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Haematologica. 2017 Oct;102(10):1683-1690. doi: 10.3324/haematol.2017.169862. Epub 2017 Jul 27.

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

Author information

1
Section of Hematology and Coagulation, Sahlgrenska University Hospital, Gothenburg, Sweden krista.vaht@vgregion.se.
2
Sahlgrenska Academy at Gothenburg University, Sweden.
3
Department of Pediatrics, The Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Umeå, Sweden.
4
Department of Hematology, Uppsala University Hospital, Umeå, Sweden.
5
Department of Hematology, Cancer Centre, University Hospital, Umeå, Sweden.
6
Department of Hematology, Skåne University Hospital, Lund University, Stockholm, Sweden.
7
Department of Hematology, Linköping University Hospital, Stockholm, Sweden.
8
Section of Hematology Department of Medicine, Faculty of Medicine and Health, Örebro University, Stockholm, Sweden.
9
Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden.
10
Centre of allogeneic stem cell transplantation (CAST), Karolinska University Hospital Huddinge, Stockholm, Sweden.
11
Section of Hematology and Coagulation, Sahlgrenska University Hospital, Gothenburg, Sweden.
12
South Älvsborg Hospital Borås, Sweden.

Abstract

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 ≥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.

PMID:
28751565
PMCID:
PMC5622852
DOI:
10.3324/haematol.2017.169862
[Indexed for MEDLINE]
Free PMC Article

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