Incidence and Outcome of Chronic Myeloproliferative Disorders: A Population-Based Study from a Cancer Registry in Northern Italy.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4650-4650
Author(s):  
Alessia Bari ◽  
Raffaella Marcheselli ◽  
Ivan Rashid ◽  
Goretta Bonacorsi ◽  
Orsola Bonanno ◽  
...  

Abstract Background Because in the past Chronic Myeloproliferative Disorders (CMPD) were not considered to be malignant conditions, cancer registries rarely recorded data on these diseases. Thus, information on incidence and outcome of CMPD in the population is limited. The aim of the present study was to better define epidemiological data of CMPD by examining all cases identified by the Modena Cancer Registry (MCR). Materials and methods We considered all cases of CMPD diagnosed in the Province of Modena (population 633.993 at 2001 Census). Cases, except Chronic Myeloid Leukemia, diagnosed from 1997 to 2005, were identified using the MCR database and the archival files of the centralized hemolymphopathological laboratory at Modena Cancer Centre according to ICD-O-3 codes 9950, 9960–64. Death certificate, cytology and histology report, both local and national reports of hospital admission, ICD-9 code reported in medical records were used as sources for identifying new CMPD cases and their outcome. All cases were checked and validated by a hematologist (A.B.) and a pathologist (G.B.) by a review of the original pathology report. Uniform diagnostic criteria were adopted, because the large majority of bone marrow aspirate and biopsy were examined by the same pathologist (G.B.). Clinical and follow-up data were retrieved by active search of discharge letters, review of hospital records and interview of general practitioners. Information on vital status was achieved from official population registries. Age standardized rates (ASR) were calculated according to the World Standard population. The dates of diagnosis and death or the closing date of study (December 2006) were used to estimate survival. Observed survival and relative survival were calculated according to Kaplan-Meier method and the Hakulinen approach, respectively. Results According to the 2001 World Health Organization (WHO) classification, a total of 380 cases of CMPD were identified. There were 155 Essential Thrombocythemia (ET) (41% of all CMPD), 114 Policythemia Vera (PV) (30%), 75 Idiopathic Myelofibrosis (20%), 2 Hypereosinophilic Syndrome/Chronic Eosinophilic Leukaemia (0.5%), 1 Chronic Neutrophilic Leukemia (0.3%) and 31 CMPD not otherwise specified (8%). The ASR of CMPD was 3.2/100,000 varying slightly (from 2.5 to 4.1/100,000) during the study period (p = 0.11); the crude incidence rate was 6.6/100,000. Median age at diagnosis was 69 years. No statistically significant differences were observed between sex regarding incidence and age at diagnosis. Overall relative survival was 97%, 89% and 88% at 1, 3 and 5 years after diagnosis, respectively. Analyzing CMPD, we observed a better survival for ET and PV in comparison with other subtypes (p = 0.01). Conclusions To our knowledge, this study is the first in Italy providing information on the incidence and outcome of CMPD using population-based data. Our results confirm that the risk of developing CMPD increases with age. The incidence of CMPD was substantially stable during the study period. Overall survival patterns reflect the well known chronic course of these diseases. As expected, we observed important differences in overall survival by WHO subtypes. We believe that the availability of precise epidemiological data, in particular those regarding outcome could help clinicians in choosing the most appropriate cost-effective treatments.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4610-4610 ◽  
Author(s):  
Alessia Bari ◽  
Raffaella Marcheselli ◽  
Ivan Rashid ◽  
Goretta Bonacorsi ◽  
Roberto Marasca ◽  
...  

