Affordable Access

deepdyve-link
Publisher Website

Early response to caplacizumab and rituximab after anaphylaxis to Octaplas plasma in a patient with thrombotic thrombocytopenic purpura.

Authors
  • Perrone, Salvatore1
  • Passucci, Mauro2
  • Ortu La Barbera, Elettra1
  • Capriata, Marcello2
  • Ferretti, Antonietta3
  • Mecozzi, Alessandra4
  • Giovangrossi, Piera5
  • Equitani, Francesco5
  • Cimino, Giuseppe1, 2
  • 1 Hematology, Polo Universitario Pontino, S.M. Goretti Hospital, Latina, Italy. , (Italy)
  • 2 Department of Translational and Precision Medicine, University "La Sapienza", Rome, Italy. , (Italy)
  • 3 Haemorrhagic and Thrombotic Diseases Service, Area of Hematology, Fondazione Policlinico Universitario "A. Gemelli", IRCCS, Rome, Italy. , (Italy)
  • 4 Pharmacy, Polo Universitario Pontino, S.M. Goretti Hospital, Latina, Italy. , (Italy)
  • 5 Transfusion Medicine and Immuno-Hematology Unit, S. M. Goretti Hospital, Latina, Italy. , (Italy)
Type
Published Article
Journal
Journal of clinical apheresis
Publication Date
Jun 01, 2021
Volume
36
Issue
3
Pages
499–504
Identifiers
DOI: 10.1002/jca.21877
PMID: 33459440
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

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. © 2021 Wiley Periodicals LLC.

Report this publication

Statistics

Seen <100 times