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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...

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Autores principales: Shah, Sandip, Jain, Preetam, Shah, Kamlesh, Patel, Kinnari, Parikh, Sonia, Patel, Apurva, Panchal, Harsha, Anand, Asha
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2018
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.
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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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