Abstract Background As in the past Myelodisplastic Syndromes (MDS) were considered preneoplastic conditions, rarely data on these diseases were collected by cancer registries. Thus, there are few well documented population-based studies on the incidence and outcome of MDS. The aim of this study was to collect epidemiological data and clinical characteristics of MDS by studying all cases identified by the Modena Cancer Registry (MCR). Materials and methods We examined all cases of MDS diagnosed in the Province of Modena (population 633.993 at 2001 Census). MDS from 1997 to 2005 were identified using the MCR database and the archival files of the centralized hemolymphopathological laboratory at Modena Cancer Centre according to ICD-O-3 codes 9980,9982–87,9989. Death certificate, cytology and histology report, both local and national reports of Hospital admission, ICD-9 code reported in medical records were used as sources for identifying new MDS cases and their outcome. After collection, all cases were checked and validated by a hematologist (A.B.) and a pathologist (G.B.) by a review of the original pathology report. The large majority of bone marrow aspirate and biopsy were examined by the same pathologist (G.B.) making diagnostic criteria uniform. Clinical and follow-up data were retrieved by active search of discharge letters, review of hospital records, and interview of general practitioners. Information on vital status was achieved from official population registries. Age standardized rates (ASR) were calculated according to the World Standard population (Doll et al, 1966). The dates of diagnosis and death or the closing date of study (December 2006) were used to estimate survival. Observed survival and relative survival were calculated according to Kaplan-Meier method and the Hakulinen approach, respectively. Results A total of 205 cases of MDS were identified. The ASR of MDS was 1.2/100,000 varying slightly (from 0.9 to 1.5/100,000; p > 0.05) during the study period, and the crude incidence rate was 3.6/100,000. Median age at diagnosis was 75 years for men and 78 for women. Overall, 58% of patients aged more than 75 years, while only 1% were less than 45 years old. According to French, American and British (FAB) classification there were 35 cases (17% of all MDS) of Refractory Anemia (RA), 51 (25%) of RA with ringed sideroblasts, 73 cases (36%) of RA with excess of blasts (RAEB), 11 (5%) of RAEB in transformation, 31 (15%) of MDS not otherwise specified and 4 (2%) of other MDS. Overall the prognosis of MDS was poor, although we found statistically significant differences by clinical subtypes. In our MDS population the relative survival was 68%, 36% and 26% at 1, 3 and 5 years, respectively. Conclusions To our knowledge, this study is the first in Italy providing information on the incidence and outcome of MDS using population-based data. Our results confirm that the risk of developing MDS increases with age for both men and women. The incidence of MDS was substantially stable during the study period. Overall survival was poor reflecting the aggressiveness of these diseases and the advanced age of patients at time of diagnosis. As expected, we observed important differences in overall survival by FAB subtypes. In the last few years, innovative treatments for MDS are emerging and we believe that the availability of precise epidemiological data could help clinicians in choosing the most appropriate treatment.


2014 ◽  
Vol 9 (1) ◽  
pp. 38-43 ◽  
Author(s):  
Frederick M Burkle ◽  
Christopher M Burkle

AbstractLiberia, Sierra Leone, and Guinea lack the public health infrastructure, economic stability, and overall governance to stem the spread of Ebola. Even with robust outside assistance, the epidemiological data have not improved. Vital resource management is haphazard and left to the discretion of individual Ebola treatment units. Only recently has the International Health Regulations (IHR) and World Health Organization (WHO) declared Ebola a Public Health Emergency of International Concern, making this crisis their fifth ongoing level 3 emergency. In particular, the WHO has been severely compromised by post-2003 severe acute respiratory syndrome (SARS) staffing, budget cuts, a weakened IHR treaty, and no unambiguous legal mandate. Population-based triage management under a central authority is indicated to control the transmission and ensure fair and decisive resource allocation across all triage categories. The shared responsibilities critical to global health solutions must be realized and the rightful attention, sustained resources, and properly placed legal authority be assured within the WHO, the IHR, and the vulnerable nations. (Disaster Med Public Health Preparedness. 2014;0:1-6)


2020 ◽  
Vol 30 (2) ◽  
pp. 108-109 ◽  
Author(s):  
Supot Kamsa-ard ◽  
Vor Luvira ◽  
Krittika Suwanrungruang ◽  
Siriporn Kamsa-ard ◽  
Varisara Luvira ◽  
...  

Cancers ◽  
2019 ◽  
Vol 11 (9) ◽  
pp. 1239 ◽  
Author(s):  
Abbema ◽  
Vissers ◽  
Vos-Geelen ◽  
Lemmens ◽  
Janssen-Heijnen ◽  
...  

Previous studies showed substantial improvement of survival rates in patients with cancer in the last two decades. However, lower survival rates have been reported for older patients compared to younger patients. In this population-based study, we analyzed treatment patterns and the survival of patients with breast cancer (BC) and colorectal cancer (CRC). Patients with stages I–III BC and CRC and diagnosed between 2003 and 2012 were selected from the Netherlands Cancer Registry (NCR). Trends in treatment modalities were evaluated with the Cochran-Armitage trend test. Trends in five-year overall survival were calculated with the Cox hazard regression model. The Ederer II method was used to calculate the five-year relative survival. The relative excess risk of death (RER) was estimated using a multivariate generalized linear model. During the study period, 98% of BC patients aged <75 years underwent surgery, whereas for patients ≥75 years, rates were 79.3% in 2003 and 66.7% in 2012 (p < 0.001). Most CRC patients underwent surgery irrespective of age or time period, although patients with rectal cancer aged ≥75 years received less surgery or radiotherapy over the entire study period than younger patients. The administration of adjuvant chemotherapy increased over time for CRC and BC patients, except for BC patients aged ≥75 years. The five-year relative survival improved only in younger BC patients (adjusted RER 0.95–0.96 per year), and was lower for older BC patients (adjusted RER 1.00, 95% Confidence Interval (CI) 0.98–1.02, and RER 1.00; 95% CI 0.98–1.01 per year for 65–74 years and ≥75 years, respectively). For CRC patients, the five-year relative survival improved over time for all ages (adjusted RER on average was 0.95 per year). In conclusion, the observed survival trends in BC and CRC patients suggest advances in cancer treatment, but with striking differences in survival between older and younger patients, particularly for BC patients.


