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Background: The outcome for diffuse large B‐cell lymphoma (DLBCL) patients has improved with the immunochemotherapy combination R‐CHOP. An increased rate of heart failure is well documented following this treatment, whereas incidence and outcome of other cardiac complications, for example myocardial infarction, are less well known. Method: We identified 3548 curatively treated DLBCL patients in Sweden diagnosed between 2007 and 2014, and 35474 matched lymphoma‐free general population comparators. The incidence, characteristics and outcome of acute myocardial infarctions (AMIs) were assessed using population‐based registers up to 11 years after diagnosis. The rate of AMI was estimated using flexible parametric models. Results: Overall, a 33% excess rate of AMI was observed among DLBCL patients compared with the general population (HR: 1.33, 95% CI: 1.14–1.55). The excess rate was highest during the first year after diagnosis and diminished after 2 years. High age, male sex and comorbidity were the strongest risk factors for AMI. Older patients (>70 years) with mild comorbidities (i.e. hypertension or diabetes) had a 61% higher AMI rate than comparators (HR: 1.61, 95% CI: 1.10–2.35), whereas the corresponding excess rate was 28% for patients with severe comorbidities (HR: 1.28, 95% CI: 1.01–1.64). Among younger patients (≤70), a short‐term excess rate of AMI was limited to those with severe comorbidities. There was no difference in AMI characteristics, pharmacological treatment or 30‐day survival among patients and comparators. Conclusion: DLBCL patients have an increased risk of AMI, especially during the first 2 years, which calls for improved cardiac monitoring guided by age and comorbidities. Importantly, DLBCL was not associated with differential AMI management or survival. [ABSTRACT FROM AUTHOR]

A few patients with MCL present with limited stage disease and for these, involved node radiotherapy may be a well tolerable alternative to immunochemotherapy regimens. How to choose between curative radiotherapy or chemotherapy in low stage MCL? Here, we aim to evaluate progression free survival (PFS) and overall survival (OS) in relation to given treatment and prognostic factors in patients with stage I-II MCL. [Extracted from the article]

B Introduction: b With modern treatments, mantle cell lymphoma (MCL) patients may experience long-lasting remission resulting in a growing population of long-term survivors. Higher rates in patients were observed across all primary treatment groups: Nordic-MCL2 protocol (250 patients, HR SB adj sb = 1.4(1.0-2.0)), R-CHOP single (144 patients, HR SB adj sb = 2.0(1.3-2.9)), R-bendamustine (302 patients, HR SB adj sb = 2.2(1.7-2.7)), lenalidomide (16 patients, HR SB adj sb = 4.3(2.0-9.0)) (Figure 1), R-CHOP/Cytarabine (145 patients, HR SB adj sb = 1.6(1.0-2.5)), and ibrutinib (9 patients, HR SB adj sb = 4.8(1.2-18.7)) contrasted with comparators. B Conclusions: b MCL survivors have an increased risk of SM, particularly patients given primary treatment with R-bendamustine, but also patients treated with other chemotherapies or novel targeted drugs (lenalidomide, ibrutinib), though only few patients were included in the latter group and the results should be interpreted accordingly. [Extracted from the article]

We speculate that the higher survival rate among young high-risk patients may be driven by the introduction of CAR-T in the last few years, whereas the more general and successive improvement among older patients could be due to intensified treatment schemes in the primary as well as relapsed settings. B Introduction: b Most patients diagnosed with diffuse large B-cell lymphoma (DLBCL) are cured with primary immunochemotherapy, but about one in four experience relapsed/refractory (R/R) disease with worsened outcome. In young high-risk patients, the 2-year RS increased from 71% among patients diagnosed 2007-2009, to 83% among patients diagnosed 2019-2021 ( I p i SB overall sb < 0.001). [Extracted from the article]

25% had low Ig, defined as any deficiency, 18% had low IgG, 37% lymphopenia and 13% both low Ig and lymphopenia. Furthermore, hypogammaglobulinemia was associated with an increased risk of severe infections, indicating that hypogammaglobulinemia is common in DLBCL at diagnosis and may have clinical impact in terms of treatment complications and outcome. [Extracted from the article]

