Portal de investigación
ADAMTS13 recovery in acute thrombotic thrombocytopenic purpura after caplacizumab therapy
Fecha de publicación:
Fecha Ahead of Print:
Autores de IIS La Fe
Participantes ajenos a IIS La Fe
- Mingot-Castellano, ME
- García-Candel, F
- Martínez-Nieto, J
- García-Arroba, J
- Paciello-Coronel, ML
- Valcárcel-Ferreiras, D
- Jiménez, M
- Cid, J
- Lozano, M
- García-Gala, JM
- Angós-Vazquez, S
- Vara-Pampliega, M
- Guerra-Domínguez, L
- Avila-Idrobo, LF
- Oliva-Hernandez, A
- Zalba-Marcos, S
- Tallón-Ruiz, I
- Ortega-Sánchez, S
- Goterris-Viciedo, R
- Moreno-Jiménez, G
- Domínguez-Acosta, L
- Araiz-Ramírez, M
- Hernández-Mateos, L
- Flores-Ballesteros, E
- del Río-Garma, J
- Pascual-Izquierdo, C
- Spanish Apheresis Grp
- Spanish Thrombotic Thrombocytopenic Purpura Registry
Grupos
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 caplacizumabtreated 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 P = .033). This fi nding could be related to a significantly fi cantly 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 noncaplacizumab-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 fi cial effects by shortening PEX requirement without major safety concerns.
Datos de la publicación
- ISSN/ISSNe:
- 0006-4971, 1528-0020
- Tipo:
- Article
- Páginas:
- 1807-1815
- PubMed:
- 38237147
- Factor de Impacto:
- 4,834 SCImago ℠
- Cuartil:
- Q1 SCImago ℠
BLOOD ELSEVIER
Citas Recibidas en Web of Science: 12
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Filiaciones
Campos de Estudio
Cita
Mingot ME,García F,Martínez J,García J,DE LA RUBIA J,GÓMEZ I,Paciello ML,Valcárcel D,Jiménez M,Cid J,Lozano M,García JM,Angós S,Vara M,Guerra L,Avila LF,Oliva A,Zalba S,Tallón I,Ortega S,Goterris R,Moreno G,Domínguez L,Araiz M,Hernández L,Flores E,del Río J,Pascual C,Spanish GRP,Spanish Thrombotic Thrombocytopenic Purpura R. ADAMTS13 recovery in acute thrombotic thrombocytopenic purpura after caplacizumab therapy. Blood. 2024. 143. (18):p. 1807-1815. IF:23,100. (1).