Time between inhibitor detection and start of immune tolerance induction: Association with outcome in the BrazIT study.
| Autor: | Camelo RM; Faculty of Medicine, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil.; Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, the Netherlands., Dias MM; Faculty of Medicine, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil., Caram-Deelder C; Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, the Netherlands.; Center of Clinical Transfusion Research, Sanquin Research, Leiden, The Netherlands., Gouw S; Amsterdam UMC, University of Amsterdam, Department of Pediatric Hematology, Amsterdam, the Netherlands., de Magalhães LP; Faculty of Medicine, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil., Zuccherato LW; Faculty of Medicine, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil., Jardim LL; Faculty of Medicine, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil.; Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, the Netherlands., de Oliveira AG; Fundação HEMOMINAS, Belo Horizonte, Brazil., de Albuquerque Ribeiro R; Centro de Hematologia e Hemoterapia do Ceará (HEMOCE), Fortaleza, Brazil.; Hospital Universitário Walter Cantídeo, Universidade Federal do Ceará, Fortaleza, Brazil., Franco VKB; Centro de Hematologia e Hemoterapia de Santa Catarina (HEMOSC), Florianópolis, Brazil., do Rosário Ferraz Roberti M; Hemocentro de Goiás (HEMOGO), Goiânia, Brazil.; Faculty of Medicine, Universidade Federal de Goiás, Goiânia, Brazil., de Araújo Callado FMR; Fundação de Hematologia e Hemoterapia de Pernambuco (HEMOPE), Recife, Brazil., Etto LY; Hemocentro da Paraíba (HEMOÍBA), João Pessoa, Brazil.; Department of Internal Medicine, Center of Medical Sciences, Universidade Federal da Paraíba, João Pessoa, Brazil., de Cerqueira MAF; Centro de Hematologia e Hemoterapia do Piauí (HEMOPI), Teresina, Brazil., Cerqueira MH; Instituto de Hematologia do Estado do Rio de Janeiro (HEMORIO), Rio de Janeiro, Brazil., Lorenzato CS; Coagulopathy Clinic, Hemocentro do Paraná (HEMEPAR), Curitiba, Brazil., de Souza IS; Department of Clinical Haematology, Centro de Hematologia e Hemoterapia do Pará (HEMOPA), Belém, Brazil.; Universitary Hospital João de Barros Barreto, Universidade Federal do Pará, Belém, Brazil., Serafim ÉSS; Hemocentro Dalton Cunha (HEMONORTE), Natal, Brazil.; Liga Mossoroense de Combate ao Câncer, Mossoró, Brazil., Garcia AA; Centro de Sangue de São José do Rio Preto, São José do Rio Preto, Brazil.; Fundação Faculdade Regional de Medicina de São José do Rio Preto, São José do Rio Preto, Brazil., Anegawa TH; Centro de Hematologia Regional de Londrina (HEMEPAR Londrina), Londrina, Brazil.; Faculty of Medicine, Universidade Estadual de Londrina, Londrina, Brazil., Neves DCF; Fundação Hemocentro de Rondônia (FHEMERON), Porto Velho, Brazil.; Department of Medicine, Universidade de Rondônia, Porto Velho, Brazil., Tan DM; Department of Paediatric Onco-haematology, Faculdade de Medicina de Marília, Marília, Brazil., van der Bom J; Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, the Netherlands., Rezende SM; Faculty of Medicine, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil. |
|---|---|
| Jazyk: | angličtina |
| Zdroj: | Journal of thrombosis and haemostasis : JTH [J Thromb Haemost] 2022 Nov; Vol. 20 (11), pp. 2526-2537. Date of Electronic Publication: 2022 Sep 26. |
| DOI: | 10.1111/jth.15878 |
| Abstrakt: | Background: Immune tolerance induction (ITI) is the treatment of choice for eradication of anti-factor VIII (FVIII) neutralizing alloantibodies (inhibitors) in people with inherited hemophilia A and high-responding inhibitor (PwHA-HRi). The association between ITI outcome and time elapsed between inhibitor detection and start of ITI (∆t Objective: The aim of this study was to evaluate this association among a large cohort of severe PwHA-HRi. Methods: Severe (factor VIII activity level <1%) PwHA-HRi on ITI (n = 142) were enrolled in 15 hemophilia treatment centers. PwHA-HRi were treated according to the Brazilian ITI Protocol. ITI outcomes were defined as success (i.e., recovered responsiveness to exogenous FVIII) and failure (i.e., no responsiveness to exogenous FVIII and requirement of bypassing agents to control bleeding). Results: Median ages at inhibitor detection and at ITI start were 3.2 years (interquartile range [IQR], 1.6-8.1) and 6.9 years [IQR, 2.6-20.1), respectively. PwHA-HRi were stratified according to ∆t Conclusion: In conclusion, delayed ITI start is not associated with failure of ITI in PwHA-HRi. Therefore, ITI should be offered for these patients, regardless of the time elapsed between the detection of inhibitor and the ITI start. (© 2022 International Society on Thrombosis and Haemostasis.) |
| Databáze: | MEDLINE |
| Externí odkaz: |
|
Plný text