significant, continuous excess mortality. There were more cerebrovascular and cardiovascular deaths in this group of patients than expected in the general Finnish population. Location other than transverse and sigmoid sinuses was also associated with excess mortality. Conclusions In the patients with DAVF there was excess mortality during the first 12 months, mainly due to treatment complications. Thereafter, their overall long-term survival became similar to that of the matched general population. However, DAVFs with CVD and those located in regions other than transverse and sigmoid sinuses were associated with marked long-term excess mortality after the first 12 months.



2001 ◽  
Vol 127 (3) ◽  
pp. 501-507 ◽  
Author(s):  
M. P. MUÑOZ ◽  
A. DOMÍNGUEZ ◽  
L. SALLERAS

Varicella is a disease caused by varicella-zoster virus. It is transmitted via the respiratory route, is highly communicable and mainly affects young children. An effective vaccine is now available, whose routine use is advised by health authorities in the USA and which can prevent severe disease, although breakthrough infections do occur. In deciding whether or not to include a vaccine in the routine vaccination schedule, knowledge of the morbidity of the disease in question is fundamental. Although reporting of varicella is compulsory in Catalonia, doctors only have to report the weekly number of cases diagnosed, and not their age distribution. Given that recent data on the prevalence of the infection in Catalonia according to age groups is available, it was considered that, using these data, an estimation of age-related incidence could be made.The objective of the present study was to estimate the incidence of varicella in Catalonia on the basis of the available seroprevalence data. A curve was fitted to the observed prevalence and point prevalence estimates for all ages were obtained. The incidence was derived by smoothed prevalence for each of these age groups. Estimated variance of the estimated incidence was obtained by the delta method. Predicted prevalence in the 0–4 years age group was calculated by the smoothed prevalence.The model that best fitted the sample prevalence was the exponential function. The estimated number of varicella cases in this study was 46419 (95% CI 40507–52270). As the population in Catalonia in 1996 was 6090040, the previous results give an incidence rate of 762·2 per 100000 persons/year with their 95% CI (666·1–858·3).The method described may be applied to the study of incidence rates in relation to the prevalence of diseases if we accept that the infection produces permanent immunity; the risk of mortality is the same for infected and non-infected subjects and that the disease incidence and population remain constant in time.



Author(s):  
Ljupcho Efremov ◽  
Semaw Ferede Abera ◽  
Ahmed Bedir ◽  
Dirk Vordermark ◽  
Daniel Medenwald

Abstract Introduction Glioblastoma multiforme (GBM) is a primary malignant brain tumour characterized by a very low long-term survival. The aim of this study was to analyse the distribution of treatment modalities and their effect on survival for GBM cases diagnosed in Germany between 1999 and 2014. Methods Cases were pooled from the German Cancer Registries with International Classification of Diseases for Oncology, third edition (ICD-O-3) codes for GBM or giant-cell GBM. Three periods, first (January 1999–December 2005), second (January 2006–December 2010) and a third period (January 2011–December 2014) were defined. Kaplan–Meier plots with long-rank test compared median overall survival (OS) between groups. Survival differences were assessed with Cox proportional-hazards models adjusted for available confounders. Results In total, 40,138 adult GBM cases were analysed, with a mean age at diagnosis 64.0 ± 12.4 years. GBM was more common in men (57.3%). The median OS was 10.0 (95% CI 9.0–10.0) months. There was an increase in 2-year survival, from 16.6% in the first to 19.3% in the third period. When stratified by age group, period and treatment modalities, there was an improved median OS after 2005 due to treatment advancements. Younger age, female sex, surgical resection, use of radiotherapy and chemotherapy, were independent factors associated with better survival. Conclusion The inclusion of temozolomide chemotherapy has considerably improved median OS in the older age groups but had a lesser effect in the younger age group of cases. The analysis showed survival improvements for each treatment option over time.



2019 ◽  
Vol 54 (6) ◽  
pp. 498-505
Author(s):  
Gaëlle Romain ◽  
Anne-Sophie Mariet ◽  
Valérie Jooste ◽  
Gauthier Duloquin ◽  
Quentin Thomas ◽  
...  

