Hematopoietic Stem Cell Transplantation in Patients with Severe Acquired Aplastic Anemia: Iranian Experience

Introduction: Severe acquired aplastic anemia (SAA) is a rare disease and matched related hematopoietic stem cell transplantation (HSCT) is the treatment of choice especially in pediatric patients. Immunosuppressive therapy is the alternative treatment in patients who do not have a donor. We retrosp...

Full description

Bibliographic Details
Main Authors: Masoud Iravani, Seyyed Asadollah Mousavi, Babak Bahar, Mohammad Jahani, Amir Hamdi, Amir Ali Hamidieh, Kamran Alimoghaddam, Mahdi Jalili, Arash Jalali, Ardeshir Ghavamzadeh
Format: Article
Language:English
Published: Tehran University of Medical Sciences 2011-10-01
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/20862.pdf&manuscript_id=20862
id doaj-b78d0a91b2ce41a7b9a035431ff4a257
record_format Article
spelling doaj-b78d0a91b2ce41a7b9a035431ff4a2572020-11-25T03:57:10ZengTehran University of Medical SciencesInternational Journal of Hematology-Oncology and Stem Cell Research2008-30092008-22072011-10-01542227Hematopoietic Stem Cell Transplantation in Patients with Severe Acquired Aplastic Anemia: Iranian ExperienceMasoud IravaniSeyyed Asadollah MousaviBabak BaharMohammad JahaniAmir HamdiAmir Ali HamidiehKamran AlimoghaddamMahdi JaliliArash JalaliArdeshir GhavamzadehIntroduction: Severe acquired aplastic anemia (SAA) is a rare disease and matched related hematopoietic stem cell transplantation (HSCT) is the treatment of choice especially in pediatric patients. Immunosuppressive therapy is the alternative treatment in patients who do not have a donor. We retrospectively analyzed patients who received allogeneic HSCT at our institution. Methods: Between 1991 and 2011, 190 patients received allogeneic HSCT from HLA-matched donors (182 siblings and 8 other relatives). Median age was 20.5 years (range 1 to 50 years). The graft source was peripheral blood stem cells in majority of patients. Conditioning regimen consist with a myeloablative regimen containing cyclophosphamide with or without antithymocyte globulin. For graft-versus-host disease (GvHD) prophylaxis, we used cyclosporine with or without methotrexate at the standard doses. Results: The median follow-up time was 30 months and 3 year overall survival and disease free survival was 82% and 75% respectively. The median time to neutrophil engraftment and median time to platelet engraftment was 12 day and 15 day respectively. Grade 3 and 4 of aGvHD occurred in 26 (23.7%) patients and chronic GvHD occurred in 46 (29.1%) of survived patients 100 days after HSCT. At time of report 82.1% of patients were alive with normal hematologic parameters. The engraftment failure rate was about 8%. The most common cause of death was GvHD. Conclusions: However an available treatment in SAA is immunosuppressive therapy, HSCT should be seriously considered as a therapeutic option particularly if a matched sibling donor is available. The outcome of allogeneic HSCT in patients with SAA at our center was consistent with the result of other previous studies.http://journals.tums.ac.ir/PdfMed.aspx?pdf_med=/upload_files/pdf/20862.pdf&manuscript_id=20862Severe acquired aplastic anemiaHematopoietic stem cell transplantation
collection DOAJ
language English
format Article
sources DOAJ
author Masoud Iravani
Seyyed Asadollah Mousavi
Babak Bahar
Mohammad Jahani
Amir Hamdi
Amir Ali Hamidieh
Kamran Alimoghaddam
Mahdi Jalili
Arash Jalali
Ardeshir Ghavamzadeh
spellingShingle Masoud Iravani
Seyyed Asadollah Mousavi
Babak Bahar
Mohammad Jahani
Amir Hamdi
Amir Ali Hamidieh
Kamran Alimoghaddam
Mahdi Jalili
Arash Jalali
Ardeshir Ghavamzadeh
Hematopoietic Stem Cell Transplantation in Patients with Severe Acquired Aplastic Anemia: Iranian Experience
International Journal of Hematology-Oncology and Stem Cell Research
Severe acquired aplastic anemia
Hematopoietic stem cell transplantation
author_facet Masoud Iravani
Seyyed Asadollah Mousavi
Babak Bahar
Mohammad Jahani
Amir Hamdi
Amir Ali Hamidieh
Kamran Alimoghaddam
Mahdi Jalili
Arash Jalali