Purpose: This study seeks to investigate the long-term public health burden of Hodgkin lymphoma (HL) in terms of work loss following contemporary treatment protocols and associations with established treatment complications and lymphoma relapse.Methods: We identified 1,989 Swedish HL patients (1,082 with clinical information) aged 18-60 (median 33) years at diagnosis 1992-2009, and matched 1:4 to population comparators. Sick leave, disability pension (work loss), and comorbidity were retrieved through September 2013. Relative risks (RR) with 95% confidence intervals (CI) were calculated using Poisson regression, and mean lost work days were estimated yearly during follow-up.Results: The risk of annual work loss was elevated in HL survivors versus comparators up to the 15th year post-diagnosis (RR(5th year) 1.64, 95% CI 1.46-1.84; RR(10th year) 1.33, 95% CI 1.15-1.34; and RR(15th year) 1.30, 95% CI 1.04-1.62). The risk remained elevated up to the 10th year after adjustment for secondary malignancies and cardiovascular disease (RR(10th year) 1.31, 95% CI 1.13-1.52). Advanced-stage patients had more lost days than comparators (mean number(5th year) 66 versus 33, mean difference 34, 95% CI 20-48) as did patients receiving 6-8 chemotherapy courses (62 versus 33, mean difference(5th year) 30, 95 % CI 17-43). Among patients in the first complete remission, a difference was still observed for advanced-stage (51 versus 33, mean difference(5th year) 19, 95% CI 5-34) but not early-stage disease.Conclusions: Advanced-stage HL survivors treated with full-dose chemotherapy were at increased risk of work loss, not only explained by relapse, secondary malignancies, or cardiovascular disease.Implications For Cancer Survivors: The results call for increased awareness and evaluation of reasons for long-term work disability following intensive chemotherapy among young HL survivors. [ABSTRACT FROM AUTHOR]

There are some evidences that hypogammaglobulinemia in newly diagnosed diffuse large B cell lymphoma (DLBCL) is a predictor for inferior outcome, but the risk for infection-related admissions specifically related to hypogammaglobulinemia is not known. The aim was to explore if hypogammaglobulinemia in untreated DLBCL in a Swedish cohort was associated with inferior outcome, and to assess the relationship between low immunoglobulin (Ig) levels and infections. Using data from the Swedish Lymphoma Register, we retrospectively identified patients above18 years diagnosed with DLBCL, receiving anthracycline-based curative therapy during 2000-2015 in Southern Sweden with Ig-levels tested at baseline. Data on Ig levels and infections were collected from medical records. Five hundred eighty-five patients were included, median age was 69 years. Hypogammaglobulinemia was detected at baseline in 24%, the most common Ig deficiency was IgG (18%), followed by IgA (10%) and IgM (8%). Hypogammaglobulinemia was associated with inferior overall survival (HR 1.4, 95% CI 1.0-1.8, p-value 0.018), but not when adjusted for International Prognostic Index (IPI). Low levels of Ig were associated with more infections during lymphoma treatment (p-value 0.013), also when adjusted for IPI (p-value < 0.001). Among patients with IgG deficiency, 47% had ≥ 1 infections versus 35% in patients with normal IgG (HR 1.2, p = 0.025). In conclusion, hypogammaglobulinemia was a frequent finding in patients with newly diagnosed DLBCL, with clinical impact in terms of treatment complications and outcome., (© 2024 John Wiley & Sons Ltd.)

Background and Purpose: The Swedish Lymphoma Register (SLR) was initiated in the year 2000 with the aim to monitor quality of care in diagnostics, treatment and outcome of all lymphomas diagnosed nationally among adults. Here, we present the first systematic validation of SLR records as a basis for improved register quality and patient care., Patients and Methods: We evaluated timeliness and completeness of register records among patients diagnosed with lymphoma in the SLR (n = 16,905) compared with the National Cancer Register for the period 2013-2020. Comparability was assessed through evaluation of coding routines against national and international guidelines. Accuracy of 42 variables was evaluated through re-abstraction of data from medical records among 600 randomly selected patients diagnosed in 2016-2017 and treated across all six Swedish healthcare regions.  Results: Completeness was high, >95% per year for the period 2013-2018, and >89% for 2019-2020 compared to the National Cancer Register. One in four patients was registered within 3 months, and 89.9% within 2 years of diagnosis. Registration instructions and coding procedures followed the prespecified guidelines. Missingness was generally low (<5%), but high for occasional variables, for example, those describing maintenance and consolidative treatment. Exact agreement of categorical variables was high overall (>80% for 24/34 variables), especially for treatment-related data (>80% for 17/19 variables)., Interpretation: Completeness and accuracy are high in the SLR, while timeliness could be improved. Finetuning of variable registration guided by this validation can further improve reliability of register reports and advance service to lymphoma patients and health care in the future.

