Survival in Primary Myelofibrosis: A Population-based Analysis in the Netherlands.
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ABSTRACT:
SUBMITTER: Slot S
PROVIDER: S-EPMC8238331 | biostudies-literature | 2021 Jul
REPOSITORIES: biostudies-literature
Publications
Survival in Primary Myelofibrosis: A Population-based Analysis in the Netherlands.
Slot Stefanie S Dinmohamed Avinash G AG Visser Otto O Te Boekhorst Peter A W PAW Zweegman Sonja S
HemaSphere 20210628 7
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Project description:Supplemental Digital Content is available in the text.
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Project description:In this nationwide, population-based study, we assessed trends in primary treatment and survival among 687 patients with nodular lymphocyte-predominant Hodgkin lymphoma (75% males; median age, 40 years; and 74% stage-I/II disease) diagnosed in the Netherlands between 1993-2016. There were no noteworthy changes in the application of primary therapy over time among adult patients across the different disease stages and age groups. Survival among various subgroups of adult patients was largely comparable to the expected survival of the general population. A particularly encouraging finding was that young adult patients experienced virtually no excess mortality, as compared to the general population.
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Project description:It is unclear how treatment advances impacted the population-level survival of patients with lymphoplasmacytic lymphoma/Waldenström macroglobulinaemia (LPL/WM). Therefore, we assessed trends in first-line therapy and relative survival (RS) among patients with LPL/WM diagnosed in the Netherlands between 1989 and 2018 (N = 6232; median age, 70 years; 61% males) using data from the nationwide Netherlands Cancer Registry. Patients were grouped into three age groups (<65, 66-75 and >75 years) and four calendar periods. Overall, treatment with anti-neoplastic agents within 1 year post-diagnosis gradually decreased over time, following a broader application of an initial watch-and-wait approach. Approximately 40% of patients received anti-neoplastic therapy during 2011-2018. Furthermore, use of chemotherapy alone decreased over time, following an increased application of chemoimmunotherapy. Detailed data among 1596 patients diagnosed during 2014-2018 revealed that dexamethasone-rituximab-cyclophosphamide was the most frequently applied regimen; its use increased from 14% to 39% between 2014 and 2018. The 5-year RS increased significantly over time, particularly since the introduction of rituximab in the early-mid 2000s. The 5-year RS during 1989-1995 was 75%, 65%, and 46% across the age groups compared to 93%, 85%, and 79% during 2011-2018. However, the survival improvement was less pronounced after 2011. Collectively, the impressive survival improvement may be accounted for by broader application of rituximab-containing therapy. The lack of survival improvement in the post-rituximab era warrants studies across multiple lines of therapy to further improve survival in LPL/WM.
| S-EPMC9292034 | biostudies-literature
Project description:Contemporary diagnosed WM patients, compared to the general population, continue to experience excess mortality regardless of having survived up to 15 years post-diagnosis. This gradual increase in excess mortality might result from the incurable nature of this disease characterized by multiple relapses throughout the disease course with limited efficacious treatment options in the released/refractory setting.
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Project description:Current cytogenetic risk stratification in primary myelofibrosis (PMF) is two-tiered: 'favorable' and 'unfavorable'. Recent studies have suggested prognostic heterogeneity within the unfavorable risk category. In 1002 consecutive patients, we performed stepwise analysis of impact on survival from individual and prognostically ordered cytogenetic abnormalities, leading to a revised three-tiered risk model: 'very high risk (VHR)'-single/multiple abnormalities of -7, i(17q), inv(3)/3q21, 12p-/12p11.2, 11q-/11q23, or other autosomal trisomies not including?+?8/?+?9 (e.g., +21, +19); 'favorable'-normal karyotype or sole abnormalities of 13q-, +9, 20q-, chromosome 1 translocation/duplication or sex chromosome abnormality including -Y; 'unfavorable'-all other abnormalities. Median survivals for VHR (n?=?75), unfavorable (n?=?190) and favorable (n?=?737) risk categories were 1.2 (HR 3.8, 95% CI 2.9-4.9), 2.9 (HR 1.7, 95% CI 1.4-2.0) and 4.4 years and survival impact was independent of clinically derived prognostic systems, driver and ASXL1/SRSF2 mutations. The revised model was also effective in predicting leukemic transformation: HRs (95% CI) were 4.4 (2.0-9.4) for VHR and 2.0 (1.2-3.4) for unfavorable. The impact of driver mutations on survival was confined to favorable and that of ASXL1/SRSF2 mutations to favorable/unfavorable cytogenetic risk categories. The current study clarifies the prognostic hierarchy of genetic risk factors in PMF and provides a more refined three-tiered cytogenetic risk model.
