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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 LU ; Sandstedt, Anna; Uggla, Bertil; Winiarski, Jacek; Ljungman, Per and Brune, Mats, et al. (2017) In Haematologica 102(10). p.1683-1690
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... (More)

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.

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Haematologica
volume
102
issue
10
pages
8 pages
publisher
Ferrata Storti Foundation
external identifiers
  • scopus:85030308835
  • wos:000411964200020
ISSN
0390-6078
DOI
10.3324/haematol.2017.169862
language
English
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yes
id
9329f123-0d1e-492a-b647-c08a1fe5c3a8
date added to LUP
2017-10-24 16:07:55
date last changed
2018-10-03 11:38:21
@article{9329f123-0d1e-492a-b647-c08a1fe5c3a8,
  abstract     = {<p>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.</p>},
  author       = {Vaht, Krista and Göransson, Magnus and Carlson, Kristina and Isaksson, Cecilia and Lenhoff, Stig and Sandstedt, Anna and Uggla, Bertil and Winiarski, Jacek and Ljungman, Per and Brune, Mats and Andersson, Per Ola},
  issn         = {0390-6078},
  language     = {eng},
  month        = {09},
  number       = {10},
  pages        = {1683--1690},
  publisher    = {Ferrata Storti Foundation},
  series       = {Haematologica},
  title        = {Incidence and outcome of acquired aplastic anemia : Real-world data from patients diagnosed in Sweden from 2000–2011},
  url          = {http://dx.doi.org/10.3324/haematol.2017.169862},
  volume       = {102},
  year         = {2017},
}