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ABSTRACT: Reported survival in patients with myeloproliferative neoplasms (MPNs) shows great variation. Patients with primary myelofibrosis (PMF) have substantially reduced life expectancy, whereas patients with polycythemia vera (PV) and essential thrombocythemia (ET) have moderately reduced survival in most, but not all, studies. We conducted a large population-based study to establish patterns of survival in more than 9,000 patients with MPNs.
We identified 9,384 patients with MPNs (from the Swedish Cancer Register) diagnosed from 1973 to 2008 (divided into four calendar periods) with follow-up to 2009. Relative survival ratios (RSRs) and excess mortality rate ratios were computed as measures of survival.
Patient survival was considerably lower in all MPN subtypes compared with expected survival in the general population, reflected in 10-year RSRs of 0.64 (95% CI, 0.62 to 0.67) in patients with PV, 0.68 (95% CI, 0.64 to 0.71) in those with ET, and 0.21 (95% CI, 0.18 to 0.25) in those with PMF. Excess mortality was observed in patients with any MPN subtype during all four calendar periods (P < .001). Survival improved significantly over time (P < .001); however, the improvement was less pronounced after the year 2000 and was confined to patients with PV and ET.
We found patients with any MPN subtype to have significantly reduced life expectancy compared with the general population. The improvement over time is most likely explained by better overall clinical management of patients with MPN. The decreased life expectancy even in the most recent calendar period emphasizes the need for new treatment options for these patients.
Journal of Clinical Oncology 07/2012; 30(24):2995-3001. · 18.37 Impact Factor
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Expert Review of Anti-infective Therapy 03/2012; 12(3):291-4. · 2.65 Impact Factor
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Leukemia & lymphoma 04/2011; 52(5):734-5. · 2.40 Impact Factor
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ABSTRACT: Patients with multiple myeloma are at an increased risk of venous thromboembolism (VTE), especially when treated with the immunomodulatory drugs, thalidomide and lenalidomide, in combination with dexamethasone and/or chemotherapy. Several studies have shown that patients with multiple myeloma precursor disease (monoclonal gammopathy of undetermined significance [MGUS]) also have a higher risk of thrombosis compared to the general population. The underlying mechanisms for the hypercoagulable state are not completely understood. In this review, we discuss risk factors for thrombosis in multiple myeloma, as well as prophylactic strategies, the evidence for thrombosis among patients with MGUS, and proposed mechanisms for the hypercoagulability.
Seminars in Hematology 01/2011; 48(1):46-54. · 3.99 Impact Factor
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Sigurdur Yngvi Kristinsson
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ABSTRACT: Patients with multiple myeloma (MM) are at an increased risk of venous and arterial thrombosis. The pathogenesis remains unclear, but probably involves several factors such as activation of procoagulant factors, acquired activated protein C resistance, and inflammation. In addition to general risk factors for venous thromboembolism, such as older age, immobility, surgery, and inherited thrombophilia, there are some MM-specific and treatment-related factors that contribute to the increased risk. The risk for venous thromboembolism is high when patients are treated with thalidomide or lenalidomide in combination with dexamethasone or multi-agent chemotherapy. Thromboprophylaxis should be given in these settings. Which agent is the most appropriate is a matter of debate, but aspirin, low-molecular-weight heparin, and warfarin all seem to be effective. This review discusses risk factors for thromboembolism in MM and general, disease-specific and treatment-related mechanisms for thrombosis. Recommendations for thromboprophylaxis are described and treatment choices for venous thrombosis in MM patients are reviewed.
