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Feasibility of therapeutic plasma exchange-based combination therapy in the treatment of acquired hemophilia A: A retrospective 6 case series

Poor availability and a lack of affordability of bypassing agents (recombinant activated factor VII and activated prothrombin complex concentrate) in west China prompted us to investigate an alternative cost-effective combination therapy. We aimed to explore the feasibility of therapeutic plasma exc...

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Autores principales: Wang, Lin-Yue, Shen, Yan, Zeng, Han-Qing, Zhang, Ying, Lou, Shi-Feng, Deng, Jian-Chuan, Luo, Yun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8294921/
https://www.ncbi.nlm.nih.gov/pubmed/34398013
http://dx.doi.org/10.1097/MD.0000000000026587
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author Wang, Lin-Yue
Shen, Yan
Zeng, Han-Qing
Zhang, Ying
Lou, Shi-Feng
Deng, Jian-Chuan
Luo, Yun
author_facet Wang, Lin-Yue
Shen, Yan
Zeng, Han-Qing
Zhang, Ying
Lou, Shi-Feng
Deng, Jian-Chuan
Luo, Yun
author_sort Wang, Lin-Yue
collection PubMed
description Poor availability and a lack of affordability of bypassing agents (recombinant activated factor VII and activated prothrombin complex concentrate) in west China prompted us to investigate an alternative cost-effective combination therapy. We aimed to explore the feasibility of therapeutic plasma exchange (TPE)-based combination therapy in the treatment of acquired hemophilia A (AHA). We retrospectively investigated the clinical features of AHA in 6 patients who were treated with a combination of TPE, corticosteroids, and rituximab in our department for 9 years between January, 2011 and December, 2019. We examined 1 male and 5 female patients. The median age at diagnosis of AHA was 51 years (18–66 years). In all patients, FVIII activity levels were low (median: 1.5%; 1–3%), FVIII inhibitor titers were high (median: 24.5 BU/mL; 13.2–48.6 BU/mL), and activated partial thromboplastin time was markedly prolonged (median: 99.4 s; 60.9–110.1 s). They underwent 2 to 8 cycles of plasma exchange and were given varying combinations of dexamethasone, methylprednisolone, prednisone, and rituximab. After TPE bleeding gradually stopped, and activated partial thromboplastin time decreased. After 3 months of treatment, FVIII inhibitors completely disappeared. TPE when combined with corticosteroids and rituximab, as adjunctive immunosuppressive agents, may be an effective and reliable treatment for AHA. When there is no alternative, intensive first-line treatment including TPE may be lifesaving.
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spelling pubmed-82949212021-07-24 Feasibility of therapeutic plasma exchange-based combination therapy in the treatment of acquired hemophilia A: A retrospective 6 case series Wang, Lin-Yue Shen, Yan Zeng, Han-Qing Zhang, Ying Lou, Shi-Feng Deng, Jian-Chuan Luo, Yun Medicine (Baltimore) 4800 Poor availability and a lack of affordability of bypassing agents (recombinant activated factor VII and activated prothrombin complex concentrate) in west China prompted us to investigate an alternative cost-effective combination therapy. We aimed to explore the feasibility of therapeutic plasma exchange (TPE)-based combination therapy in the treatment of acquired hemophilia A (AHA). We retrospectively investigated the clinical features of AHA in 6 patients who were treated with a combination of TPE, corticosteroids, and rituximab in our department for 9 years between January, 2011 and December, 2019. We examined 1 male and 5 female patients. The median age at diagnosis of AHA was 51 years (18–66 years). In all patients, FVIII activity levels were low (median: 1.5%; 1–3%), FVIII inhibitor titers were high (median: 24.5 BU/mL; 13.2–48.6 BU/mL), and activated partial thromboplastin time was markedly prolonged (median: 99.4 s; 60.9–110.1 s). They underwent 2 to 8 cycles of plasma exchange and were given varying combinations of dexamethasone, methylprednisolone, prednisone, and rituximab. After TPE bleeding gradually stopped, and activated partial thromboplastin time decreased. After 3 months of treatment, FVIII inhibitors completely disappeared. TPE when combined with corticosteroids and rituximab, as adjunctive immunosuppressive agents, may be an effective and reliable treatment for AHA. When there is no alternative, intensive first-line treatment including TPE may be lifesaving. Lippincott Williams & Wilkins 2021-07-23 /pmc/articles/PMC8294921/ /pubmed/34398013 http://dx.doi.org/10.1097/MD.0000000000026587 Text en Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0 (https://creativecommons.org/licenses/by/4.0/)
spellingShingle 4800
Wang, Lin-Yue
Shen, Yan
Zeng, Han-Qing
Zhang, Ying
Lou, Shi-Feng
Deng, Jian-Chuan
Luo, Yun
Feasibility of therapeutic plasma exchange-based combination therapy in the treatment of acquired hemophilia A: A retrospective 6 case series
title Feasibility of therapeutic plasma exchange-based combination therapy in the treatment of acquired hemophilia A: A retrospective 6 case series
title_full Feasibility of therapeutic plasma exchange-based combination therapy in the treatment of acquired hemophilia A: A retrospective 6 case series
title_fullStr Feasibility of therapeutic plasma exchange-based combination therapy in the treatment of acquired hemophilia A: A retrospective 6 case series
title_full_unstemmed Feasibility of therapeutic plasma exchange-based combination therapy in the treatment of acquired hemophilia A: A retrospective 6 case series
title_short Feasibility of therapeutic plasma exchange-based combination therapy in the treatment of acquired hemophilia A: A retrospective 6 case series
title_sort feasibility of therapeutic plasma exchange-based combination therapy in the treatment of acquired hemophilia a: a retrospective 6 case series
topic 4800
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8294921/
https://www.ncbi.nlm.nih.gov/pubmed/34398013
http://dx.doi.org/10.1097/MD.0000000000026587
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