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ADAMTS13 RECOVERY IN ACUTE THROMBOTIC THROMBOCYTOPENIC PURPURA AFTER CAPLACIZUMAB THERAPY. THE SPANISH REGISTRY. 2024
Maria Eva Mingot-Castellano, and
Faustino García-Candel, and
Jorge Martinez Nieto, and
José García-Arroba Peinado, and
Javier de la Rubia Comos, and
Ines Gomez-Segui, and
María-Liz Paciello-Coronel, and
David Valcarcel, and
Moraima Jiménez, and
Joan Cid, and
Miquel Lozano, and
José-María García-Gala, and
Sonia Angós-Vazquez, and
Miriam Vara Pampliega, and
Luisa Guerra Dominguez, and
Laura-Francisca Ávila-Idrovo, and
Ana Oliva Hernandez, and
Saioa Zalba, and
Inmaculada Tallón Ruiz, and
Sandra Ortega Sánchez, and
Rosa Goterris, and
Maria Gemma Moreno Jimenez, and
Lourdes Domínguez-Acosta, and
María Araiz-Ramírez, and
Luis M Hernández-Mateo, and
Elena Flores Ballester, and
Julio Del Rio-Garma, and
Cristina Pascual Izquierdo
Hospital Universitario Virgen del Rocio, Instituto de Biomedicina de Sevilla, Sevilla, Spain.
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 non-treated episodes (14.5 [7.7-27.2] vs. 13.0 [8.0-29.0] days, median [IQR], P=.653). However, considering the 36 episodes where caplacizumab started <=3 days after iTTP diagnosis, the time to ADAMTS13 restoration from PEX end was higher than in those episodes where caplacizumab 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.
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