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Incidence and outco...
Incidence and outcome of acquired aplastic anemia: real-world data from patients diagnosed in Sweden from 2000-2011
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- Vaht, Krista, 1973 (author)
- Gothenburg University,Göteborgs universitet,Institutionen för medicin,Institute of Medicine,Section of Hematology and Coagulation, Sahlgrenska University Hospital, Gothenburg, Sweden; Sahlgrenska Academy, Gothenburg University, Gothenburg, Sweden,Sahlgrenska Academy,Sahlgrens Univ Hosp, Sect Hematol & Coagulat, Gothenburg, Sweden.;Gothenburg Univ, Sahlgrenska Acad, Gothenburg, Sweden.
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- Goransson, M. (author)
- Queen Silvia Children’s Hospital,Section of Hematology and Coagulation, Sahlgrenska University Hospital, Gothenburg, Sweden; Sahlgrenska Academy, Gothenburg University, Gothenburg, Sweden,Sahlgrens Univ Hosp, Queen Silvia Childrens Hosp, Dept Pediat, Gothenburg, Sweden.
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- Carlson, Kristina (author)
- Uppsala universitet,Hematologi,Uppsala University Hospital, Sweden
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- Isaksson, C. (author)
- Norrland University Hospital,Department of Hematology, Cancer Centre, University Hospital, Uppsala, Sweden,Univ Hosp, Dept Hematol, Canc Ctr, Umea, Sweden.
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- Lenhoff, Stig (author)
- Lund University,Lunds universitet,Avdelningen för hematologi och transfusionsmedicin,Institutionen för laboratoriemedicin,Medicinska fakulteten,Division of Hematology and Transfusion Medicine,Department of Laboratory Medicine,Faculty of Medicine,Department of Hematology, Skåne University Hospital, Lund University, Malmö, Sweden,Lund Univ, Skane Univ Hosp, Dept Hematol, Lund, Sweden.
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- Sandstedt, Anna (author)
- Linköpings universitet,Institutionen för samhälls- och välfärdsstudier,Hälsouniversitetet,Region Östergötland, Hematologiska kliniken US
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- Uggla, Bertil, 1962- (author)
- Örebro universitet,Örebro University,Institutionen för medicinska vetenskaper,Department of Medicine, Section of Hematology, , Faculty of Health and Medical Sciences, Örebro University, Örebro, Sweden,Orebro Univ, Fac Med & Hlth, Dept Med, Sect Hematol, Orebro, Sweden.
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- Winiarski, J. (author)
- Karolinska Institutet
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- Ljungman, P. (author)
- Karolinska Institutet
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- Brune, Mats, 1950 (author)
- Gothenburg University,Göteborgs universitet,Institutionen för medicin,Institute of Medicine,Section of Hematology and Coagulation, Sahlgrenska University Hospital, Gothenburg, Sweden; Sahlgrenska Academy, Gothenburg University, Gothenburg, Sweden,Sahlgrenska University Hospital,Sahlgrens Univ Hosp, Sect Hematol & Coagulat, Gothenburg, Sweden.;Gothenburg Univ, Sahlgrenska Acad, Gothenburg, Sweden.
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- Andersson, Per-Ola, 1964 (author)
- Gothenburg University,Göteborgs universitet,Institutionen för medicin,Institute of Medicine,South Älvsborg Hospital, Borås, Sweden; Sahlgrenska Academy, Gothenburg University, Gothenburg, Sweden,South Alvsborg Hosp Boras, Boras, Sweden.;Gothenburg Univ, Sahlgrenska Acad, Gothenburg, Sweden.
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(creator_code:org_t)
- 2017-07-27
- 2017
- English.
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In: Haematologica. - Pavia, Italy : Ferrata Storti Foundation (Haematologica). - 0390-6078 .- 1592-8721. ; 102:10, s. 1683-1690
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Abstract
Subject headings
Close
- 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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Gastroenterologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Gastroenterology and Hepatology (hsv//eng)
Keyword
- bone-marrow-transplantation
- rabbit antithymocyte globulin
- immunosuppressive therapy
- working party
- antilymphocyte globulin
- prospective multicenter
- donor transplants
- european group
- older
- patients
- cyclosporine
- Hematology
Publication and Content Type
- ref (subject category)
- art (subject category)
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- By the author/editor
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Vaht, Krista, 19 ...
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Goransson, M.
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Carlson, Kristin ...
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Isaksson, C.
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Lenhoff, Stig
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Sandstedt, Anna
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Uggla, Bertil, 1 ...
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Winiarski, J.
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Ljungman, P.
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Brune, Mats, 195 ...
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Andersson, Per-O ...
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- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Hematology
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Gastroenterology ...
- Articles in the publication
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Haematologica
- By the university
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University of Gothenburg
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Lund University
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Örebro University
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Uppsala University
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Linköping University
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Karolinska Institutet