1. Time between inhibitor detection and start of immune tolerance induction: Association with outcome in the BrazIT study
- Author
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Camelo, R.M., Dias, M.M., Caram-Deelder, C., Gouw, S., Magalhaes, L.P. de, Zuccherato, L.W., Jardim, L.L., Oliveira, A.G. de, Ribeiro, R.D., Franco, V.K.B., Roberti, M.D.F., Callado, F.M.R.D., Etto, L.Y., Cerqueira, M.A.F. de, Cerqueira, M.H., Lorenzato, C.S., Souza, I.S. de, Serafim, E.S.S., Garcia, A.A., Anegawa, T.H., Neves, D.C.F., Tan, D.M., Bom, J. van der, Rezende, S.M., Brazilian Immune Tolerance BraziT, Paediatric Haematology, and ARD - Amsterdam Reproduction and Development
- Subjects
immune tolerance induction ,Infant ,Hemorrhage ,Hematology ,Hemostatics ,inhibitor ,risk factor ,Isoantibodies ,Child, Preschool ,Immune Tolerance ,outcome ,Humans ,hemophilia A ,Child - Abstract
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 ( increment t(inhi-ITI)) is debatable. 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 0.6-1.7 year), third (>1.7-9.2 years), and fourth quartile (>9.2-24.5 years). The overall success rate was 65.5% (93/142), with no difference among first, second, third, and fourth quartiles (62.9%, 69.4%, 58.3%, and 71.4%, respectively) even after adjusting the analyses for potential confounders. 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.
- Published
- 2022