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 ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 247-247 ◽  
Author(s):  
Dana E. Rollison ◽  
Matthew Hayat ◽  
Martyn Smith ◽  
Sara S. Strom ◽  
William D. Merritt ◽  
...  

Abstract BACKGROUND: Incidence rates for myelodysplastic syndromes (MDS) and chronic myeloproliferative disorders (CMD) in the United States were unavailable prior to the addition of these stem cell malignancies to the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) Program and other central cancer registries in 2001. Description of national incidence rates for 2001–2003 will provide an important baseline for future studies of secular trends and allow for the examination of rates by selected demographic factors to define risk profiles of these malignancies in the American population. METHODS: Incidence rates of MDS and CMD were calculated for 18 SEER areas between 2001–2003. These rates were stratified by disease subtype using the FAB classification (including chronic myelomonocytic leukemia [CMML]) with the addition of the WHO deletion 5q category, sex, age at diagnosis and race. Based on the observed SEER incidence rates, counts were estimated for the entire U.S. population. RESULTS: In 2003, 2,538 cases of MDS and 1,421 cases of CMD were observed for all 18 SEER areas combined. Similar numbers of cases were observed in 2001 and 2002. Age-adjusted incidence rates for 2001–2003 were significantly higher among males than females for MDS (4.5 per 100,000 in males vs. 2.7 per 100,000 in females, p <0.0001) and CMD (2.4 per 100,000 in males vs. 1.7 per 100,000 in females, p<0.0001). This gender rate difference was observed consistently across all disease subtypes, including refractory anemia (2.0 per 100,000 in males vs. 1.2 per 100,000 in females (p<0.0001). Incidence rates were significantly associated with age at diagnosis for both MDS (p=0.01) and CMD (p=0.001), and were highest among White, non-Hispanics (2.4 per 100,000 for CMD; 4.2 per 100,000 for MDS). An estimated national total of 14,648 cases of MDS (including CMML) and CMD were diagnosed in 2003, with overall incidence rates for MDS and CMD of 3.1 and 1.9 per 100,000, respectively. The MDS incidence rate for the U.S. is remarkably similar to those previously reported from European countries including England and Wales (3.6 per 100,000), Germany (4.1 per 100,000), Sweden (3.6 per 100,000) and France (3.2 per 100,000). Estimated incidence rates in the U.S. were greater among men than women for all diseases, including CMML (0.40 per 100,000 in males versus 0.3 per 100,000 in females, p< 0.0001). Disease incidence increased with age for MDS, CMD, and CMML, although the increase was greatest for MDS, with an approximate five-fold difference in estimated rates for those diagnosed at ages 60–69 years vs. 80 years and older (7.4 per 100,000 vs. 36.3 per 100,000). The increase in MDS incidence with age was greater for males than females, whereas the age-related increase in CMD and CMML incidence was similar across sexes. Rates of CMD, MDS and CMML were all estimated to be highest among White, non-Hispanics. CONCLUSION: Male sex and advanced age are important risk factors for the development of CMD and MDS. Diagnostic recording differences may underestimate the total annual U.S. MDS and CMD case burden. Future prevention intervention and disease causality studies of MDS and CMD should target high-risk groups.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 1858-1858
Author(s):  
Christina Roaldsnes ◽  
Anders Waage ◽  
Mette Nørgaard ◽  
Waleed Ghanima

