2021
DOI: 10.1111/jth.15419
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Treatment‐related risk factors for inhibitor development in non‐severe hemophilia A after 50 cumulative exposure days: A case‐control study

Abstract: Background: Non-severe hemophilia A patients have a life-long inhibitor risk. Yet, no studies have analyzed risk factors for inhibitor development after 50 factor VIII (FVIII) exposure days (EDs).Objectives: This case-control study investigated treatment-related risk factors for inhibitor development in non-severe hemophilia A and assessed whether these risk factors were different for early versus late inhibitor development.This is an open access article under the terms of the Creative Commons Attribution-NonC… Show more

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Cited by 10 publications
(9 citation statements)
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“…Approximately 25–40% of patients with severe hemophilia A acquire neutralizing antibodies (inhibitors) against FVIII, making them resistant to FVIII replacement therapy [ 5 ]. Notably, young patients usually develop inhibitors during the first 20–40 exposure days to FVIII replacement therapy [ 11 ]. Moreover, the only technique that has been proven to eradicate inhibitors is ITI, which is a difficult parental decision since it entails regularly injecting FVIII concentrates and invasive catheterization [ 12 ].…”
Section: Discussionmentioning
confidence: 99%
“…Approximately 25–40% of patients with severe hemophilia A acquire neutralizing antibodies (inhibitors) against FVIII, making them resistant to FVIII replacement therapy [ 5 ]. Notably, young patients usually develop inhibitors during the first 20–40 exposure days to FVIII replacement therapy [ 11 ]. Moreover, the only technique that has been proven to eradicate inhibitors is ITI, which is a difficult parental decision since it entails regularly injecting FVIII concentrates and invasive catheterization [ 12 ].…”
Section: Discussionmentioning
confidence: 99%
“…Combined treatment with factor concentrates and desmopressin could reduce the need for high peak FVIII doses, thereby reducing the risk of inhibitor development. 30 , 31 Therefore, future research is needed to explore reasons for potential underuse of desmopressin from both a patient and physician perspective. If side effects prevent patients from using desmopressin, further studies should explore potential opportunities to improve motivation for desmopressin.…”
Section: Discussionmentioning
confidence: 99%
“…Suboptimal use of desmopressin should especially be addressed in patients with a high risk of inhibitor development. Combined treatment with factor concentrates and desmopressin could reduce the need for high peak FVIII doses, thereby reducing the risk of inhibitor development 30,31 . Therefore, future research is needed to explore reasons for potential underuse of desmopressin from both a patient and physician perspective.…”
Section: Discussionmentioning
confidence: 99%
“…Age-related immune dysregulation and late loss of tolerance could be particularly relevant for the nonsevere hemophilia cohort, as exposure to therapeutic FVIII concentrates is distributed throughout a lifetime and often skewed toward later decades for elective operative interventions. Moreover, both high-dose FVIII treatment and surgical interventions could increase the risk of inhibitor development in nonsevere HA patients ( 39 , 45 ). In the INSIGHT study, the all-cause mortality rate in nonsevere HA patients with inhibitors was more than five times higher compared to those without inhibitors ( 46 ).…”
Section: Pathophysiology Of Fviii Inhibitor Developmentmentioning
confidence: 99%