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[Emergency treatment of haemophilia A with factor VIII inhibitors using activated prothrombin complex concentrates
Insights
Activated prothrombin complex concentrates effectively treated severe rectal bleeding in a boy with hemophilia A and factor VIII inhibitors. This treatment resolved bleeding despite initial resistance to factor VIII concentrates.
Area of Science:
- Hematology
- Pediatric Hemorrhage Management
Background:
- Severe rectal bleeding in pediatric patients with hemophilia A and factor VIII inhibitors presents a significant treatment challenge.
- Standard factor VIII concentrates may be ineffective in patients with high-titer inhibitors.
Observation:
- A 6-year-old boy with hemophilia A and factor VIII inhibitors experienced severe rectal bleeding unresponsive to factor VIII concentrates.
- Transient amaurosis (vision loss) occurred after the initial dose of activated prothrombin complex concentrates.
Findings:
- Activated prothrombin complex concentrates (fraction FEIBA) successfully controlled the severe rectal bleeding over an eight-day period.
- The amaurosis resolved spontaneously within minutes.
- Laboratory tests indicated accelerated intravascular coagulation, with elevated fibrin monomers and fibrin/fibrinogen degradation products.
Implications:
- Activated prothrombin complex concentrates are a viable therapeutic option for managing severe bleeding in hemophilia A patients with factor VIII inhibitors.
- Monitoring for coagulopathy is crucial during treatment with prothrombin complex concentrates.
- The transient visual disturbance warrants further investigation but did not preclude successful treatment.
Abstract:
Severe rectal bleeding in a 6-year-old boy with haemophilia A and factor VIII inhibitors could not be stopped with factor VIII concentrates. But a good effect was achieved with activated prothrombin complex concentrates (fraction FEIBA), given over eight days. Amaurosis occurred as a complication after injection of the first dose, but disappeared completely within several minutes. Tests revealed accelerated intravascular coagulation with increased fibrin monomers and fibrin/fibrinogen degradation products.