Hematology 01/2010; 2010:437-44. · 1.49 Impact Factor
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ABSTRACT: Large age-dependent differences in temporal trends in 1- and 5-year relative survival have been observed in patients with acute myeloid leukaemia (AML) in Sweden. This investigation used an alternative approach to studying patient survival that simultaneously estimated the proportion of patients cured from their cancer and the survival of the 'uncured'. We conducted a population-based study including 6439 AML patients aged 19-80 years in Sweden between 1973 and 2001. Mixture cure models were estimated, with age at diagnosis categorised (19-40, 41-60, 61-70 and 71-80) and year of diagnosis modelled using splines. In 1975 the cure proportion was < or =6% in all age groups and the median survival time for 'uncured' patients was <0.5 years. In 2000 the cure proportion was 68% (95% confidence interval 56-77%) in the youngest group, and 32% (25-39%), 8% (3-21%), and 4% (2-8%) in the other groups, respectively. The median survival times for 'uncured' were 0.74 (0.43-1.26), 0.71 (0.53-0.97), 0.69 (0.51-0.95) and 0.37 (0.31-0.44) years, respectively. A dramatic improvement in the cure proportion was seen in younger patients, whereas improvement in older ages was mainly within the survival of the 'uncured'. This novel approach of analysing survival data could be a valuable tool for physicians, patients, health care planners and health economists.
British Journal of Haematology 12/2009; 148(6):918-24. · 4.94 Impact Factor
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ABSTRACT: An association between socioeconomic status (SES) and survival in acute myeloid leukemia (AML) and multiple myeloma (MM) has not been established in developed countries. We assessed the impact of SES on survival in two large population-based cohorts of AML and MM patients diagnosed in Sweden 1973 to 2005.
The relative risk of death (all cause and cause specific) in relation to SES was estimated using Cox's proportional hazards regression. We also conducted analyses stratified by calendar periods (1973 to 1979, 1980 to 1989, 1990 to 1999, and 2000 to 2005).
We identified a total of 9,165 and 14,744 patients with AML and MM, respectively. Overall, higher white-collar workers had a lower mortality than other SES groups for both AML (P = .005) and MM (P < .005). In AML patients, a consistently higher overall mortality was observed in blue-collar workers compared with higher white-collar workers in the last three periods (hazard ratio [HR], 1.26; 95% CI, 1.05 to 1.51; HR, 1.23; 95% CI, 1.05 to 1.45; HR, 1.28; 95% CI, 1.04 to 1.57, respectively). In MM, no difference was observed in the first two calendar periods. However, in 1990 to 1999, self-employed (HR, 1.18; 95% CI, 1.02 to 1.37), blue-collar workers (HR, 1.18; 95% CI, 1.04 to 1.32), and retired (HR, 1.45; 95% CI, 1.16 to 1.80) had a higher mortality compared to higher white-collar workers. In 2000 to 2005, blue-collar workers had a higher mortality (HR, 1.31; 95% CI, 1.07 to 1.60) compared with higher white-collar workers.
SES was significantly associated with survival in both AML and MM. Most conspicuously, a lower mortality was observed among the highest SES group during more recent calendar periods. Differences in management, comorbidity, and lifestyle, are likely factors to explain these findings.
Journal of Clinical Oncology 04/2009; 27(12):2073-80. · 18.37 Impact Factor
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ABSTRACT: We evaluated survival patterns for all registered acute myeloid leukemia (AML) patients diagnosed in Sweden in 1973 to 2005 (N = 9729; median age, 69 years). Patients were categorized into 6 age groups and 4 calendar periods (1973-1980, 1981-1988, 1989-1996, and 1997-2005). Relative survival ratios were computed as measures of patient survival. One-year survival improved over time in all age groups, whereas 5- and 10-year survival improved in all age groups, except for patients 80+ years. The 5-year relative survival ratios in the last calendar period were 0.65, 0.58, 0.36, 0.15, 0.05, and 0.01 for the age groups 0 to 18, 19 to 40, 41 to 60, 61 to 70, 71 to 80, and 80+ years, respectively. Intensified chemotherapy, a continuous improvement in supportive care, and allogeneic stem cell transplantation are probably the most important factors contributing to this finding. In contrast, there was no improvement in survival in AML patients with a prior diagnosis of a myelodysplastic syndrome during 1993 to 2005 (n = 219). In conclusion, AML survival has improved during the last decades. However, the majority of AML patients die of their disease and age remains an important predictor of prognosis. New effective agents with a more favorable toxicity profile are needed to improve survival, particularly in the elderly.