<b><i>Objective:</i></b> The aim of this study was to assess long-term survival after stroke and to compare survival profiles of patients according to stroke subtypes, age, and sex, using relative survival (RS) method. <b><i>Methods:</i></b> All patients with a first-ever stroke were prospectively recorded in the population-based Dijon Stroke Registry from 1987 to 2016. RS is the survival that would be observed if stroke was the only cause of death. Ten-year RS was estimated using a flexible parametric model of the cumulative excess mortality rate, which was obtained by matching the observed all-cause mortality in the stroke cohort to the expected mortality in the general population. A separate model was fitted for each stroke subtypes, first fitted for each age and sex separately, and then adjusted for age and sex. <b><i>Results:</i></b> In total, 5,259 patients (mean age 74.9 ± 14.3 years, 53% women) were recorded including 4,469 ischemic strokes (IS), 655 intracerebral hemorrhages (ICH), and 135 undetermined strokes. In IS patients, unadjusted RS was 82% at 1 year and decreased to 62% at 10 years. Adjusted RS showed a lower survival in older age groups (<i>p</i> &#x3c; 0.001), but no difference between men and women (<i>p</i> = 0.119). In ICH patients, unadjusted RS was 56 and 42% at 1 and 10 years, respectively, with a lower adjusted survival in older age groups (<i>p</i> &#x3c; 0.001), but no sex differences (<i>p</i> = 0.184). <b><i>Conclusion:</i></b> This study showed that RS after stroke is lower in older than in younger patients but without significant sex differences, and survival profiles differ according to stroke subtypes. Since RS allows a better estimation of stroke-related death than observed survival does, especially in old patients, such a method is adapted to provide reliable information when considering long-term outcome.



2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Haomin Li ◽  
Gang Yu ◽  
Cong Dong ◽  
Zheng Jia ◽  
Jiye An ◽  
...  

AbstractEpidemiological knowledge of pediatric diseases may improve professionals’ understanding of the pathophysiology of and risk factors for diseases and is also crucial for decision making related to workforce and resource planning in pediatric departments. In this study, a pediatric disease epidemiology knowledgebase called PedMap (http://pedmap.nbscn.org) was constructed from the clinical data from 5 447 202 outpatient visits of 2 189 868 unique patients at a children’s hospital (Hangzhou, China) from 2013 to 2016. The top 100 most-reported pediatric diseases were identified and visualized. These common pediatric diseases were clustered into 4 age groups and 4 seasons. The prevalence, age distribution and co-occurrence diseases for each disease were also visualized. Furthermore, an online prediction tool based on Gaussian regression models was developed to predict pediatric disease incidence based on weather information. PedMap is the first comprehensive epidemiological resource to show the full view of age-related, seasonal, climate-related variations in and co-occurrence patterns of pediatric diseases.



Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4645-4645
Author(s):  
Avinash G Dinmohamed ◽  
Mirian Brink ◽  
Otto Visser ◽  
Pieter Sonneveld ◽  
Arjan A van de Loosdrecht ◽  
...  

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.



Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1553-1553
Author(s):  
Jan Sjoberg ◽  
Cat Halthur ◽  
Sigurdur Y Kristinsson ◽  
Ola Landgren ◽  
Paul W Dickman ◽  
...  

Abstract Abstract 1553 Poster Board I-576 We evaluated survival for all patients diagnosed with Hodgkin lymphoma (HL) in Sweden 1973-2005 (n=6,136; 3,515 men and 2,621 women; median age 40 years). Patients were categorized into six age groups and four calendar periods (1973-1980, 1981-1988, 1989-1996, and 1997-2005). Relative survival ratios (RSRs) were computed as measures of patient survival. Cumulative relative survival improved over time in all age groups with the greatest improvement in patients 51-65 years. Also in patients 66-80 years, significant improvements were recorded in both five- and ten year RSRs. Importantly, a plateau in relative survival was observed after 5 years in patients below ≤ 35 years of age during the last calendar period suggesting a lack of long-term treatment-related mortality. The ten-year RSRs in this calendar period were 0.95, 0.95, 0.92, 0.80, and 0.51 for the age groups 0-18, 19-35, 36-50, 51-65, and 66-80, respectively. Thus, despite this progress, age at diagnosis remains an important predictor of outcome. We also found a significantly better survival for women when adjusted for age and calendar period. During the study period, refined treatment options 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 patients still do poorly and targeted treatment options associated with fewer side effects will advance the clinical HL field. Cumulative relative survival stratified by age at diagnosis and calendar period of diagnosis Cumulative relative survival stratified by age at diagnosis and calendar period of diagnosis Disclosures No relevant conflicts of interest to declare.



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