11
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Aplastic anemia in the elderly: a nationwide survey on behalf of the French Reference Center for Aplastic Anemia

      research-article

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          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 analyzed 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-thymocyte 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 globulin and cyclosporine-A; this regimen should be used as a first-line treatment in elderly patients if they have a good performance status and low comorbidity index score.

          Related collections

          Most cited references24

          • Record: found
          • Abstract: found
          • Article: not found

          Somatic Mutations and Clonal Hematopoiesis in Aplastic Anemia.

          In patients with acquired aplastic anemia, destruction of hematopoietic cells by the immune system leads to pancytopenia. Patients have a response to immunosuppressive therapy, but myelodysplastic syndromes and acute myeloid leukemia develop in about 15% of the patients, usually many months to years after the diagnosis of aplastic anemia.
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Severe aplastic anemia: a prospective study of the effect of early marrow transplantation on acute mortality.

            A prospective randomized trial of therapy for severe aplastic anemia was designed to compare early bone marrow transplantation with conventional treatments. All patients with a sibling matched at the major histocompatibility region were transplanted. Transplantation was performed with 17-100 (median 33) days of original diagnosis. Conventional treatments included transfusion support with or without androgens. Twenty-four of 36 patients intered on the transplant arm are alive after 4-20 (median 9) mo with full marrow reconstitution. Only two are limited by chronic graft-versus-host disease. In contrast only 12 of 31 conventionally treated patients are alive. Six of these survivors have improved, five incompletely. The 19 nontransplant deaths have occurred within 1-11 (median 3) mo of diagnosis. Compared to nontransplant regimens, early transplantation more effectively restores normal marrow function and decreases the acute mortality of severe marrow aplasia (p = 0.006). Pending longer follow-up, early marrow transplantation appears to be the most effective available treatment for severe aplastic anemia.
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Cytogenetic features in myelodysplastic syndromes

              Myelodysplastic syndromes (MDS) comprise a group of bone marrow diseases characterized by profound heterogeneity in morphologic presentation, clinical course, and cytogenetic features. Roughly 50% of patients display clonal chromosome abnormalities. In several multicentric studies, the karyotype turned out to be one of the most important prognostic parameters and was incorporated into statistical models aiming for a better prediction of the individual prognosis like the International Prognostic Scoring System. However, due to the profound cytogenetic heterogeneity, the impact of many rare abnormalities as well as combinations of anomalies occurring in a substantial portion of patients with MDS is still unknown and can only be delineated on the basis of large international multicentric cooperations. Recently, the German–Austrian MDS Study Group presented cytogenetic findings in 2,072 patients with MDS, which serve as a basis for the characterization of the cytogenetic subgroups discussed in this article. The availability of new therapeutic options for low- and high-risk MDS targeted against distinct entities characterized by specific chromosome abnormalities, like 5q-deletions, monosomy 7, and complex abnormalities underlines the important role of cytogenetics for the clinical management of MDS. This article thus focuses on the clinical and prognostic relevance, the molecular background, and therapeutic perspectives in these three cytogenetic subgroups.
                Bookmark

                Author and article information

                Journal
                Haematologica
                Haematologica
                haematol
                Haematologica
                Haematologica
                Ferrata Storti Foundation
                0390-6078
                1592-8721
                February 2019
                27 September 2018
                : 104
                : 2
                : 256-262
                Affiliations
                [1 ]French Reference Center for Aplastic Anemia, CHU Saint Louis, Paris
                [2 ]Hematology department, CHU Cochin, Paris
                [3 ]Paris Descartes University, Sorbonne Paris Cité
                [4 ]Medical informatics and biostatistics department, CHU Saint-Louis, Paris
                [5 ]Department of biological hematology, CHU Necker, Paris
                [6 ]Hematology department, Centre Henri Becquerel, Rouen
                [7 ]Hematology department, CHU Saint-Louis, Paris
                [8 ]Paris Diderot University
                [9 ]Senior hematology department, CHU Saint-Louis, Paris
                [10 ]Hematology department, CHU Necker, Paris
                [11 ]Hematology department, CHU Amiens
                [12 ]Hematology department, CHU Estaing, Clermont-Ferrand
                [13 ]Clinical immunology department, CHU Lille
                [14 ]Hematology department, CHU Lyon-Sud
                [15 ]Hematology department, CH Côte Basque, Bayonne
                [16 ]Hematology department, CHU Grenoble
                [17 ]Biological hematology department, CHU Saint-Antoine
                [18 ]Hematology department, CHU La Miletrie, Poitiers
                [19 ]Hematology department, CHU Pitié-Salpêtrière, Paris
                [20 ]Hematology department, CHU Strasbourg
                [21 ]Bone-marrow transplantation department, CHU Saint-Louis, Paris
                [22 ]Inserm UMR 1160, CHU Saint Louis, Paris, France
                Author notes
                Article
                1040256
                10.3324/haematol.2018.198440
                6355477
                30262561
                c3ddbc53-8545-4364-9ed6-dea30533537a
                Copyright © 2019 Ferrata Storti Foundation

                Material published in Haematologica is covered by copyright. All rights are reserved to the Ferrata Storti Foundation. Use of published material is allowed under the following terms and conditions:

                https://creativecommons.org/licenses/by-nc/4.0/legalcode. Copies of published material are allowed for personal or internal use. Sharing published material for non-commercial purposes is subject to the following conditions:

                https://creativecommons.org/licenses/by-nc/4.0/legalcode, sect. 3. Reproducing and sharing published material for commercial purposes is not allowed without permission in writing from the publisher.

                History
                : 24 May 2018
                : 24 September 2018
                Categories
                Article
                Bone Marrow Failure

                Comments

                Comment on this article