Blood 12/2008; 113(16):3666-72. · 9.90 Impact Factor
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ABSTRACT: To define patterns of survival among all multiple myeloma (MM) patients diagnosed in Sweden during a 30-year period.
A total of 14,381 MM patients (7,643 males; 6,738 females) were diagnosed in Sweden from 1973 to 2003 (median age, 69.9 years; range 19 to 101 years). Patients were categorized into six age categories and four calendar periods (1973 to 1979, 1980 to 1986, 1987 to 1993, and 1994 to 2003). We computed relative survival ratios (RSRs) as measures of patient survival.
One-year survival improved (P < .001) over time in all age groups and RSRs were 0.73, 0.78, 0.80, and 0.82 for the four calendar periods; however, improvement in 5-year (P < .001) and 10-year (P < .001) RSR was restricted to patients younger than 70 years and younger than 60 years, respectively. For the first time, in analyses restricted to MM patients diagnosed at age younger than 60 years, we found a 29% (P < .001) reduced 10-year mortality in the last calendar period (1994 to 2003) compared with the preceding calendar period (1987 to 1993). Females with MM had a 3% (P = .024) lower excess mortality than males.
One-year MM survival has increased for all age groups during the last decades; 5-year and 10-year MM survival has increased in younger patients (younger than 60 to 70 years). High-dose melphalan with subsequent autologous stem-cell transplantation, thalidomide, and a continuous improvement in supportive care measures are probably the most important factors contributing to this finding. New effective agents with a more favorable toxicity profile are needed to improve survival further, particularly in the elderly.
Journal of Clinical Oncology 06/2007; 25(15):1993-9. · 18.37 Impact Factor
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ABSTRACT: Hairy cell leukemia (HCL) is a rare B-cell lymphoproliferative disorder. Previous epidemiological studies have mainly focused on cases derived from single institutions or from localized cancer registries. This is the first study in which all cases diagnosed nationwide over a long period of time in a well defined population are analysed. We report the epidemiology of all HCL patients in Iceland, their clinical characteristics, treatment and follow-up.
: All patients diagnosed with HCL in Iceland over a 20 year period, were included in this study. Data was collected retrospectively.
Sixteen patients, 13 males and three females were diagnosed with HCL in Iceland from 1981-2000, giving a mean incidence of 4.7/million/year (95% CI: 2.7-7.6) in the population 20 years and older. Eleven patients were treated with a purine analogue, 10 of whom achieved CR. One other patient obtained CR following splenectomy and IFN, giving a total CR rate of 69%. Three other patients (19%) obtained PR, giving a total response rate of 88%. One patient had a variant of HCL and did not respond to any therapy and one patient died of sepsis before any chemotherapy could be given. Six patients with HCL have died, one from complications of HCL. Three patients developed a second malignancy (19%).
The mean incidence of HCL in Iceland is 4.7/million/year. This is slighty higher than the reported incidence in England and Wales, although not significantly higher. The incidence is based on a nationwide information from a well defined stable and racially homogenous island population. Other results are in accordance with previously published studies.
The Hematology Journal 02/2002; 3(3):145-7. · 1.86 Impact Factor
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ABSTRACT: In the twenty-first century, melphalan-prednisone can no longer be regarded as the standard treatment of multiple myeloma patients not eligible for high-dose melphalan followed by autologous stem cell transplantation. The introduction of thalidomide, lenalidomide, and bortezomib has improved the arsenal of therapeutic options in multiple myeloma. Indeed, randomized studies have shown that melphalan-prednisone-thalidomide and melphalan-prednisone-bortezomib are superior to melphalan-prednisone alone. In addition, other combinations, including lenalidomide, are under study. In this review, we discuss the role of novel therapies in multiple myeloma in elderly multiple myeloma patients. Important aspects, such as toxicity and the role of prognostic factors, are also addressed.
The Cancer Journal 15(6):473-8. · 3.26 Impact Factor
