Managing inhibitors is important for long-term patient health
Managing inhibitors is important for patient health
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Patients with hemophilia A or B may develop inhibitors, which are IgG alloantibodies to exogenous clotting factor VIII (FVIII) or factor IX (FIX) that neutralize the function of infused clotting factor concentrates (CFCs). Inhibitors are present more frequently in patients with severe hemophilia.1
Occurrence of inhibitors in hemophilia patients
Hemophilia A
Hemophilia A
Hemophilia B
Hemophilia B
aAmong previously untreated patients
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Risk factors for inhibitor formation in hemophilia A1
Severity of disease
Family history of inhibitors
Black African ancestry
Hispanic ancestry
Genetic variants
High-intensity factor exposure
Importance of detecting and managing inhibitors
According to guidelines from the World Federation of Hemophilia (WFH), controlling bleeds in patients with inhibitors is more challenging—making early identification and careful management of inhibitors crucial to patient health.1
The WFH recommends testing patients with hemophilia A or B for inhibitors:
- After the initial factor exposure
- After intensive factor exposure, e.g., daily exposure for more than 5 days
- When a patient experiences recurrent bleeds or target joint bleeds, despite adequate CFC replacement therapy
- Upon failure to respond to adequate CFC replacement therapy
- After lower than expected factor recovery or half- life after CFC replacement therapy
- Upon suboptimal clinical or laboratory response to CFC replacement therapy
- Before surgery
- Upon suboptimal post-operative response to CFC replacement therapy
CFC=clotting factor concentrate.
(Content above adapted from World Federation of Hemophilia guidelines and used with permission.)
Inhibitors to FVIII or FIX are associated with a higher disease burden, including increased risk of musculoskeletal complications, pain, physical limitations, and treatment challenges.1
– WFH Guidelines for the Management of Hemophilia
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CHwI patients are 2x as likely to be hospitalized for a bleeding complication2
Explore a clinical pathway
See the common steps for treating emergent bleeds in patients with CHwI.
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Learn about MASAC recommendations for CHwI
The National Hemophilia Foundation’s Medical and Scientific Advisory Council (MASAC) guidelines address key areas of hemophilia management, including for patients with inhibitors.
References:
- World Federation of Hemophilia. Guidelines for the Management of Hemophilia, 3rd edition. Haemophilia. 2020;26(Suppl 6):1–158.
- Centers for Disease Control and Prevention. Hemophilia. https://www.cdc.gov/ncbddd/hemophilia/inhibitors.html. September 6, 2018. Accessed April 22, 2021.