دورية أكاديمية
Transplant results in adults with Fanconi anaemia
| العنوان: | Transplant results in adults with Fanconi anaemia |
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| المؤلفون: | Bierings M., Bonfim C., Peffault De Latour R., Aljurf M., Mehta P. A., Knol C., Boulad F., Tbakhi A., Esquirol A., McQuaker G., Sucak G. A., Othman T. B., Halkes C. J. M., Carpenter B., Niederwieser D., Zecca M., Kroger N., Michallet M., Risitano A. M., Ehninger G., Porcher R., Dufour C., Bonfirm C., Socie G., Gurman G., Ghavamzadeh A., Hamladji R. -M., van Lint M. T., Stepensky P., Koh M., Ozkurt Z. N., Veelken J. H., Bunjes D., Beelen D., Campos A., Robinson S., Alessandrino E. P., Unal A., Fernandez Navarro J. M., Mufti G. J., Velardi A., Passweg J., Apperley J., Sengeloev H., Ljungman P., Foa R., Alegre A., Espiga C. R., Cornelissen J. J., Di Bartolomeo P., Cordonnier C., Browne P., Jubert C., Gastl G., Pierelli L., Johansson J. -E., Fagioli F., Moraleda J., Zuckerman T., Bazarbachi A., Sedlacek P., Rossig C., Wynn R. F., Hallek M., Toren A., Zudaire T., Clausen J., Spencer A., Grazon Lopez S., Schots R., Komarnicki M., Gonzalez Muniz S., Vitek A., Rambaldi A., Merli F., Rubio M. T., Cabrera Marin J. R., Porto F., Kerre T., Metzner B., Stein J., Bertrand Y., Ciceri F., Chybicka A., Diez-Martin J. L., Bayoumy M., de la Fuente J., Fegueux N. |
| المساهمون: | Bierings, M., Bonfim, C., Peffault De Latour, R., Aljurf, M., Mehta, P. A., Knol, C., Boulad, F., Tbakhi, A., Esquirol, A., Mcquaker, G., Sucak, G. A., Othman, T. B., Halkes, C. J. M., Carpenter, B., Niederwieser, D., Zecca, M., Kroger, N., Michallet, M., Risitano, A. M., Ehninger, G., Porcher, R., Dufour, C., Bonfirm, C., Socie, G., Gurman, G., Ghavamzadeh, A., Hamladji, R. -M., van Lint, M. T., Stepensky, P., Koh, M., Ozkurt, Z. N., Veelken, J. H., Bunjes, D., Beelen, D., Campos, A., Robinson, S., Alessandrino, E. P., Unal, A., Fernandez Navarro, J. M., Mufti, G. J., Velardi, A., Passweg, J., Apperley, J., Sengeloev, H., Ljungman, P., Foa, R., Alegre, A., Espiga, C. R., Cornelissen, J. J., Di Bartolomeo, P., Cordonnier, C., Browne, P., Jubert, C., Gastl, G., Pierelli, L., Johansson, J. -E., Fagioli, F., Moraleda, J., Zuckerman, T., Bazarbachi, A., Sedlacek, P., Rossig, C., Wynn, R. F., Hallek, M., Toren, A., Zudaire, T., Clausen, J., Spencer, A., Grazon Lopez, S., Schots, R., Komarnicki, M., Gonzalez Muniz, S., Vitek, A., Rambaldi, A., Merli, F., Rubio, M. T., Cabrera Marin, J. R., Porto, F., Kerre, T., Metzner, B., Stein, J., Bertrand, Y., Ciceri, F., Chybicka, A., Diez-Martin, J. L., Bayoumy, M., de la Fuente, J., Fegueux, N. |
| بيانات النشر: | Blackwell Publishing Ltd |
| سنة النشر: | 2018 |
| مصطلحات موضوعية: | allogeneic transplant, Fanconi anaemia, inborn bone marrow failure syndrome, myelodysplasia, Adolescent, Adult, Cause of Death, Fanconi Anemia, Graft vs Host Disease, Humans, Middle Aged, Neoplasms, Second Primary, Prognosis, Retrospective Studies, Risk Factors, Survival Analysis, Tissue Donors, Transplantation Conditioning, Transplantation, Homologous, Treatment Outcome, Young Adult, Hematopoietic Stem Cell Transplantation |
| الوصف: | The outcomes of adult patients transplanted for Fanconi anaemia (FA) have not been well described. We retrospectively analysed 199 adult patients with FA transplanted between 1991 and 2014. Patients were a median of 16years of age when diagnosed with FA, and underwent transplantation at a median age of 23years. Time between diagnosis and transplant was shortest (median 2years) in those patients who had a human leucocyte antigen identical sibling donor. Fifty four percent of patients had bone marrow (BM) failure at transplantation and 46% had clonal disease (34% myelodysplasia, 12% acute leukaemia). BM was the main stem cell source, the conditioning regimen included cyclophosphamide in 96% of cases and fludarabine in 64%. Engraftment occurred in 82% (95% confidence interval [CI] 76–87%), acute graft-versus-host disease (GvHD) grade II–IV in 22% (95% CI 16–28%) and the incidence of chronic GvHD at 96months was 26% (95% CI 20–33). Non-relapse mortality at 96months was 56% with an overall survival of 34%, which improved with more recent transplants. Median follow-up was 58months. Patients transplanted after 2000 had improved survival (84% at 36months), using BM from an identical sibling and fludarabine in the conditioning regimen. Factors associated with improved outcome in multivariate analysis were use of fludarabine and an identical sibling or matched non-sibling donor. Main causes of death were infection (37%), GvHD (24%) and organ failure (12%). The presence of clonal disease at transplant did not significant impact on survival. Secondary malignancies were reported in 15 of 131 evaluable patients. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| العلاقة: | info:eu-repo/semantics/altIdentifier/wos/WOS:000418802600013; volume:180; issue:1; firstpage:100; lastpage:109; numberofpages:10; journal:BRITISH JOURNAL OF HAEMATOLOGY; http://hdl.handle.net/20.500.11768/110199Test; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85032830886 |
| DOI: | 10.1111/bjh.15006 |
| الإتاحة: | https://doi.org/20.500.11768/110199Test https://doi.org/10.1111/bjh.15006Test https://hdl.handle.net/20.500.11768/110199Test |
| رقم الانضمام: | edsbas.AF165C5B |
| قاعدة البيانات: | BASE |
| DOI: | 10.1111/bjh.15006 |
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