Abstract Background: Polycythemia vera (PV), essential thrombocythemia (ET) and myelofibrosis (MF) are clonal hematological disorders collectively named as myeloproliferative neoplasms (MPN). Discovery of JAK2 mutation in 2005, altered WHO classification for MPN diagnosis in 2008 and availability of new treatment of MPN may have substantial effect on epidemiology of MPN. Published data on epidemiology of MPN after the discovery of JAK2 mutation and the introduction of 2008 WHO classifications for MPN, in particular on the prevalence of MPN, are scarce. We aimed to study the epidemiology of MPN in Norway and to explore the impact of JAK-2 mutation and new guidelines on the incidence of MPN using data from the Norwegian cancer registry. Method: We identified 2344 persons diagnosed with MPN from the Norwegian Cancer Registry diagnosed between 1995 and 2012. Registration of cancer in the Norwegian Cancer Registry is mandatory according to the law. We report age-adjusted incidence, prevalence and relative survival of MPN. Age adjusted incidence was reported for 2 years periods from 1995 to 2012. The prevalence was calculated according to the Norwegian population per 31.12.2011. Results: A total of 945 cases of PV was identified with a median age at diagnosis of 70 years; 471 males (50%) and 474 females (50%). The overall age-adjusted incidence rate both genders was 0.4/10⁵ in 1995-1997, 0.5/10⁵ in 1998-2000, 0.7/10⁵ in 2001-2003, 0.8/10⁵ in 2004-2007, 2008-2009 and 0.7/10⁵ in 2010-12. We identified a total of 762 cases of ET with a median age at diagnosis of 65 years, 297 males (39%) and 465 females (61%). The overall age adjusted incidence rate both genders being 0.3/10⁵ in 1995-1997 and 1998-2000, 0.5/10⁵ in 2001-2003 and 2004-2006, 0.9/10⁵ in 2007-2009 and 2010-2012. A total of 418 cases of MF was identified with a median age at diagnosis of 71 years; 243 males (58%) and 175 females (42%). Age adjusted incidence rates of both genders were 0.2/10⁵ from 1995-2006, 0.3/10⁵ in 2007-2009 and 0.5/10⁵ in 2010-2012. There were a total of 219 persons with unclassified MPN both genders,119 males (54%) and 100 females (46%) and age adjusted incidence rate varied from 0.1-0.2 to 0.1/10⁵ 1995-2012. Per 31.12.2011 the prevalence of PV, ET and MF was 9.2, 8.6 and 3.0 per 10⁵ inhabitants respectively. The survival curves for males and females for the three conditions are shown in the figure. Conclusions: This population-based study shows that the incidence of ET and MF almost doubled during the years 2007-2012 as compared to 1995-2006 as shown in the table. This increment in the incidence may possibly be related to improved diagnostics including the JAK2 mutation and the introduction of 2008 WHO-guidelines for MPN. Surprisingly, the discovery of JAK2 does not seem to have had impact on the incidence of PV as indicated by steady incidence rates since 2001. The relative survival was only slightly reduced for PV and ET, but substantially reduced for MF. Only 50% of patients with MF survive for more than 5 years. Table Incidence of MPN per 105 inhabitants during the period 1995 to 2012 in Norway 1995-97 1998-2000 2001-03 2004-06 2007-09 2010-12 PV 0.4 0.5 0.7 0.8 0.8 0.7 ET 0.3 0.3 0.5 0.5 0.9 0.9 MF 0.2 0.2 0.2 0.2 0.3 0.5 Figure showing the relative survival of PV, ET and MF Figure. showing the relative survival of PV, ET and MF Disclosures Roaldsnes: Novartis Norge AS: Research Funding. Ghanima:Novartis: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e13058-e13058
Author(s):  
Klaus Pietzner ◽  
Nina Buttmann ◽  
Jalid Sehouli ◽  
Klaus Kraywinkel

e13058 Background: Sarcoma of the female genital tract are rare tumors. They are described to be associated with a poor prognosis, when compared to gynecogical carcinoma. Aim of this study was to report incidence patterns and survival rates for gynecological sarcoma in Germany. Methods: Clinical data and survival rates for patients with gynecological sarcoma diagnosed in Germany between 2009 and 2013 were extracted from the German national centre for population-based cancer registry data. Incidence patterns and 5-year-relative survival rates were calculated. Results: A total of 2,106 gynecological sarcoma (GS) were eligible for analysis. The uterus was the most common site with 87.2% of all cases. The annual age-standardized incidence rate was 7.7 per 1 million women for all gynecological sarcoma. The median age at diagnosis was 59 years. The prognosis ranged according to site and subtype from a poor 5-year-relative survival of 47.6% (uterine leiomyosarcoma) to a very good 5-year-relative survival of 97.2% (endometrial stromal sarcoma). Conclusions: Despite the rareness of gynecological sarcoma, the size of the dataset allows a differentiation of subtypes according to morphology and site of origin. Clinically relevant differences in incidence and prognosis between subgroups were observed.


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