Background: Achieving lasting remission for at least 2 years is a good indicator for favourable prognosis long term after Diffuse large B-cell lymphoma (DLBCL). The aim of this study was to provide real-world probabilities, useful in risk communication and clinical decision-making, of the chance for lasting remissions by clinical characteristics., Methods: DLBCL patients in remission after primary treatment recorded in the Swedish Lymphoma register 2007-2014 (n = 2941) were followed for relapse and death using multistate models to study patient trajectories. Flexible parametric models were used to estimate transition rates., Results: At 2 years, 80.7% (95% CI: 79.0-82.2) of the patients were predicted to remain in remission and 13.2% (95% CI: 11.9-14.6) to have relapsed. The relapse risk peaked at 7 months, and the annual decline of patients in remission stabilised after 2 years. The majority of patients in the second remission transitioned into a new relapse. The probability of a lasting remission was reduced by 20.4% units for patients with IPI 4-5 compared to patients with IPI 0-1, and time in remission was shortened by 3.5 months., Conclusion: The long-term prognosis was overall favourable with 80% achieving durable first remissions. However, prognosis varied by clinical subgroups and relapsing patients seldom achieved durable second remissions., (© 2022. The Author(s).)

We performed a national population-based study of all patients diagnosed with diffuse large B-cell lymphoma (DLBCL) in Sweden in 2007-2014 to assess treatment intent and risk of relapsed/refractory disease, including central nervous system (CNS) relapse, in the presence of competing risks. Overall, 84% of patients started treatment with curative intent (anthracycline-based) (n = 3550, median age 69 years), whereas 14% did not (n = 594, median age 84 years) (for 2% the intent was uncertain). Patients treated with curative intent had a 5-year OS of 65.3% (95% CI: 63.7-66.9). The median OS among non-curatively treated patients was 2.9 months. The 5-year cumulative incidence of relapsed/refractory disease in curative patients was 23.1% (95% CI: 21.7-24.6, n = 847). The 2-year cumulative incidence of CNS relapse was 3.0% (95% CI: 2.5-3.6, n = 118) overall, and 8.0% (95% CI: 6.0-10.6, n = 48) among patients with high CNS-IPI (4-6), when considering other relapse locations and death as competing events. The incidence of relapsed/refractory DLBCL overall and in the CNS was lower than in previous reports, still one in seven patients was not considered fit enough to start standard immunochemotherapy at diagnosis. These results are important for quantification of groups of DLBCL patients with poor prognosis requiring completely different types of interventions.

Survival for patients diagnosed with mantle cell lymphoma (MCL) has improved drastically in recent years. However, patients carrying mutations in tumour protein p53 (TP53) do not benefit from modern chemotherapy-based treatments and have poor prognosis. Thus, there is a clinical need to identify missense mutations through routine analysis to enable patient stratification. Sequencing is not widely implemented in clinical practice for MCL, and immunohistochemistry (IHC) is a feasible alternative to identify high-risk patients. The aim of the present study was to investigate the accuracy of p53 as a tool to identify patients with TP53 missense mutations and the prognostic impact of overexpression and mutations in a Swedish population-based cohort. In total, 317 cases were investigated using IHC and 255 cases were sequenced, enabling analysis of p53 and TP53 status among 137 cases divided over the two-cohort investigated. The accuracy of predicting missense mutations from protein expression was 82%, with sensitivity at 82% and specificity at 100% in paired samples. We further show the impact of p53 expression and TP53 mutations on survival (hazard ratio of 3·1 in univariate analysis for both), and the association to risk factors, such as high MCL International Prognostic Index, blastoid morphology and proliferation, in a population-based setting., (© 2020 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.)

