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Early response to caplacizumab and rituximab after anaphylaxis to Octaplas plasma in a patient with thrombotic thrombocytopenic purpura.
Perrone, Salvatore; Passucci, Mauro; Ortu La Barbera, Elettra; Capriata, Marcello; Ferretti, Antonietta; Mecozzi, Alessandra; Giovangrossi, Piera; Equitani, Francesco; Cimino, Giuseppe.
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  • Perrone S; Hematology, Polo Universitario Pontino, S.M. Goretti Hospital, Latina, Italy.
  • Passucci M; Department of Translational and Precision Medicine, University "La Sapienza", Rome, Italy.
  • Ortu La Barbera E; Hematology, Polo Universitario Pontino, S.M. Goretti Hospital, Latina, Italy.
  • Capriata M; Department of Translational and Precision Medicine, University "La Sapienza", Rome, Italy.
  • Ferretti A; Haemorrhagic and Thrombotic Diseases Service, Area of Hematology, Fondazione Policlinico Universitario "A. Gemelli", IRCCS, Rome, Italy.
  • Mecozzi A; Pharmacy, Polo Universitario Pontino, S.M. Goretti Hospital, Latina, Italy.
  • Giovangrossi P; Transfusion Medicine and Immuno-Hematology Unit, S. M. Goretti Hospital, Latina, Italy.
  • Equitani F; Transfusion Medicine and Immuno-Hematology Unit, S. M. Goretti Hospital, Latina, Italy.
  • Cimino G; Hematology, Polo Universitario Pontino, S.M. Goretti Hospital, Latina, Italy.
J Clin Apher ; 36(3): 499-504, 2021 Jun.
Article en En | MEDLINE | ID: mdl-33459440
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.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Púrpura Trombocitopénica Trombótica / Plasmaféresis / Anticuerpos de Dominio Único / Rituximab Tipo de estudio: Etiology_studies / Risk_factors_studies Límite: Adult / Female / Humans Idioma: En Revista: J Clin Apher Año: 2021 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Estados Unidos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Púrpura Trombocitopénica Trombótica / Plasmaféresis / Anticuerpos de Dominio Único / Rituximab Tipo de estudio: Etiology_studies / Risk_factors_studies Límite: Adult / Female / Humans Idioma: En Revista: J Clin Apher Año: 2021 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Estados Unidos