Cargando…
Combined immunosuppression for acquired hemophilia A: CyDRi is a highly effective low-toxicity regimen
Acquired hemophilia A (AHA) is a rare severe autoimmune bleeding disorder with significant morbidity and mortality. Although critical for disease control, there is no consensus for the best immunosuppressive regimen. Most authors use steroids first line, followed by other agents for steroid failures...
Autores principales: | , , , , , , , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The American Society of Hematology
2022
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10653022/ https://www.ncbi.nlm.nih.gov/pubmed/35930747 http://dx.doi.org/10.1182/blood.2022016873 |
_version_ | 1785136334287929344 |
---|---|
author | Simon, Barbara Ceglédi, Andrea Dolgos, János Farkas, Péter Gaddh, Manila Hankó, László Horváth, Robert Kaposi, Ambrus Magyar, Lászlóné Masszi, Tamás Szederjesi, Attila Wohner, Nikolett Bodó, Imre |
author_facet | Simon, Barbara Ceglédi, Andrea Dolgos, János Farkas, Péter Gaddh, Manila Hankó, László Horváth, Robert Kaposi, Ambrus Magyar, Lászlóné Masszi, Tamás Szederjesi, Attila Wohner, Nikolett Bodó, Imre |
author_sort | Simon, Barbara |
collection | PubMed |
description | Acquired hemophilia A (AHA) is a rare severe autoimmune bleeding disorder with significant morbidity and mortality. Although critical for disease control, there is no consensus for the best immunosuppressive regimen. Most authors use steroids first line, followed by other agents for steroid failures. Upfront combined regimens offer the advantage of reduced steroid exposure and toxicity as well as increased efficacy. We retrospectively analyzed data from 32 patients with AHA treated on an identical such institutional protocol: cyclophosphamide 1000 mg on days 1 and 22, dexamethasone 40 mg on days 1, 8, 15, and 22, and rituximab 100 mg on days 1, 8, 15, and 22 (the regimen was termed CyDRi). All patients received at least 1 cycle of CyDRi. If necessary, CyDRi was repeated until remission, no sooner than day 43 of the previous cycle. Bleeding control was rapidly achieved. The median time for bleeding control was 15.5 days (range, 0-429 days; interquartile range, 2.5-29.5 days). Thirty-one (96.8%) of 32 patients achieved durable complete remission (CR); 29 (90.6%) of 32 patients were alive at last follow-up, all of them in CR. The median time to reach first CR was 77 days (range, 19-939 days; interquartile range, 31-115 days). Toxicity and side effects were acceptable and milder than those of commonly used, prolonged steroid therapies. In conclusion, the CyDRi regimen produced markedly higher CR rates and overall survival than currently used sequential regimens. Taken together, CyDRi proved to be an attractive option for the immunosuppression of elderly patients with AHA. |
format | Online Article Text |
id | pubmed-10653022 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | The American Society of Hematology |
record_format | MEDLINE/PubMed |
spelling | pubmed-106530222022-08-09 Combined immunosuppression for acquired hemophilia A: CyDRi is a highly effective low-toxicity regimen Simon, Barbara Ceglédi, Andrea Dolgos, János Farkas, Péter Gaddh, Manila Hankó, László Horváth, Robert Kaposi, Ambrus Magyar, Lászlóné Masszi, Tamás Szederjesi, Attila Wohner, Nikolett Bodó, Imre Blood Thrombosis and Hemostasis Acquired hemophilia A (AHA) is a rare severe autoimmune bleeding disorder with significant morbidity and mortality. Although critical for disease control, there is no consensus for the best immunosuppressive regimen. Most authors use steroids first line, followed by other agents for steroid failures. Upfront combined regimens offer the advantage of reduced steroid exposure and toxicity as well as increased efficacy. We retrospectively analyzed data from 32 patients with AHA treated on an identical such institutional protocol: cyclophosphamide 