Non-Hodgkin lymphoma (NHL) prognosis has improved in recent years, yet the number of patients living with the diagnosis, i.e. the prevalence, has seldom been reported. The prevalence provides a measure of the burden of disease, useful for healthcare planning and to optimise resource allocation. We provide a systematic presentation of temporal trends in absolute numbers of prevalent patients by NHL subtypes, linking them to trends in incidence, survival and mortality. Patients diagnosed 2000-2016 were identified in the national Swedish lymphoma register. Incidence and mortality rates, relative survival and prevalence were estimated for NHL overall and for major clinical and morphological subtypes. Poisson regression was used to test for temporal trends. Increasing incidence and improved survival have led to a 47% increase in the five-year prevalence of NHL overall in 2016 compared to 2004. An increasing prevalence was observed for all investigated subtypes during the study period, but most notably for diffuse large B cell lymphomas among aggressive subtypes (66%), and marginal zone lymphomas among indolent subtypes (135%). This dramatic increase in NHL prevalence underscores the need to develop and evaluate alternative follow-up schemes to use resources efficiently and still ensure optimal care of lymphoma survivors., (© 2020 British Society for Haematology and John Wiley & Sons Ltd.)

Background: Comorbidity impacts overall survival amongst patients with diffuse large B-cell lymphoma (DLBCL). However, associations of comorbidity with lymphoma characteristics, treatment selection and lymphoma-specific mortality are less well known., Objective: To examine the impact of comorbidity on DLBCL characteristics, treatment intent and cause of death., Methods: We identified 3905 adult patients diagnosed with DLBCL 2007-2013 through the Swedish Lymphoma Register. We assessed comorbid disease history according to the Charlson comorbidity index (CCI). Comorbidity data and causes of death were collected through register linkage. Associations were estimated using multinomial regression and flexible parametric survival models., Results: Overall, 45% of the patients (n = 1737) had a history of at least one comorbidity at DLBCL diagnosis (cardiovascular disease, diabetes and solid cancer were most frequent), and 997 (26%) had a CCI score of ≥2. The relative probability of presenting with poor performance status (PS > 2) was higher amongst comorbid patients [Relative Risk Ratio (RRR) PS >2 : 2.02, 95% CI: 1.63-2.51]. Comorbid patients had a substantially lower relative probability of receiving curative treatment (RRR: 0.48, 95% CI: 0.38-0.61). Amongst all patients, CCI ≥ 1 was associated with a significantly increased risk of all-cause and lymphoma-specific death after adjustments. Amongst patients selected for curative treatment, comorbidity was associated with an increased risk of all-cause death (HR CCI >1 : 1.54, 95% CI: 1.32-1.80), but not with lymphoma-specific death (HR CCI >1 : 1.05, 95% CI: 0.86-1.28)., Conclusion: Comorbidity is associated with inferior DLBCL outcome, mainly due to a lower likelihood of receiving treatment with curative intent. Possibly, more comorbid DLBCL patients could be treated with curative intent if comorbid conditions were optimized in parallel., (© 2018 The Association for the Publication of the Journal of Internal Medicine.)

High body mass index (BMI) is associated with development of hematological malignancies (HMs). However, although BMI is a well-established measurement of excess weight, it does not fully reflect body composition and can sometimes misclassify individuals. This study aimed at investigating what body composition measurements had highest association with development of HM. Body composition measurements on 27,557 individuals recorded by healthcare professionals as part of the Malmö Diet and Cancer study conducted in Sweden between 1991-1996 were matched with data from national registers on cancer incidence and causes of death. Cox regression models adjusted for age and sex were used to test the association between one standard deviation increments in body composition measurements and risk of HM. During a median follow-up of 20 years, 564 persons developed an HM. Several body composition measurements were associated with risk of developing an HM, but the strongest association was found for multiple myeloma (MM). Waist circumference (HR 1.31, p = 0.04) and waist-hip ratio (HR 1.61, p = 0.05) had higher risk estimates than BMI (HR 1.18, p = 0.07) for MM. In conclusion, our study shows that measurements of abdominal adiposity better predict the risk of developing HM, particularly MM, compared to BMI., Competing Interests: The authors have declared that no competing interests exist.

