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

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.

SUBMITTER: Contejean A

PROVIDER: S-EPMC6355477 | biostudies-literature | 2019 Feb

REPOSITORIES: biostudies-literature

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

Contejean Adrien A Resche-Rigon Matthieu M Tamburini Jérôme J Alcantara Marion M Jardin Fabrice F Lengliné Etienne E Adès Lionel L Bouscary Didier D Marçais Ambroise A Lebon Delphine D Chabrot Cécile C Terriou Louis L Barraco Fiorenza F Banos Anne A Bussot Lucile L Cahn Jean-Yves JY Hirsch Pierre P Maillard Natacha N Simon Laurence L Fornecker Luc-Matthieu LM Socié Gerard G de Latour Regis Peffault RP de Fontbrune Flore Sicre FS

Haematologica 20180927 2

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 6 ...[more]

PMID: 30262561

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Project description:Few therapeutic options are available for patients with aplastic anemia who are ineligible for transplantation or refractory to immunosuppressive therapy. Eltrombopag was recently shown to produce trilineage responses in refractory patients. However, the effects of real-life use of this drug remain unknown. This retrospective study (2012-2016) was conducted by the French Reference Center for Aplastic Anemia on patients with relapsed/refractory aplastic anemia, and patients ineligible for antithymocyte globulin or transplantation, who received eltrombopag for at least 2 months. Forty-six patients with aplastic anemia were given eltrombopag without prior antithymocyte globulin treatment (n=11) or after antithymocyte globulin administration (n=35) in a relapsed/refractory setting. Eltrombopag (median daily dose 150 mg) was introduced 17 months (range, 8-50) after the diagnosis of aplastic anemia. At last followup, 49% were still receiving treatment, 9% had stopped due to a robust response, 2% due to toxicity and 40% due to eltrombopag failure. Before eltrombopag treatment, all patients received regular transfusions. The overall rates of red blood cell and platelet transfusion independence were 7%, 33%, 46% and 46% at 1, 3, 6 months and last follow-up. Responses were slower to develop in antithymocyte treatment-naïve patients. In patients achieving transfusion independence, hemoglobin concentration and platelet counts improved by 3 g/dL (interquartile range, 1.4-4.5) and 42×109/L (interquartile range, 11-100), respectively. Response in at least one lineage (according to National Institutes of Health criteria) was observed in 64% of antithymocyte treatment-naïve and 74% of relapsed/refractory patients, while trilineage improvement was observed in 27% and 34%, respectively. We found high rates of hematologic improvement and transfusion independence in refractory aplastic anemia patients but also in patients ineligible for antithymocyte globulin receiving first-line treatment. In conclusion, elderly patients unfit for antithymocyte globulin therapy may benefit from eltrombopag.

Project description:Protective ventilation is associated with a lower incidence of pulmonary complications. However, there are few published data on routine pulmonary management in adult cardiac surgery. The present study's primary objective was to survey pulmonary management in this high-risk population, as practiced by anesthesiologists in France. All 460 registered France-based cardiac anesthesiologists were invited (by e-mail) to participate in an online survey in January-February 2015. The survey's questionnaire was designed to assess current practice in pre-, per-, and postoperative pulmonary management. In all, 198 anesthesiologists (43% of those invited) participated in the survey. Other than during the cardiopulmonary bypass (CPB) per se, 179 anesthesiologists (91% of respondees) [95% confidence interval (CI): 87-95] used a low-tidal-volume approach (6-8 mL/kg), whereas techniques based on positive end-expiratory pressure and recruitment maneuvers vary greatly from 1 anesthesiologist to another. During CPB, 104 (53%) [95% CI: 46-60] anesthesiologists withdrew mechanical ventilation (with disconnection, in some cases) and 97 (49%) [95% CI: 42-56] did not prescribe positive end-expiratory pressure. One hundred sixty-five (83%) [95% CI: 78-88] anesthesiologists stated that a written protocol for peroperative pulmonary management was not available. Twenty (10%) [95% CI: 6-14] and 11 (5%) [95% CI: 2-8] anesthesiologists stated that they did use protocols for ventilator use and recruitment maneuvers, respectively. The preoperative period (pulmonary examinations and prescription of additional assessments) and the postoperative period (extubation, and noninvasive ventilation) periods vary greatly from 1 anesthesiologist to another. The great majority of French cardiac anesthesiologists use a low tidal volume during cardiac surgery (other than during CPB per se). However, pulmonary management procedures varied markedly from 1 anesthesiologist to another. There is a clear need for large clinical studies designed to identify best practice in pulmonary management.

Dataset Information

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

Publications

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

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