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Incidence and outcome of acquired aplastic anemia: real-world data from patients diagnosed in Sweden from 2000-2011
Sahlgrens University Hospital, Sweden; Gothenburg University, Sweden.
Sahlgrens University Hospital, Sweden.
Uppsala University Hospital, Sweden.
University Hospital, Sweden.
Show others and affiliations
2017 (English)In: Haematologica, ISSN 0390-6078, E-ISSN 1592-8721, Vol. 102, no 10, p. 1683-1690Article in journal (Refereed) Published
Abstract [en]

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.

Place, publisher, year, edition, pages
FERRATA STORTI FOUNDATION , 2017. Vol. 102, no 10, p. 1683-1690
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Hematology
Identifiers
URN: urn:nbn:se:liu:diva-141923DOI: 10.3324/haematol.2017.169862ISI: 000411964200020PubMedID: 28751565OAI: oai:DiVA.org:liu-141923DiVA, id: diva2:1149200
Note

Funding Agencies|ALF Vastra Gotaland; Gothenburg Medical Society; Alexion Sweden

Available from: 2017-10-13 Created: 2017-10-13 Last updated: 2017-11-06

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