The international prognostic index (IPI) and similar models form the cornerstone of clinical assessment in newly diagnosed diffuse large B-cell lymphoma (DLBCL). While being simple and convenient to use, their inadequate use of the available clinical data is a major weakness. In this study, we compared performance of the International Prognostic Index (IPI) and its variations (R-IPI and NCCN-IPI) to a Cox proportional hazards (CPH) model using the same covariates in nondichotomized form. All models were tested in 4863 newly diagnosed DLBCL patients from population-based Nordic registers. The CPH model led to a substantial increase in predictive accuracy as compared to conventional prognostic scores when evaluated by the area under the curve and other relevant tests. Furthermore, the generation of patient-specific survival curves rather than assigning patients to one of few predefined risk groups is a relevant step toward personalized management and treatment. A test-version is available on lymphomapredictor.org., (© 2017 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

The use of routine imaging for patients with classical Hodgkin lymphoma (HL) in complete remission (CR) is controversial. In a population-based study, we examined the post-remission survival of Danish and Swedish HL patients for whom follow-up practices were different. Follow-up in Denmark included routine imaging, usually for a minimum of 2 years, whereas clinical follow-up without routine imaging was standard in Sweden. A total of 317 Danish and 454 Swedish comparable HL patients aged 18-65 years, diagnosed in the period 2007-2012 and having achieved CR following ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine)/BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisone) therapy, were included in the study. The cumulative progression rates in the first 2 years were 4% (95% confidence interval [CI] 1-7) for patients with stage I-II disease vs. 12% (95% CI 6-18) for patients with stage III-IV disease. An imaging-based follow-up practice was not associated with a better post-remission survival in general (P = 0·2) or in stage-specific subgroups (P = 0·5 for I-II and P = 0·4 for III-IV). Age ≥45 years was the only independent adverse prognostic factor for survival. In conclusion, relapse of HL patients with CR is infrequent and systematic use of routine imaging in these patients does not improve post-remission survival. The present study supports clinical follow-up without routine imaging, as encouraged by the recent Lugano classification., (© 2016 John Wiley & Sons Ltd.)

Purpose: Routine imaging for diffuse large B-cell lymphoma (DLBCL) in first complete remission (CR) is controversial and plays a limited role in detecting relapse. This population-based study compared the survival of Danish and Swedish patients with DLBCL for whom traditions for routine imaging have been different., Patients and Methods: Patients from the Danish and Swedish lymphoma registries were included according to the following criteria: newly diagnosed DLBCL from 2007 to 2012, age 18 to 65 years, and CR after R-CHOP/CHOEP. Follow-up for Swedish patients included symptom assessment, clinical examinations, and blood tests at 3- to 4-month intervals for 2 years, with longer intervals later in follow-up. Imaging was only recommended when relapse was clinically suspected. Follow-up for Danish patients was similar but included routine imaging (usually computed tomography every 6 months for 2 years)., Results: Danish (n = 525) and Swedish (n = 696) patients with DLBCL had comparable baseline characteristics. Cumulative 2-year progression rate after CR was 6% (95% CI, 4 to 9) for International Prognostic Index (IPI) ≤ 2 versus 21% (95% CI, 13 to 28) for IPI > 2. Age > 60 years (hazard ratio [HR], 2.3; 95% CI, 1.6 to 3.4), elevated lactate dehydrogenase (HR, 2.3; 95% CI, 1.4 to 3.8), B symptoms (HR, 1.7; 95% CI, 1.1 to 2.5), and Eastern Cooperative Oncology Group performance status ≥ 2 (HR, 1.8; 95% CI, 1.0 to 3.0) were associated with worse post-CR survival. Imaging-based follow-up strategy had no impact on survival, neither for all patients nor for IPI-specific subgroups., Conclusion: DLBCL relapse after first CR is infrequent, and the widespread use of routine imaging in Denmark did not translate into better survival. This favors follow-up without routine imaging and, more generally, a shift of focus from relapse detection to improved survivorship., (© 2015 by American Society of Clinical Oncology.)

