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Ferrata Storti Foundation
Haematologica 2019 Volume 104(2):256-262
Bone Marrow Failure
Aplastic anemia in the elderly: a nationwide survey on behalf of the French Reference Center for Aplastic Anemia
Adrien Contejean,1,2,3 Matthieu Resche-Rigon,4 Jérôme Tamburini,2,3
Marion Alcantara,3,5,6 Fabrice Jardin,6 Etienne Lengliné,1,7,8 Lionel Adès,8,9 Didier Bouscary,2,3 Ambroise Marçais,3,10 Delphine Lebon,11 Cécile Chabrot,12 Louis Terriou,13 Fiorenza Barraco,14 Anne Banos,15 Lucile Bussot,16
Jean-Yves Cahn,16 Pierre Hirsch,17 Natacha Maillard,18 Laurence Simon,19
Luc-Matthieu Fornecker,
Gerard Socié, 1,21
1,8,21,22 1,8,21 Regis Peffault de Latour
20 and Flore Sicre de Fontbrune
1French Reference Center for Aplastic Anemia, CHU Saint Louis, Paris; 2Hematology department, CHU Cochin, Paris; 3Paris Descartes University, Sorbonne Paris Cité; 4Medical informatics and biostatistics department, CHU Saint-Louis, Paris; 5Department of biological hematology, CHU Necker, Paris; 6Hematology department, Centre Henri Becquerel, Rouen; 7Hematology department, CHU Saint-Louis, Paris; 8Paris Diderot University; 9Senior hematology department, CHU Saint-Louis, Paris; 10Hematology department, CHU Necker, Paris; 11Hematology department, CHU Amiens; 12Hematology department, CHU Estaing, Clermont-Ferrand; 13Clinical immunology department, CHU Lille; 14Hematology department, CHU Lyon-Sud; 15Hematology department, CH Côte Basque, Bayonne; 16Hematology department, CHU Grenoble; 17Biological hematology department, CHU Saint-Antoine; 18Hematology department, CHU La Miletrie, Poitiers; 19Hematology department, CHU Pitié-Salpêtrière, Paris; 20Hematology department, CHU Strasbourg; 21Bone-marrow transplantation department, CHU Saint-Louis, Paris; 22Inserm UMR 1160, CHU Saint Louis, Paris, France
ABSTRACT
Aplastic anemia is a rare but potentially life-threatening disease that may affect older patients. Data regarding the treatment of aplastic anemia in this ageing population remains scarce. We conducted a retrospective nationwide multicenter study in France to examine current treatments for aplastic anemia patients over 60 years old. Our aims were to evaluate efficacy and tolerance, and to analyze predictive factors for response and survival. Over the course of a decade, 88 patients (median age 68.5 years) were identified in 19 centers, with a median follow up of 2.7 years; 21% had very severe and 36% severe aplastic anemia. We ana- lyzed 184 treatment lines, mostly involving the standard combination of anti-thymocyte globulin and cyclosporine-A (33%), which was also the most frequent first-line treatment (50%). After first-line therapy, 32% of patients achieved a complete response, and 15% a partial response. Responses were significantly better in first line and in patients with good performance status, as well as in those that had followed an anti-thymo- cyte globulin and cyclosporine-A regimen (overall response rate of 70% after first-line treatment). All treatments were well tolerated by patients, including over the age of 70. Three-year survival was 74.7% (median 7.36 years). Age, Charlson comorbidity index and very severe aplastic anemia were independently associated with mortality. Age, per se, is not a limiting factor to aplastic anemia treatment with anti-thymocyte glob- ulin and cyclosporine-A; this regimen should be used as a first-line treat- ment in elderly patients if they have a good performance status and low comorbidity index score.
Introduction
Aplastic anemia (AA) affects two to seven individuals per million annually with a higher incidence in Asia than in the west.1–3 Its incidence varies with age, occur- ring most frequently over the age of 60.4–6 Members of this patient population share specific epidemiological characteristics of the disease, suggesting a different
Correspondence:
regis.peffaultdelatour@aphp.fr
Received: May 24, 2018.
Accepted: September 24, 2018. Pre-published: September 27, 2018.
doi:10.3324/haematol.2018.198440
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