1000 mg on days 1 and 22, dexamethasone 40 mg on days 1, 8, 15, and 22, and rituximab 100 mg on days 1, 8, 15, and 22 (the regimen was termed CyDRi). All patients received at least 1 cycle of CyDRi. If necessary, CyDRi was repeated until remission, no sooner than day 43 of the previous cycle. Bleeding control was rapidly achieved. The median time for bleeding control was 15.5 days (range, 0-429 days; interquartile range, 2.5-29.5 days). Thirty-one (96.8%) of 32 patients achieved durable complete remission (CR); 29 (90.6%) of 32 patients were alive at last follow-up, all of them in CR. The median time to reach first CR was 77 days (range, 19-939 days; interquartile range, 31-115 days). Toxicity and side effects were acceptable and milder than those of commonly used, prolonged steroid therapies. In conclusion, the CyDRi regimen produced markedly higher CR rates and overall survival than currently used sequential regimens. Taken together, CyDRi proved to be an attractive option for the immunosuppression of elderly patients with AHA. The American Society of Hematology 2022-11-03 2022-08-09 /pmc/articles/PMC10653022/ /pubmed/35930747 http://dx.doi.org/10.1182/blood.2022016873 Text en © 2022 by The American Society of Hematology. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Thrombosis and Hemostasis Simon, Barbara Ceglédi, Andrea Dolgos, János Farkas, Péter Gaddh, Manila Hankó, László Horváth, Robert Kaposi, Ambrus Magyar, Lászlóné Masszi, Tamás Szederjesi, Attila Wohner, Nikolett Bodó, Imre Combined immunosuppression for acquired hemophilia A: CyDRi is a highly effective low-toxicity regimen |
title | Combined immunosuppression for acquired hemophilia A: CyDRi is a highly effective low-toxicity regimen |
title_full | Combined immunosuppression for acquired hemophilia A: CyDRi is a highly effective low-toxicity regimen |
title_fullStr | Combined immunosuppression for acquired hemophilia A: CyDRi is a highly effective low-toxicity regimen |
title_full_unstemmed | Combined immunosuppression for acquired hemophilia A: CyDRi is a highly effective low-toxicity regimen |
title_short | Combined immunosuppression for acquired hemophilia A: CyDRi is a highly effective low-toxicity regimen |
title_sort | combined immunosuppression for acquired hemophilia a: cydri is a highly effective low-toxicity regimen |
topic | Thrombosis and Hemostasis |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10653022/ https://www.ncbi.nlm.nih.gov/pubmed/35930747 http://dx.doi.org/10.1182/blood.2022016873 |
work_keys_str_mv | AT simonbarbara combinedimmunosuppressionforacquiredhemophiliaacydriisahighlyeffectivelowtoxicityregimen AT ceglediandrea combinedimmunosuppressionforacquiredhemophiliaacydriisahighlyeffectivelowtoxicityregimen AT dolgosjanos combinedimmunosuppressionforacquiredhemophiliaacydriisahighlyeffectivelowtoxicityregimen AT farkaspeter combinedimmunosuppressionforacquiredhemophiliaacydriisahighlyeffectivelowtoxicityregimen AT gaddhmanila combinedimmunosuppressionforacquiredhemophiliaacydriisahighlyeffectivelowtoxicityregimen AT hankolaszlo combinedimmunosuppressionforacquiredhemophiliaacydriisahighlyeffectivelowtoxicityregimen AT horvathrobert combinedimmunosuppressionforacquiredhemophiliaacydriisahighlyeffectivelowtoxicityregimen AT kaposiambrus combinedimmunosuppressionforacquiredhemophiliaacydriisahighlyeffectivelowtoxicityregimen AT magyarlaszlone combinedimmunosuppressionforacquiredhemophiliaacydriisahighlyeffectivelowtoxicityregimen AT masszitamas combinedimmunosuppressionforacquiredhemophiliaacydriisahighlyeffectivelowtoxicityregimen AT szederjesiattila combinedimmunosuppressionforacquiredhemophiliaacydriisahighlyeffectivelowtoxicityregimen AT wohnernikolett combinedimmunosuppressionforacquiredhemophiliaacydriisahighlyeffectivelowtoxicityregimen AT bodoimre combinedimmunosuppressionforacquiredhemophiliaacydriisahighlyeffectivelowtoxicityregimen |