Trends in Hodgkin lymphoma (HL) survival among patients treated outside of clinical trials provide real-world benchmark estimates of prognosis and help identify patient subgroups for targeted trials. In a Swedish population-based cohort of 1947 HL patients diagnosed in 1992-2009 at ages 18-59 years, we estimated relative survival (RS), cure proportions (CP), and median survival times using flexible parametric cure models. Overall, the CP was 89% (95% CI: 0.87-0.91) and median survival of the uncured was 4.6 years (95% CI: 3.0-6.3). For patients aged 18-50 years diagnosed after the year 2000, CP was high and stable, whereas for patients of 50-59 years, cure was not reached. The survival of relapse-free patients was similar to that of the general population (RS5-year : 0.99; 95% CI: 0.98-0.99, RS15-year : 0.95; 95% CI: 0.92-0.97). The excess mortality of relapsing patients was 19 times (95% CI: 12-31) that of relapse-free patients. Despite modern treatments, patients with adverse prognostic factors (e.g., advanced stage) still had markedly worse outcomes [CP stage: IIIB 0.82 (95% CI: 0.73-0.89); CP stage: IVB 0.72, (95% CI: 0.60-0.81)] and patients with international prognostic score (IPS) ≥3 had 2.7 times higher excess mortality (95% CI: 1.0-7.0, p = 0.04) than patients with IPS <3. High-risk patients selected for 6-8 courses of BEACOPP (bleomycin, etoposide, doxorubicin, cyclofosphamide, vincristine, procarbazine, prednisone)-chemotherapy had a 15-year relative survival of 87%, (95% CI: 0.80-0.92), whereas the corresponding estimate for patients selected for 6-8 courses of ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) was 93% (95% CI: 0.88-0.97). These population-based results indicate limited fatal side-effects in the 15-year perspective with contemporary treatments, while the unmet need of effective relapse treatment remains of concern. BEACOPP-chemotherapy was still not sufficient in high-risk HL patients., (© 2015 Wiley Periodicals, Inc.)

Background: Diffuse large B-cell lymphoma (DLBCL) is a heterogeneous group of B-cell lymphomas. Five clinical adverse risk factors are merged into the International Prognostic Index (IPI), which is the major tool for prognostication. In contrast to Hodgkin's lymphoma, gender is not considered as an adverse risk factor for DLBCL patients. As we clinically had observed a very good survival rate in young female patients we hypothesised that there was a gender difference in survival due to the hormonal status of the patient., Material and Methods: We conducted a registry-based retrospective cohort study of all Swedish DLBCL patients diagnosed between 2000 and 2013, to evaluate the impact of gender for survival from DLBCL., Results: In total, 7166 patients were included for further analysis. No survival difference was found between the genders when the entire population was analysed. However, analysis of 880 young patients of pre-menopausal age (i.e. 52 years) revealed that women had a longer survival compared to men of the same age group (p=0.007). This was not found for patients older than menopausal age. In a relative survival multifactorial model adjusted for stage, ECOG performance status, serum lactate dehydrogenase and two or more extranodal sites, male gender was found to be an adverse risk factor for patients younger than 52 years (RR 1.51, 95% CI 1.14-1.88), but not for older patients (RR 0.99, 95% CI 0.89-1.10)., Conclusion: This is one of the largest population-based studies of DLBCL presented to date. Most interestingly, we found male gender to be a significant adverse risk factor compared to fertile women whereas we found no survival differences between genders in the older sub-population.

Background: For Diffuse large B-cell lymphoma (DLBCL), the International Prognostic Index is the major tool for prognostication and considers an age above 60 years as a risk factor. However, there are several indications that increasing age is associated with more biological complexity, resulting in differences in DLBCL biology depending on age., Methods: We conducted a registry-based retrospective cohort study of all Swedish DLBCL patients diagnosed 2000-2013, to evaluate the importance of age at diagnosis for survival of DLBCL patients., Results: In total, 7166 patients were included for further analysis. Survival declined for every 10-year age group and every age group above the age of 39 had a statistically decreased survival compared to the reference group of 20-29 years. In an analysis of relative survival, and in a multifactorial model adjusted for stage, ECOG performance status, serum lactate dehydrogenase and involvement of extranodal sites, each age group above age 39 had a significantly higher risk ratio (p=0.01) compared to the reference group., Conclusion: This is one of the largest population-based studies of DLBCL published to date. In this study, age persisted as a significant adverse risk factor for patients as young as 40 years, even after adjustment for other risk factors.