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Project description: Not available
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Project description:PurposeWe analysed incidence, treatment, survival, occurrence of ductal carcinoma in situ (DCIS) and invasive breast cancer (IBC) after lobular carcinoma in situ (LCIS) in the Netherlands.MethodsAll women diagnosed with classic LCIS between 1989 and 2017 were identified from the Netherlands Cancer Registry. We calculated overall (OS), relative survival (RS) and cumulative incidence functions (CIF, accounting for competing risks) of mortality, DCIS and IBC. For IBC, standardised incidence ratios (SIR) of IBC were calculated. Analyses were stratified for surgical treatment.ResultsWe included 1890 patients. Median age was 51 years. Median follow-up was 8.5 years. In 1989-2017, LCIS incidence increased from 41 to 124, surgical treatment decreased from 100% to 41.1 % - mostly BCS. 10-year OS and 20-year RS exceeded 90 % in all subgroups. Overall, 48 (2.5 %) and 270 (14.3 %) patients were diagnosed with DCIS and IBC. IBCs were mostly early-stage. After mastectomy, 13 of 14 IBCs presented contralaterally. In the other groups, 64.8-70.9 % of IBCs presented ipsilaterally, 34.5-53.9 % of these were lobular. The SIR of ipsilateral IBC was highest after no surgery (6.9, 95%CI:4.9-9.4), lowest after mastectomy (0.2, 95%CI:0.4-0.8).ConclusionLCIS incidence increased, surgical treatment decreased. The low mortality risks support consideration of active surveillance. However, the increased IBC incidence suggests careful monitoring.
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Project description:BackgroundThe aim of this study was to develop and validate reliable nomograms to predict individual overall survival (OS) and cancer-specific survival (CSS) for patients with primary tracheal tumors and further estimate the role of postoperative radiotherapy (PORT) for these entities.Patients and methodsA total of 405 eligible patients diagnosed between 1988 and 2015 were selected from the Surveillance, Epidemiology, and End Results database. All of them were randomly divided into training (n=303) and validation (n=102) sets. For the purpose of establishing nomograms, the Akaike information criterion was employed to select significant prognostic factors in multivariate Cox regression models. Both internal and external validations of the nomograms were evaluated by Harrell's concordance index (C-index) and calibration plots. Propensity score matching (PSM) method was performed to reduce the influence of selection bias between the PORT group and the non-PORT group.ResultsTwo nomograms shared common variables including age at diagnosis, histology, N and M stages, tumor size, and treatment types, while gender was only incorporated in the CSS nomogram. The C-indices of OS and CSS nomograms were 0.817 and 0.813, displaying considerable predictive accuracy. The calibration curves indicated consistency between the nomograms and the actual observations. When the nomograms were applied to the validation set, the results remained reconcilable. Moreover, the nomograms showed superiority over the Bhattacharyya's staging system with regard to the C-indices. After PSM, PORT was not associated with significantly better OS or CSS. Only squamous cell carcinoma (SCC) patients in the PORT group had improved OS compared to non-PORT group.ConclusionThe first two nomograms for predicting survival in patients with primary tracheal tumors were proposed in the present study. PORT seems to improve the prognosis of SCC patients, which needs further exploration.
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Project description:BackgroundStudies on the epidemiological information and prognosis of primary malignant lacrimal gland tumors (MLGTs) are rare for its low occurrence. The goal of our research was to investigate the epidemiological characteristics and survival outcomes of patients with MLGTs.MethodsIncidence and demographic information of patients with MLGTs were collected from the Surveillance, Epidemiology, and End Results (SEER) database. To identify independent prognostic factors for disease-specific survival (DSS) and overall survival (OS), univariate and multivariate Cox regression analysis were performed.ResultsThe overall incidence of primary MLGTs from 1975 to 2020 was 0.413/1,000,000 (according to the 2000 American standard population), with a steadily increasing incidence over years. A total of 964 patients with primary MLGTs were diagnosed, with an average age of 59.3 years. Of these, 53.2% were aged ≥60 years, 57.4% were female, and 77.1% were whites. Multivariate Cox regression analysis demonstrated that year of diagnosis, age, sex, histological type, SEER stage, surgery, and chemotherapy were independent prognostic factors of DSS or OS.ConclusionsAlthough primary MLGT is rare, its incidence has steadily increased in the past 46 years, and surgery was related to a better prognosis.
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Project description:It is unclear whether survival in diffuse large B-cell lymphoma (DLBCL) continues to increase in an era where rituximab-containing chemotherapy reigns for almost two decades. Therefore, we evaluated trends in primary therapy and relative survival (RS) among Dutch DLBCL patients diagnosed between 1989 and 2018. Analyses were performed separately according to the stage I (N = 6952) and stage II-IV disease (N = 20,676), stratified by calendar period and age (18-64, 65-74, and ≥75 years). The use of chemotherapy ± radiotherapy increased over time across all age and stage groups. As of the mid-2000s, >95% of chemotherapy-treated patients received chemoimmunotherapy, irrespective of age and stage. Overall, RS increased significantly over time across all age groups, especially after 2003 when rituximab-containing chemotherapy had become the standard of care. However, RS increased less pronounced between 2003-2010 and 2011-2018 than between 1989-2002 and 2003-2010. These findings were congruent across all studied stage groups. Five-year RS across the three age groups during 2011-2018 was 96%, 84%, and 67% for stage I DLBCL and 75%, 60%, and 46% for stage II-IV DLBCL. Collectively, survival in DLBCL increased modestly beyond the initial introduction of rituximab, with apparent survival differences across age and stage that warrant novel treatment approaches.
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