Combined immunosuppression for acquired hemophilia A : CyDRi is a highly effective low-toxicity regimen
© 2022 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved..
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.
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CommentIn: Blood. 2022 Nov 3;140(18):1923-1924. - PMID 36326794 |
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| Medienart: |
E-Artikel |
| Erscheinungsjahr: |
2022 |
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| Erschienen: |
2022 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:140 |
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| Enthalten in: |
Blood - 140(2022), 18 vom: 03. Nov., Seite 1983-1992 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Simon, Barbara [VerfasserIn] |
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| Links: |
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| Themen: |
8N3DW7272P |
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| Anmerkungen: |
Date Completed 07.11.2022 Date Revised 19.11.2023 published: Print CommentIn: Blood. 2022 Nov 3;140(18):1923-1924. - PMID 36326794 Citation Status MEDLINE |
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| doi: |
10.1182/blood.2022016873 |
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| 245 | 1 | 0 | |a Combined immunosuppression for acquired hemophilia A |b CyDRi is a highly effective low-toxicity regimen |
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| 500 | |a CommentIn: Blood. 2022 Nov 3;140(18):1923-1924. - PMID 36326794 | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2022 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved. | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Journal Article | |
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| 700 | 1 | |a Ceglédi, Andrea |e verfasserin |4 aut | |
| 700 | 1 | |a Dolgos, János |e verfasserin |4 aut | |
| 700 | 1 | |a Farkas, Péter |e verfasserin |4 aut | |
| 700 | 1 | |a Gaddh, Manila |e verfasserin |4 aut | |
| 700 | 1 | |a Hankó, László |e verfasserin |4 aut | |
| 700 | 1 | |a Horváth, Robert |e verfasserin |4 aut | |
| 700 | 1 | |a Kaposi, Ambrus |e verfasserin |4 aut | |
| 700 | 1 | |a Magyar, Lászlóné |e verfasserin |4 aut | |
| 700 | 1 | |a Masszi, Tamás |e verfasserin |4 aut | |
| 700 | 1 | |a Szederjesi, Attila |e verfasserin |4 aut | |
| 700 | 1 | |a Wohner, Nikolett |e verfasserin |4 aut | |
| 700 | 1 | |a Bodó, Imre |e verfasserin |4 aut | |
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