Peripheral T-cell lymphomas (PTCLs) are rare lymphomas with mostly poor outcome with current treatment. The addition of etoposide to cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) and upfront consolidation with autologous stem cell transplantation (auto-SCT) have shown promising results but have never been tested in randomized trials. As a complement to retrospective analyses of clinical trials, we aimed at analyzing prognostic factors and outcome in an unselected, population-based cohort. Through the Swedish Lymphoma Registry, we identified 755 PTCL patients diagnosed during a 10-year period. In addition to International Prognostic Index factors, male gender was associated with an adverse overall survival (OS) (hazard ratio [HR], 1.28; P = .011) and progression-free survival (PFS) (HR, 1.26; P = .014). In an intention-to-treat analysis in 252 nodal PTCL and enteropathy-associated T-cell lymphoma patients (excluding anaplastic lymphoma kinase-positive anaplastic large cell lymphoma), upfront auto-SCT was associated with a superior OS (HR, 0.58; P = .004) and PFS (HR, 0.56; P = .002) compared with patients treated without auto-SCT. The addition of etoposide to CHOP resulted in superior PFS in patients ≤60 years (HR, 0.49; P = .008). This study is the largest population-based PTCL cohort reported so far and provides important information on outcome in PTCL outside the setting of clinical trials., (© 2014 by The American Society of Hematology.)

Our aim was to describe a large population-based cohort of diffuse large B-cell lymphoma (DLBCL) during the last decade, evaluating possible improvement in survival and to identify subgroups in need of novel treatment strategies. The study population encompassed all patients diagnosed with DLBCL in Sweden from 2000 through 2010. Altogether 5349 patients were identified. There was no increase in incidence for females, but for males there was an estimated yearly increase in incidence by 0.019 per 10 000. When adjusted for age and gender, the improvement in overall survival for the whole group was estimated at 4.5% per year, most prominent in the age group 60-78 years, and in patients with good performance status. In this large dataset, we were able to detect a clear improvement in overall survival in DLBCL, although restricted to specific prognostic subgroups, and to identify specific disease presentations that significantly affect overall survival.

Background: T-cell lymphoblastic lymphoma (T-LBL) is a rare neoplasm of precursor lymphoblast origin, for which there is no standard treatment for adults. Results of current treatment strategies in selected populations do exist but are largely unreported for unselected series. Here, we aimed to investigate treatment outcome in a population-based cohort., Material and Methods: Patients were identified through the Swedish Lymphoma Registry and data was retrospectively collected for all adult (≥ 18 years) Swedish T-LBL patients diagnosed during 2000-2009., Results: A total of 39 patients with median age 40 years (range 18-78) were identified with females being significantly older than males (median age 66 vs. 37, p = 0.027). The five-year overall survival for all patients was 42%. Female gender was associated with shorter survival also when adjusted for treatment strategy and age [hazard ratio (HR) 4.29; p = 0.002]. Thirty patients received intensive chemotherapy, otherwise used for treatment of acute lymphoblastic leukemia (ALL), which resulted in an overall response rate of 97% and a five-year progression-free survival (PFS) of 49%. In this group only CNS involvement at diagnosis predicted shorter PFS (HR 13.3; p = 0.03). Among patients treated with hyper-CVAD the addition of mediastinal irradiation resulted in prolonged time to progression compared to patients receiving only chemotherapy (p = 0.047). The major reason for treatment failure was relapse and in this series 18-fluoro-deoxyglucose positron emission tomography (PET) did not predict this risk., Conclusion: This population-based study indicates that all fit T-LBL patients should be considered for intensive treatment. Our results also suggest a beneficial effect of mediastinal irradiation in combination with hyper-CVAD treatment. Relapsing patients have a dismal outcome irrespective of salvage treatment.

Background: Standard treatment of adult Burkitt lymphoma is not defined due to the lack of randomised trials. In this situation, population-based data may represent a useful contribution in order to identify an optimal treatment strategy., Patients and Methods: The aims of this study were to investigate the outcome for adult HIV-negative BL with different chemotherapy regimens, and to assess possible improvement within the time frame of the study. The study population was identified through the Swedish and Danish lymphoma registries 2000-2009., Results: A total of 258 patients were identified. Since 2000, overall survival (OS) improved significantly only for younger patients (<65 years). Intensive regimens such as the Berlin-Frankfurt-Münster, hyper-fractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone (hyper-CVAD) and cyclophosphamide, vincristine, doxorubicin, methotrexate, ifosfamide, etoposide, and cytarabine (CODOX-M/IVAC) were associated with a favourable 2-year OS of 82%, 83%, and 69%, respectively. The low-intensive CHOP/CHOEP regimens achieved a 2-year OS of 38.8%, confirming their inadequacy for the treatment of BL. In a multivariate analysis, rituximab was not significantly associated with improved OS., Conclusions: In this population-based retrospective series of adult BL, intensive chemotherapy regimens were associated with favourable outcome. The impact of the addition of rituximab remains uncertain and warrants further investigation.

