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

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Abstract

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.

Detaljer

Författare
  • Krista Vaht
  • Magnus Göransson
  • Kristina Carlson
  • Cecilia Isaksson
  • Stig Lenhoff
  • Anna Sandstedt
  • Bertil Uggla
  • Jacek Winiarski
  • Per Ljungman
  • Mats Brune
  • Per Ola Andersson
Enheter & grupper
Externa organisationer
  • Sahlgrenska University Hospital
  • Sahlgrenska Academy
  • Queen Silvia Children’s Hospital
  • Uppsala University Hospital
  • Norrland University Hospital
  • Skåne University Hospital
  • Linköping University Hospital
  • Örebro University
  • Karolinska University Hospital
Forskningsområden

Ämnesklassifikation (UKÄ) – OBLIGATORISK

  • Gastroenterologi
Originalspråkengelska
Sidor (från-till)1683-1690
Antal sidor8
TidskriftHaematologica
Volym102
Utgivningsnummer10
StatusPublished - 2017 sep 30
PublikationskategoriForskning
Peer review utfördJa