.
Objectives. To compare the incidence and survival of acute de novo leukaemias with particular reference to political/socio‐economic and environmental factors in two neighbouring countries over the three 5‐year periods (1982–1996).
Patients. The present report covers only patients diagnosed when aged ≥65 years.
Setting. A well‐defined area of Sweden, the so‐called Western Swedish Health Care Region and Estonia. Population‐wise, the western Swedish Region and Estonia are very similar; area‐wise they are also well comparable.
Results. The number of acute de novo leukaemias was quite dissimilar in the two countries (Estonia, n = 137, Sweden, n = 354). The age standardized incidence rates regarding the total number of acute de novo leukaemias was 5.31 per 100 000 inhabitants/year for Estonia and 7.99 for Sweden, this difference being statistically significant. However, the difference was merely attributable to incidence rates as regards acute myeloblastic leukaemias (AML); on the contrary, differences as regards acute lymphoblastic leukaemias (ALL) and non‐classifiable, undifferentiated or biphenotypic acute leukaemias (uAL) were negligible. The relative survival for the total material of patients was significantly higher for Swedish when compared with Estonian patients (P < 0.001). Thus, the relative survival for the total material of patients aged ≥65 years in Estonia at 1 year was 8.5% and at 3 years 3.5% respectively. The corresponding figures for the Swedish patients were considerably higher, 22.7 and 7.7% respectively. This difference, however, applied only for patients with AML (P < 0.001), whereas the results for patients with ALL and uAL were equally dismal.
Conclusion. The results clearly reflect how political and socio‐economic factors may influence the survival of acute leukemia patients in two neighbouring countries.
In the present work the incidence and survival of acute de novo leukaemias in two neighbouring countries, were studied retrospectively over three 5-year periods, 1982-1996. The aim was to compare the above variables, particularly with respect to political/socio-economic and environmental factors, in a well defined area of Sweden, the so-called Western Swedish Health Care Region, with Estonia. Population-wise the Western Swedish Region and Estonia are very similar; area-wise they are also well comparable. The present report covers only patients diagnosed between the ages of 16-64 years. The number of acute de novo leukaemias in the two regions was quite similar (Western Sweden n = 282 and Estonia n = 237). The age standardized incidence rate regarding total acute de novo leukaemias was slightly lower in Estonia than in Western Sweden (1.49/100,000 inhabitants/year for Estonia and 1.76 for Sweden, respectively), the difference being not statistically significant. However, the survival data for the two countries were highly different (P < 0.001). Thus, the relative survival for the total group of patients aged 16-64 years in Estonia at 1 year was 20.7% and at 5 years 3.6%, respectively. The corresponding figures for the Swedish patients were considerably higher, 65.2 and 29.4%, respectively. Further, the 5 year survival significantly (P < 0.05) increased for the Swedish patients over the 3 consecutive 5-year periods. No such improvement was recorded for the Estonian patients.
Objectives Acquired von Willebrand syndrome (AVWS) is a rare and frequently underdiagnosed bleeding disorder with an unknown prevalence. The diagnosis of AVWS is made based on laboratory investigations and the presence of clinical symptoms. Evaluation and management of affected patients are complex due to the need for multiple laboratory assays.
Materials and Methods Here, we describe the clinical and laboratory data of seven patients with a diagnosis of AVWS. All patients met the criteria for AVWS based on laboratory findings, bleeding symptoms, and the absence of any previous history of a bleeding disorder.
Results In all cases, the laboratory findings, lack of bleeding anamnesis, and family history suggested the presence of AVWS. Von Willebrand factor multimeric analysis showed decreased high-molecular weight (HMW) multimers in six cases. Patients with lower HMW multimers experienced more severe bleeding complications.
Conclusions The diagnosis of AVWS is complex and requires extensive laboratory evaluation. Interdisciplinary collaboration and complex laboratory evaluations are of paramount importance for the early recognition of AVWS and optimal AVWS diagnosis as well as successful clinical management.
Background: In a recent retrospective study, we investigated the incidence and survival of de novo acute leukemia (AL) patients aged 16–64 years over three 5-year periods (1982–1996) in Estonia and in the Western Swedish Health Care Region. The incidence rates were similar in the two countries, but the survival data were highly different. Thus, relative survival at 5 years for de novo AL patients in Estonia was virtually negligible, whereas the corresponding figures for the Swedish patients increased from 20.3 to 38.9% during the study period. Aim: To prospectively compare the results for incidence and outcome of de novo AL between the two countries during 1997–2001. Results: Incidence rates for de novo AL were lower in Estonia than in western Sweden but not significantly so. However, the survival for de novo AL patients in Estonia had improved considerably, with the relative survival at 5 years being 16.4%; such improvement was particularly seen in acute myeloid leukemia patients. For the Swedish patients, no change in survival was recorded. Conclusion: In Estonia, a remarkable improvement in outcome for young de novo AL patients was seen after 1996. Nevertheless, relative survival for the Estonian patients had still not reached the levels found in the Swedish cohort.
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