Burkitt lymphoma (BL) is a rare neoplasm constituting 1-2% of adult lymphomas. Our aims in this study were to establish prognostic factors for overall survival in adult BL and evaluate the efficacy of different chemotherapy regimens in a population based setting. The study population was collected from the Swedish Lymphoma Registry 2000-2010. During this period, 156 adult patients with BL were identified. In multivariate analysis, age and performance status (PS) were significant adverse prognostic factors for overall survival. A modified prognostic index, based on: age >40 years, PS >1, and lactate dehydrogenase > upper limit of normal (ULN) was proposed. Patients with a score of 0-1, 2, and 3 were found to have a 2-year survival of 91.2%, 58.4%, and 27.5%, respectively. High-intensity regimens were associated with more favorable overall survival. Rituximab addition was not significantly associated with improvement in survival. A modified prognostic index may be valuable for adult BL, as proposed.

Abstract In recent years, more intensive chemotherapy regimens for mantle cell lymphoma (MCL) have been associated with prolongation of survival. In this study, our aim was to investigate prognostic factors and evaluate improvement in survival in MCL on a population level. The cohort included all patients diagnosed with MCL from 1 January 2000 to 31 March 2010 in the Swedish Lymphoma Registry. At total of 785 patients with MCL were identified. Age, performance status, and B-symptoms were significant prognostic factors for overall survival (OS) in multivariate analysis. In addition, OS was markedly improved (hazard ratio 0.8, 95% confidence interval 0.7-0.9) for patients diagnosed during the latest time period, 2006-2010, also when corrected for prognostic factors as above. Estimated OS at 3 years was 62%, compared to 47% for patients diagnosed earlier (p < 0.01). The reasons for this dramatic improvement in OS are not yet clear, but may be due to the introduction of specific and more potent therapeutic regimens.

Purpose: From 1995 to 2003, 603 adult patients from Sweden and Norway with metastatic testicular nonseminomatous germ cell tumor (NSGCT) were included prospectively in a population-based protocol with strict guidelines for staging, treatment, and follow-up. Patients with extragonadal primary tumor or previous treatment for contralateral testicular tumor were excluded. The basic strategy was to individualize treatment according to initial tumor marker response., Methods: Initial treatment for all patients was two courses of standard bleomycin, etoposide, and cisplatin (BEP), with tumor markers analyzed weekly. Good response was defined as a half-life (t(1/2)) for α-fetoprotein (AFP) of ≤ 7 days and/or for β-human chorionic gonadotropin (β-HCG) of ≤ 3 days. Patients with prolonged marker t(1/2) (ie, poor response) received intensification with addition of ifosfamide (BEP-if/PEI) in step 1. If poor response continued, the treatment was intensified with high-dose chemotherapy with stem-cell rescue as step 2., Results: Overall, 99% of all patients with metastatic testicular NSGCT in the population were included in the protocol. Median follow-up was 8.2 years. Seventy-seven percent of the patients were treated with BEP alone; 18% received intensification step 1%, and 5% received intensification step 2. Grouped according to International Germ Cell Consensus Classification, 10-year overall survival was 94.7% in good-prognosis patients, 90.0% in intermediate-prognosis patients, and 67.4% in poor-prognosis patients., Conclusion: With detailed treatment protocols and a dedicated collaborative group of specialists, treatment results comparable to those reported from large single institutions can be achieved at national level. With the treatment principles used in Swedish-Norwegian Testicular Cancer Group study SWENOTECA IV, the survival of intermediate-prognosis patients is remarkable and close to that of good-prognosis patients.

Patients with febrile urinary tract infections with (80 patients) or without (88 patients) positive blood cultures were reviewed. Eighty-nine percent of the infections were community acquired. The bacteremic patients were older, Escherichia coli was the most commonly found organism in both groups. The most important finding in this study was increased frequency of resistance to three common urinary tract antibiotics (ampicillin, cephalothin and trimethoprim-sulfamethoxazole) in E. coli from patients with non-bacteremic compared with bacteremic infections. Complications occurred in 28 bacteremic and in three non-bacteremic patients. Six patients died, all with bacteremia. The significantly higher temperature at admittance among patients with gram-negative versus gram-positive bacteremic infection possibly reflects an effect by endotoxin.