Early response to caplacizumab and rituximab after anaphylaxis to Octaplas plasma in a patient with thrombotic thrombocytopenic purpura
© 2021 Wiley Periodicals LLC..
Management of aTTP in patients who refuse or are intolerant to plasma remains challenging, but new drugs can be implemented with success. A 39-year-old woman presented to the Emergency department for bruises at the upper and lower limbs together with worsening anemia and thrombocytopenia; PLASMIC score was seven, indicative of high risk to have a thrombotic microangiopathy due to severe ADAMTS-13 deficiency: indeed, it was 1.4%. We immediately started Plasma Exchange, but after the third procedure she developed severe anaphylaxis to Octaplas plasma, so PEXs were discontinued. We proceeded to a salvage strategy with rituximab and caplacizumab that was rapidly effective to resolve symptoms and hemolysis. It has been already reported a case in which a patient developed severe reactions to fresh-frozen plasma that required discontinuation of PEX. Differently from this case, our patient was already using the less immunogenic pooled plasma units Octaplas, therefore a strategy with caplacizumab was the only available option. Moreover, rituximab is associated with a shorter time to obtain a durable remission in aTTP and a faster time (15 days) to final ADAMTS13 activity recovery >10%. To our knowledge, this is the first case of early discontinuation of caplacizumab in a patient allergic to PEX by actively monitoring ADAMTS13 activity, allowing optimization of healthcare resources during COVID-19 pandemic.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2021 |
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| Erschienen: |
2021 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:36 |
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| Enthalten in: |
Journal of clinical apheresis - 36(2021), 3 vom: 21. Juni, Seite 499-504 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Perrone, Salvatore [VerfasserIn] |
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| Links: |
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| Anmerkungen: |
Date Completed 07.07.2021 Date Revised 07.07.2021 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1002/jca.21877 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM320220494 |
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| 245 | 1 | 0 | |a Early response to caplacizumab and rituximab after anaphylaxis to Octaplas plasma in a patient with thrombotic thrombocytopenic purpura |
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| 520 | |a © 2021 Wiley Periodicals LLC. | ||
| 520 | |a Management of aTTP in patients who refuse or are intolerant to plasma remains challenging, but new drugs can be implemented with success. A 39-year-old woman presented to the Emergency department for bruises at the upper and lower limbs together with worsening anemia and thrombocytopenia; PLASMIC score was seven, indicative of high risk to have a thrombotic microangiopathy due to severe ADAMTS-13 deficiency: indeed, it was 1.4%. We immediately started Plasma Exchange, but after the third procedure she developed severe anaphylaxis to Octaplas plasma, so PEXs were discontinued. We proceeded to a salvage strategy with rituximab and caplacizumab that was rapidly effective to resolve symptoms and hemolysis. It has been already reported a case in which a patient developed severe reactions to fresh-frozen plasma that required discontinuation of PEX. Differently from this case, our patient was already using the less immunogenic pooled plasma units Octaplas, therefore a strategy with caplacizumab was the only available option. Moreover, rituximab is associated with a shorter time to obtain a durable remission in aTTP and a faster time (15 days) to final ADAMTS13 activity recovery >10%. To our knowledge, this is the first case of early discontinuation of caplacizumab in a patient allergic to PEX by actively monitoring ADAMTS13 activity, allowing optimization of healthcare resources during COVID-19 pandemic | ||
| 650 | 4 | |a Case Reports | |
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| 650 | 4 | |a Moschcowitz syndrome | |
| 650 | 4 | |a caplacizumab | |
| 650 | 4 | |a covid19 | |
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| 650 | 4 | |a thrombotic thrombocytopenic purpura | |
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| 700 | 1 | |a Passucci, Mauro |e verfasserin |4 aut | |
| 700 | 1 | |a Ortu La Barbera, Elettra |e verfasserin |4 aut | |
| 700 | 1 | |a Capriata, Marcello |e verfasserin |4 aut | |
| 700 | 1 | |a Ferretti, Antonietta |e verfasserin |4 aut | |
| 700 | 1 | |a Mecozzi, Alessandra |e verfasserin |4 aut | |
| 700 | 1 | |a Giovangrossi, Piera |e verfasserin |4 aut | |
| 700 | 1 | |a Equitani, Francesco |e verfasserin |4 aut | |
| 700 | 1 | |a Cimino, Giuseppe |e verfasserin |4 aut | |
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