Mortality in acquired thrombotic thrombocytopenic purpura in the pre-caplacizumab era.

Autor: Del Río-Garma J; Servicio de Hematología, Complejo Hospitalario Universitario de Ourense, Rua Ramon Puga Noguerol sn, 32005, Ourense, Spain. julio.del.rio.garma@sergas.es., Bobillo S; Servicio de Hematología, Hospital Vall d´Hebron, Barcelona, Spain., de la Rubia J; Servicio de Hematología, Hospital Universitario Doctor Peset, Valencia, Spain.; School of Medicine, Catholic Univertity of Valencia, Valencia, Spain., Pascual C; Servicio de Hematología, Hospital Gregorio Marañón, Madrid, Spain., García-Candel F; Servicio de Hematología, Hospital Virgen de La Arrixaca, Murcia, Spain., García-Gala JM; Servicio de Hematología, Hospital Universitario de Asturias, Oviedo, Spain., Gonzalez R; Servicio de Hematología, Hospital Clínico Universitario de A Coruña, A Coruña, Spain., Abril L; Servicio de Hematología, Hospital Germans Trias I Pujol, Badalona, Spain., Vidan J; Servicio de Hematología, Hospital Universitario de León, León, Spain., Gomez MJ; Servicio de Hematología, Hospital Universitario de Móstoles, Móstoles, Spain., Peña F; Servicio de Hematología, Complejo Hospitalario Universitario de Vigo, Vigo, Spain., Arbona C; Servicio de Hematología, Hospital Clínico Universitario de Valencia, Valencia, Spain., Martín-Sanchez J; Servicio de Hematología, Hospital Virgen del Rocío, Sevilla, Spain., Moreno G; Servicio de Hematología, Hospital Ramón y Cajal, Madrid, Spain., Romón I; Servicio de Hematología, Hospital Marqués de Valdecilla, Santander, Spain., Viejo A; Servicio de Hematología, Hospital La Paz, Madrid, Spain., Oliva A; Servicio de Hematología, Hospital Universitario Virgen de La Candelaria, Tenerife, Spain., Linares M; Servicio de Hematología, Hospital Vall d´Hebron, Barcelona, Spain., Salinas R; Hospital del Sagrat Cor, Universitat Internacional de Catalunya and Banc de Sang I Teixits de Catalunya, Barcelona, Spain., Pérez S; Servicio de Hematología, Hospital Clínico Universitario de Valladolid, Valladolid, Spain., Garcia-Erce JA; Banco de Sangre y Tejidos de Navarra. Servicio Navarro de Salud, Pamplona, and Instituto Aragonés de Ciencias de La Salud, Pamplona, Spain., Pereira A; Servicio de Hemoterapia y Hemostasia, Hospital Clínic, Barcelona, Spain.
Jazyk: angličtina
Zdroj: Annals of hematology [Ann Hematol] 2022 Jan; Vol. 101 (1), pp. 59-67. Date of Electronic Publication: 2021 Oct 12.
DOI: 10.1007/s00277-021-04685-8
Abstrakt: Despite the effectiveness of plasma exchange (PEX) and immunosuppressants in the treatment of acquired thrombotic thrombocytopenic purpura (aTTP), a number of patients still die as a result of the disease. Whether caplacizumab could rescue these patients remains still unsettled. The objective of this study was to characterise mortality patterns and prognostic factors in the first episode of aTTP.We queried the Spanish TTP Registry for patients with a diagnosis of aTTP in their presenting episode who fulfilled complete clinical and follow-up data (n = 102). The patients were diagnosed between 2004 and 2018, and all were treated with daily PEX and corticosteroids. Clinical and laboratory data were analysed at diagnosis and during the treatment course.Eight patients (7.7%) died between 12 h and 36 days after presentation, and could be classified into three patterns: death before treatment, early death driven by acute cardiac or neurologic events, and late death due to unremitted aTTP. Stupor or coma at diagnosis and platelet count < 20 × 10 9 /L by the 6th treatment day were independently associated with increased risk of death.Stupor or coma at diagnosis and lack of response to PEX by the 6th day in patients experiencing the first episode of aTTP are strong predictors of mortality. These patients could be rescued by novel agents aimed at halting the microvascular thrombosis until adequate immunosuppression is achieved.
(© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
Databáze: MEDLINE
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