Incidence and outcome of acquired aplastic anemia: Real-world data from patients diagnosed in Sweden from 2000–2011

Forskningsoutput: TidskriftsbidragArtikel i vetenskaplig tidskrift

Standard

Incidence and outcome of acquired aplastic anemia : Real-world data from patients diagnosed in Sweden from 2000–2011. / Vaht, Krista; Göransson, Magnus; Carlson, Kristina; Isaksson, Cecilia; Lenhoff, Stig; Sandstedt, Anna; Uggla, Bertil; Winiarski, Jacek; Ljungman, Per; Brune, Mats; Andersson, Per Ola.

I: Haematologica, Vol. 102, Nr. 10, 30.09.2017, s. 1683-1690.

Forskningsoutput: TidskriftsbidragArtikel i vetenskaplig tidskrift

Harvard

Vaht, K, Göransson, M, Carlson, K, Isaksson, C, Lenhoff, S, Sandstedt, A, Uggla, B, Winiarski, J, Ljungman, P, Brune, M & Andersson, PO 2017, 'Incidence and outcome of acquired aplastic anemia: Real-world data from patients diagnosed in Sweden from 2000–2011', Haematologica, vol. 102, nr. 10, s. 1683-1690. https://doi.org/10.3324/haematol.2017.169862

APA

Vaht, K., Göransson, M., Carlson, K., Isaksson, C., Lenhoff, S., Sandstedt, A., ... Andersson, P. O. (2017). Incidence and outcome of acquired aplastic anemia: Real-world data from patients diagnosed in Sweden from 2000–2011. Haematologica, 102(10), 1683-1690. https://doi.org/10.3324/haematol.2017.169862

CBE

Vaht K, Göransson M, Carlson K, Isaksson C, Lenhoff S, Sandstedt A, Uggla B, Winiarski J, Ljungman P, Brune M, Andersson PO. 2017. Incidence and outcome of acquired aplastic anemia: Real-world data from patients diagnosed in Sweden from 2000–2011. Haematologica. 102(10):1683-1690. https://doi.org/10.3324/haematol.2017.169862

MLA

Vancouver

Author

Vaht, Krista ; Göransson, Magnus ; Carlson, Kristina ; Isaksson, Cecilia ; Lenhoff, Stig ; Sandstedt, Anna ; Uggla, Bertil ; Winiarski, Jacek ; Ljungman, Per ; Brune, Mats ; Andersson, Per Ola. / Incidence and outcome of acquired aplastic anemia : Real-world data from patients diagnosed in Sweden from 2000–2011. I: Haematologica. 2017 ; Vol. 102, Nr. 10. s. 1683-1690.

RIS

TY - JOUR

T1 - Incidence and outcome of acquired aplastic anemia

T2 - Haematologica-The Hematology Journal

AU - Vaht, Krista

AU - Göransson, Magnus

AU - Carlson, Kristina

AU - Isaksson, Cecilia

AU - Lenhoff, Stig

AU - Sandstedt, Anna

AU - Uggla, Bertil

AU - Winiarski, Jacek

AU - Ljungman, Per

AU - Brune, Mats

AU - Andersson, Per Ola

PY - 2017/9/30

Y1 - 2017/9/30

N2 - Aplastic 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 ≥60 years. Multivariate analysis showed that age (both 40-59 and ≥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 ≥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.

AB - Aplastic 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 ≥60 years. Multivariate analysis showed that age (both 40-59 and ≥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 ≥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.

UR - http://www.scopus.com/inward/record.url?scp=85030308835&partnerID=8YFLogxK

U2 - 10.3324/haematol.2017.169862

DO - 10.3324/haematol.2017.169862

M3 - Article

VL - 102

SP - 1683

EP - 1690

JO - Haematologica-The Hematology Journal

JF - Haematologica-The Hematology Journal

SN - 1592-8721

IS - 10

ER -