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Incidence and outco...
Incidence and outcome of acquired aplastic anemia: real-world data from patients diagnosed in Sweden from 2000-2011
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Vaht, Krista (författare)
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Goransson, Magnus (författare)
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Carlson, Kristina (författare)
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Isaksson, Cecilia (författare)
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Lenhoff, Stig (författare)
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Sandstedt, Anna (författare)
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Uggla, Bertil (författare)
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Winiarski, Jacek (författare)
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Ljungman, Per (författare)
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Brune, Mats (författare)
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Andersson, Per-Ola (författare)
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- FERRATA STORTI FOUNDATION 2017
- 2017
- Engelska.
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Ingår i: Haematologica. - 0390-6078. ; 102:10, 1683-1690
Abstract
Ämnesord
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- A plastic anemia is a rare life-threatening disease. However, since the introduction of immunosuppressive therapy and allogeneic stem cell transplantation, the outcome has improved considerably, and the 5-year survival is reported to be 70-80% in selected patient cohorts. Yet, contemporary population-based data on incidence and survival are lacking. We performed a national retrospective study to determine the incidence, treatment, and survival of patients with aplastic anemia diagnosed in Sweden from 2000-2011. Patients were included via the National Patient Registry, and diagnosed according to the Camitta criteria. In total, 257 confirmed cases were identified, with an overall incidence of 2.35 (95% CI: 2.06-2.64) cases per million inhabitants per year. Median age was 60 years (range: 2-92), and median follow up was 76 (0-193) months. Primary treatments included immunosuppressive therapy (63%), allogenic stem cell transplantation (10%), or single-agent cyclosporine/no specific therapy (27%). The 5-year survival was 90.7% in patients aged 0-18 years, 90.5% in patients aged 19-39 years, 70.7% in patients aged 40-59 years, and 38.1% in patients aged amp;gt;= 60 years. Multivariate analysis showed that age (both 40-59 and amp;gt;= 60 age groups), very severe aplastic anemia and single-agent cyclosporine/no specific therapy were independent risk factors for inferior survival. In conclusion, younger aplastic anemia patients experience a very good long-term survival, while that of patients amp;gt;= 60 years in particular remains poor. Apparently, the challenge today is to improve the management of older aplastic anemia patients, and prospective studies to address this medical need are warranted.
Ämnesord
- Medical and Health Sciences (hsv)
- Clinical Medicine (hsv)
- Hematology (hsv)
- Medicin och hälsovetenskap (hsv)
- Klinisk medicin (hsv)
- Hematologi (hsv)
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Vaht, Krista
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Goransson, Magnu ...
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Carlson, Kristin ...
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Isaksson, Cecili ...
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Lenhoff, Stig
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Sandstedt, Anna
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visa fler...
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Uggla, Bertil
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Winiarski, Jacek
-
Ljungman, Per
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Brune, Mats
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Andersson, Per-O ...
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visa färre...
- Av lärosätet
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