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Publications

Journal Article Research Support, Non-U.S. Gov't Ppublish 7 citations

PubMed: 38237147 DOI: 10.1182/blood.2023022725

ADAMTS13 recovery in acute thrombotic thrombocytopenic purpura after caplacizumab therapy.

Blood 143(18):1807-1815

Authors: María-Eva Mingot-Castellano, Faustino García-Candel, Jorge Martínez-Nieto, José García-Arroba, Javier de la Rubia-Comos, Inés Gómez-Seguí, María-Liz Paciello-Coronel, David Valcárcel-Ferreiras, Moraima Jiménez, Joan Cid, Miquel Lozano, José-María García-Gala, Sonia Angós-Vazquez, Miriam Vara-Pampliega, Luisa Guerra-Domínguez, Laura-Francisca Ávila-Idrobo, Ana Oliva-Hernandez, Saioa Zalba-Marcos, Inmaculada Tallón-Ruiz, Sandra Ortega-Sánchez, Rosa Goterris-Viciedo, Gemma Moreno-Jiménez, Lourdes Domínguez-Acosta, María Araiz-Ramírez, Luis Hernández-Mateos, Elena Flores-Ballesteros, Julio Del Río-Garma, Cristina Pascual-Izquierdo

Affiliations

  • Department of Hematology, Hospital Universitario Virgen del Rocío, Seville, Spain.
  • Instituto de Biomedicina de Sevilla, Seville, Spain.
  • Department of Hematology, Hospital Universitario Virgen de la Arrixaca, Murcia, Spain.
  • Department of Hematology, Hospital Clínico San Carlos, Madrid, Spain.
  • Banc de Sang i Teixits, Hospital Universitari Joan XXIII, Tarragona, Spain.
  • Department of Hematology, Hospital Universitario La Fe, Universidad Católica San Vicente Mártir, IIS La Fe, Valencia, Spain.
  • Department of Hematology, Hospital Universitario 12 de Octubre, Madrid, Spain.
  • Department of Hematology, Hospital Universitari Vall D'Hebron, Barcelona, Spain.
  • Department of Hemotherapy and Hemostasis, Apheresis and Cellular Therapy Unit, Clinical Institute of Hematological and Oncological Diseases, Instituto de Investigaciones Biomédicas August Pi i Sunyer, Hospital Clínic de Barcelona, Barcelona, Spain.
  • Department of Hematology, Hospital Universitario Central de Asturias, Institute for Bio Health Investigation of Asturias, Oviedo, Spain.
  • Department of Hematology, Hospital Universitario de Zaragoza, Zaragoza, Spain.
  • Department of Hematology, Hospital Universitario Cruces, Barakaldo, Spain.
  • Department of Hematology, Hospital Universitario Dr. Negrín, Las Palmas de Gran Canaria, Spain.
  • Department of Hematology, Hospital Universitario de Canarias, Tenerife, Spain.
  • Department of Hematology, Hospital Universitario Nuestra Señora de la Candelaria, Tenerife, Spain.
  • Department of Hematology, Hospital Universitario de Navarra, Pamplona, Spain.
  • Department of Hematology, Hospital Universitario Virgen Macarena, Seville, Spain.
  • Banc de Sang i Teixits, L'Hospitalet de Llobregat, Barcelona, Spain.
  • Department of Hematology, Hospital Clínico Universitario de Valencia, Valencia, Spain.
  • Department of Hematology, Hospital Universitario Ramón y Cajal, Madrid, Spain.
  • Department of Hematology, Hospital Universitario de Jerez de la Frontera, Jerez de la Frontera, Spain.
  • Department of Hematology, Hospital Universitario Donostia, Donostia, Spain.
  • Department of Hematology, Hospital General Universitario de Alicante, Alicante, Spain.
  • Department of Hematology, Hospital Universitario Principe de Asturias, Alcalá de Henares, Madrid, Spain.
  • Department of Hematology, Complejo Hospitalario Universitario de Ourense, Ourense, Spain.
  • Department of Hematology, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
  • Instituto de Investigación Gregorio Marañon, Madrid, Spain.

Abstract

Caplacizumab prevents the interaction between von Willebrand factor and platelets and is used to treat immune thrombotic thrombocytopenic purpura (iTTP). Its administration has been associated with a delay in ADAMTS13 activity restoration after plasma exchange (PEX) suspension. We analyzed the outcomes of 113 iTTP episodes, 75 of which were treated with caplacizumab, in 108 patients from the Spanish Registry of Thrombotic Thrombocytopenic Purpura. Caplacizumab shortened the time to platelet count normalization and reduced PEX requirement, exacerbations, and relapses. There was no difference in the time to achieve ADAMTS13 activity ≥20% after PEX end between caplacizumab-treated and nontreated episodes (median [interquartile range], 14.5 [7.7-27.2] vs 13.0 [8.0-29.0] days, P = .653). However, considering the 36 episodes in which caplacizumab was started ≤3 days after iTTP diagnosis, the time for ADAMTS13 restoration from PEX end was higher than in those episodes in which caplacizumab was started >3 days after iTTP diagnosis (20.0 [12.0-43.0] vs 11.0 [3.5-20.0] days, P = .003) or than in non-caplacizumab-treated episodes (P = .033). This finding could be related to a significantly shorter duration of PEX in early caplacizumab-treated episodes than in late caplacizumab-treated episodes (5.5 [4.0-9.0] vs 15.0 [11.0-21.5] days, P < .001) or non-caplacizumab-treated episodes (11.0 [6.0-26.0] days, P < .001). There were no differences in time to ADAMTS-13 restoration from PEX start (28.0 [17.2-47.5], 27.0 [19.0-37.5] and 29.5 [15.2-45.0] days in early caplacizumab-treated, late caplacizumab-treated and non-caplacizumab-treated episodes). Early administered caplacizumab does not prevent the requirement for immunosuppression but has beneficial effects by shortening PEX requirement without major safety concerns.

MeSH

Registries, Purpura, Thrombotic Thrombocytopenic, Hematology, Recycling, Diagnosis, Therapeutics, Blood Platelets, Purpura, Thrombocytopenic

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