Ardeshir Ghavamzadeh
author_sort Masoud Iravani
title Hematopoietic Stem Cell Transplantation in Patients with Severe Acquired Aplastic Anemia: Iranian Experience
title_short Hematopoietic Stem Cell Transplantation in Patients with Severe Acquired Aplastic Anemia: Iranian Experience
title_full Hematopoietic Stem Cell Transplantation in Patients with Severe Acquired Aplastic Anemia: Iranian Experience
title_fullStr Hematopoietic Stem Cell Transplantation in Patients with Severe Acquired Aplastic Anemia: Iranian Experience
title_full_unstemmed Hematopoietic Stem Cell Transplantation in Patients with Severe Acquired Aplastic Anemia: Iranian Experience
title_sort hematopoietic stem cell transplantation in patients with severe acquired aplastic anemia: iranian experience
publisher Tehran University of Medical Sciences
series International Journal of Hematology-Oncology and Stem Cell Research
issn 2008-3009
2008-2207
publishDate 2011-10-01
description Introduction: Severe acquired aplastic anemia (SAA) is a rare disease and matched related hematopoietic stem cell transplantation (HSCT) is the treatment of choice especially in pediatric patients. Immunosuppressive therapy is the alternative treatment in patients who do not have a donor. We retrospectively analyzed patients who received allogeneic HSCT at our institution. Methods: Between 1991 and 2011, 190 patients received allogeneic HSCT from HLA-matched donors (182 siblings and 8 other relatives). Median age was 20.5 years (range 1 to 50 years). The graft source was peripheral blood stem cells in majority of patients. Conditioning regimen consist with a myeloablative regimen containing cyclophosphamide with or without antithymocyte globulin. For graft-versus-host disease (GvHD) prophylaxis, we used cyclosporine with or without methotrexate at the standard doses. Results: The median follow-up time was 30 months and 3 year overall survival and disease free survival was 82% and 75% respectively. The median time to neutrophil engraftment and median time to platelet engraftment was 12 day and 15 day respectively. Grade 3 and 4 of aGvHD occurred in 26 (23.7%) patients and chronic GvHD occurred in 46 (29.1%) of survived patients 100 days after HSCT. At time of report 82.1% of patients were alive with normal hematologic parameters. The engraftment failure rate was about 8%. The most common cause of death was GvHD. Conclusions: However an available treatment in SAA is immunosuppressive therapy, HSCT should be seriously considered as a therapeutic option particularly if a matched sibling donor is available. The outcome of allogeneic HSCT in patients with SAA at our center was consistent with the result of other previous studies.
topic Severe acquired aplastic anemia
Hematopoietic stem cell transplantation
url http://journals.tums.ac.ir/PdfMed.aspx?pdf_med=/upload_files/pdf/20862.pdf&manuscript_id=20862
work_keys_str_mv AT masoudiravani hematopoieticstemcelltransplantationinpatientswithsevereacquiredaplasticanemiairanianexperience
AT seyyedasadollahmousavi hematopoieticstemcelltransplantationinpatientswithsevereacquiredaplasticanemiairanianexperience
AT babakbahar hematopoieticstemcelltransplantationinpatientswithsevereacquiredaplasticanemiairanianexperience
AT mohammadjahani hematopoieticstemcelltransplantationinpatientswithsevereacquiredaplasticanemiairanianexperience
AT amirhamdi hematopoieticstemcelltransplantationinpatientswithsevereacquiredaplasticanemiairanianexperience
AT amiralihamidieh hematopoieticstemcelltransplantationinpatientswithsevereacquiredaplasticanemiairanianexperience
AT kamranalimoghaddam hematopoieticstemcelltransplantationinpatientswithsevereacquiredaplasticanemiairanianexperience
AT mahdijalili hematopoieticstemcelltransplantationinpatientswithsevereacquiredaplasticanemiairanianexperience
AT arashjalali hematopoieticstemcelltransplantationinpatientswithsevereacquiredaplasticanemiairanianexperience
AT ardeshirghavamzadeh hematopoieticstemcelltransplantationinpatientswithsevereacquiredaplasticanemiairanianexperience
_version_ 1724461618615549952