Allogenic Hematopoietic Stem Cell Transplantation from Related Donors in Fanconi Anemia
"n  "n  "n  "n  "n  "n  "n  "n  "n  "n  "n  "n &a...
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Format: | Article |
Language: | English |
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Tehran University of Medical Sciences
2009-01-01
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Series: | International Journal of Hematology-Oncology and Stem Cell Research |
Subjects: | |
Online Access: | http://journals.tums.ac.ir/PdfMed.aspx?pdf_med=/upload_files/pdf/16072.pdf&manuscript_id=16072 |
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author | Amir Ali Hamidieh Ardeshir Ghavamzadeh Mohammad Jahani |
author_facet | Amir Ali Hamidieh Ardeshir Ghavamzadeh Mohammad Jahani |
author_sort | Amir Ali Hamidieh |
collection | DOAJ |
description | "n  "n  "n  "n  "n  "n  "n  "n  "n  "n  "n  "n "nIntroduction: "n  "nAllogeneic hematopoietic cell transplantation (HSCT) is the only therapeutic modality capable of "ncorrecting the hematologic manifestations of Fanconi Anemia (FA). "nThe development of well tolerated, immunosuppressive conditioning regimens for FA patients undergoing "nHSCT has proven to be a rather challenging task for hematologists. "nMethods: "n  "nWe analyzed the outcome of 30 FA patients (median age at HSCT was 9 years age range, 2-32 years) "nwho underwent HSCT between 1992 and 2008 in Shariati Hospital Tehran, Iran. . Patients were transplanted "nfrom either an HLA-identical sibling or matched relative (n=29), or an HLA-partially matched relative(n=1). "nFour different conditioning regimens without radiation were used .Graft versus host disease (GVHD) "nprevention consisted of cyclosporine with methotrexate or cyclosporine alone. "nResults: "n  "nThe median follow-up duration for survivors was 2.7 years (ranged 1 month to 12 years). The median "nsurvival time was 8.5 months. The 5-year overall survival was 43.6% (SE=10.0%). All surviving patients had "nnormal blood counts with full donor engraftment. "nThe median survival rate for patients who did or did not receive fludarabine in preparation for the allograft was "nnot statistically significant (p-value=1.0). "nConclusion: "n  "nOur study demonstrates that none of the studied variables significantly affected the survival, |
first_indexed | 2024-03-12T19:56:29Z |
format | Article |
id | doaj.art-f5843c76e6644519a138563b0f44a1b8 |
institution | Directory Open Access Journal |
issn | 2008-3009 2008-2207 |
language | English |
last_indexed | 2024-03-12T19:56:29Z |
publishDate | 2009-01-01 |
publisher | Tehran University of Medical Sciences |
record_format | Article |
series | International Journal of Hematology-Oncology and Stem Cell Research |
spelling | doaj.art-f5843c76e6644519a138563b0f44a1b82023-08-02T02:46:58ZengTehran University of Medical SciencesInternational Journal of Hematology-Oncology and Stem Cell Research2008-30092008-22072009-01-013116Allogenic Hematopoietic Stem Cell Transplantation from Related Donors in Fanconi AnemiaAmir Ali HamidiehArdeshir GhavamzadehMohammad Jahani"n  "n  "n  "n  "n  "n  "n  "n  "n  "n  "n  "n "nIntroduction: "n  "nAllogeneic hematopoietic cell transplantation (HSCT) is the only therapeutic modality capable of "ncorrecting the hematologic manifestations of Fanconi Anemia (FA). "nThe development of well tolerated, immunosuppressive conditioning regimens for FA patients undergoing "nHSCT has proven to be a rather challenging task for hematologists. "nMethods: "n  "nWe analyzed the outcome of 30 FA patients (median age at HSCT was 9 years age range, 2-32 years) "nwho underwent HSCT between 1992 and 2008 in Shariati Hospital Tehran, Iran. . Patients were transplanted "nfrom either an HLA-identical sibling or matched relative (n=29), or an HLA-partially matched relative(n=1). "nFour different conditioning regimens without radiation were used .Graft versus host disease (GVHD) "nprevention consisted of cyclosporine with methotrexate or cyclosporine alone. "nResults: "n  "nThe median follow-up duration for survivors was 2.7 years (ranged 1 month to 12 years). The median "nsurvival time was 8.5 months. The 5-year overall survival was 43.6% (SE=10.0%). All surviving patients had "nnormal blood counts with full donor engraftment. "nThe median survival rate for patients who did or did not receive fludarabine in preparation for the allograft was "nnot statistically significant (p-value=1.0). "nConclusion: "n  "nOur study demonstrates that none of the studied variables significantly affected the survival,http://journals.tums.ac.ir/PdfMed.aspx?pdf_med=/upload_files/pdf/16072.pdf&manuscript_id=16072 Fanconi anemia, Hematopoietic Stem Cell Transplantation |
spellingShingle | Amir Ali Hamidieh Ardeshir Ghavamzadeh Mohammad Jahani Allogenic Hematopoietic Stem Cell Transplantation from Related Donors in Fanconi Anemia International Journal of Hematology-Oncology and Stem Cell Research Fanconi anemia, Hematopoietic Stem Cell Transplantation |
title | Allogenic Hematopoietic Stem Cell Transplantation from Related Donors in Fanconi Anemia |
title_full | Allogenic Hematopoietic Stem Cell Transplantation from Related Donors in Fanconi Anemia |
title_fullStr | Allogenic Hematopoietic Stem Cell Transplantation from Related Donors in Fanconi Anemia |
title_full_unstemmed | Allogenic Hematopoietic Stem Cell Transplantation from Related Donors in Fanconi Anemia |
title_short | Allogenic Hematopoietic Stem Cell Transplantation from Related Donors in Fanconi Anemia |
title_sort | allogenic hematopoietic stem cell transplantation from related donors in fanconi anemia |
topic | Fanconi anemia, Hematopoietic Stem Cell Transplantation |
url | http://journals.tums.ac.ir/PdfMed.aspx?pdf_med=/upload_files/pdf/16072.pdf&manuscript_id=16072 |
work_keys_str_mv | AT amiralihamidieh allogenichematopoieticstemcelltransplantationfromrelateddonorsinfanconianemia AT ardeshirghavamzadeh allogenichematopoieticstemcelltransplantationfromrelateddonorsinfanconianemia AT mohammadjahani allogenichematopoieticstemcelltransplantationfromrelateddonorsinfanconianemia |