Allogenic Hematopoietic Stem Cell Transplantation from Related Donors in Fanconi Anemia

"n  "n  "n  "n  "n  "n  "n  "n  "n  "n  "n  "n &a...

Full description

Bibliographic Details
Main Authors: Amir Ali Hamidieh, Ardeshir Ghavamzadeh, Mohammad Jahani
Format: Article
Language:English
Published: Tehran University of Medical Sciences 2009-01-01
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
_version_ 1797764492187140096
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