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Immunosuppressive therapy for aplastic anemia: a single-center experience from western India
Immunosuppressive therapy (IST) with antithymocyte globulin (ATG) and cyclosporine A (CsA) is the first-line therapy for acquired aplastic anemia (AA) in those not suitable for bone marrow transplant. Horse ATG (hATG) is preferred for this purpose, but its use is often impeded by shortages and costs...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Berlin Heidelberg
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6334724/ https://www.ncbi.nlm.nih.gov/pubmed/30173288 http://dx.doi.org/10.1007/s00277-018-3487-2 |
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author | Shah, Sandip Jain, Preetam Shah, Kamlesh Patel, Kinnari Parikh, Sonia Patel, Apurva Panchal, Harsha Anand, Asha |
author_facet | Shah, Sandip Jain, Preetam Shah, Kamlesh Patel, Kinnari Parikh, Sonia Patel, Apurva Panchal, Harsha Anand, Asha |
author_sort | Shah, Sandip |
collection | PubMed |
description | Immunosuppressive therapy (IST) with antithymocyte globulin (ATG) and cyclosporine A (CsA) is the first-line therapy for acquired aplastic anemia (AA) in those not suitable for bone marrow transplant. Horse ATG (hATG) is preferred for this purpose, but its use is often impeded by shortages and costs. Being a rare disease, there is limited data on this therapy. This study aimed to evaluate this therapy in a large cohort of AA patients from western India. We retrospectively analyzed AA patients who received an indigenous preparation of hATG along with CsA as first-line treatment, between 2012 and 2015, at our center and evaluated the response, survival, and occurrence of adverse events. The response was further assessed separately for adults and children. During the period, 91 AA patients (4 non-severe, 57 severe and 30 very severe) were treated with IST. At 2 years, 23.5% adults and 39.1% children showed complete response and an overall of 68.1% cases became transfusion independent. More than half of the patients developed febrile neutropenia while roughly one sixth of the patients developed gum hypertrophy and/or hypertension. Two patients had clonal evolution. Mortality rate was calculated to be 31%; most common causes of death were infection and intracranial hemorrhage. The results of the study substantiate the effectiveness of IST in AA, using an inexpensive indigenous preparation of hATG along with CsA. |
format | Online Article Text |
id | pubmed-6334724 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Springer Berlin Heidelberg |
record_format | MEDLINE/PubMed |
spelling | pubmed-63347242019-02-01 Immunosuppressive therapy for aplastic anemia: a single-center experience from western India Shah, Sandip Jain, Preetam Shah, Kamlesh Patel, Kinnari Parikh, Sonia Patel, Apurva Panchal, Harsha Anand, Asha Ann Hematol Original Article Immunosuppressive therapy (IST) with antithymocyte globulin (ATG) and cyclosporine A (CsA) is the first-line therapy for acquired aplastic anemia (AA) in those not suitable for bone marrow transplant. Horse ATG (hATG) is preferred for this purpose, but its use is often impeded by shortages and costs. Being a rare disease, there is limited data on this therapy. This study aimed to evaluate this therapy in a large cohort of AA patients from western India. We retrospectively analyzed AA patients who received an indigenous preparation of hATG along with CsA as first-line treatment, between 2012 and 2015, at our center and evaluated the response, survival, and occurrence of adverse events. The response was further assessed separately for adults and children. During the period, 91 AA patients (4 non-severe, 57 severe and 30 very severe) were treated with IST. At 2 years, 23.5% adults and 39.1% children showed complete response and an overall of 68.1% cases became transfusion independent. More than half of the patients developed febrile neutropenia while roughly one sixth of the patients developed gum hypertrophy and/or hypertension. Two patients had clonal evolution. Mortality rate was calculated to be 31%; most common causes of death were infection and intracranial hemorrhage. The results of the study substantiate the effectiveness of IST in AA, using an inexpensive indigenous preparation of hATG along with CsA. Springer Berlin Heidelberg 2018-09-01 2019 /pmc/articles/PMC6334724/ /pubmed/30173288 http://dx.doi.org/10.1007/s00277-018-3487-2 Text en © The Author(s) 2018 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. |
spellingShingle | Original Article Shah, Sandip Jain, Preetam Shah, Kamlesh Patel, Kinnari Parikh, Sonia Patel, Apurva Panchal, Harsha Anand, Asha Immunosuppressive therapy for aplastic anemia: a single-center experience from western India |
title | Immunosuppressive therapy for aplastic anemia: a single-center experience from western India |
title_full | Immunosuppressive therapy for aplastic anemia: a single-center experience from western India |
title_fullStr | Immunosuppressive therapy for aplastic anemia: a single-center experience from western India |
title_full_unstemmed | Immunosuppressive therapy for aplastic anemia: a single-center experience from western India |
title_short | Immunosuppressive therapy for aplastic anemia: a single-center experience from western India |
title_sort | immunosuppressive therapy for aplastic anemia: a single-center experience from western india |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6334724/ https://www.ncbi.nlm.nih.gov/pubmed/30173288 http://dx.doi.org/10.1007/s00277-018-3487-2 |
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