From the Guidelines
The management of acute bleeding in Hemophilia A requires immediate initiation of anti-hemorrhagic treatment, with recombinant factor VIIa (rFVIIa) or activated prothrombin complex concentrates (aPCCs) being the recommended treatment options for severe bleeding symptoms, irrespective of inhibitor titer and residual FVIII activity. For the treatment of severe bleeding in patients with Hemophilia A, the following options are recommended:
- Recombinant factor VIIa (rFVIIa) at a dose of 90 μg/kg every 2-3 hours until hemostasis is achieved 1
- Activated prothrombin complex concentrates (aPCCs) at a dose of 50-100 IU/kg every 8-12 hours to a maximum of 200 IU/kg/day 1 The decision to initiate treatment should be based on the severity of bleeding symptoms, rather than the inhibitor titer or residual FVIII activity, as there is a lack of correlation between these factors and bleeding phenotype in Hemophilia A 1. In addition to these treatment options, adjunctive measures such as tranexamic acid, local pressure, ice application, and immobilization of affected limbs may be used to support hemostasis. It is essential to weigh the risks, benefits, and costs of treatment carefully and on an individual basis, particularly in elderly patients with comorbidities, and to monitor patients frequently to assess the effectiveness of treatment and adjust as needed 1.
From the FDA Drug Label
Desmopressin acetate injection will also stop bleeding in hemophilia A patients with episodes of spontaneous or trauma-induced injuries such as hemarthroses, intramuscular hematomas or mucosal bleeding The management of acute bleeding in Hemophilia A includes the use of desmopressin acetate injection to stop bleeding in patients with factor VIII coagulant activity levels greater than 5%.
- Key considerations:
- Desmopressin acetate injection is not indicated for patients with factor VIII coagulant activity levels equal to or less than 5%
- Patients with factor VIII levels between 2% to 5% should be carefully monitored if desmopressin acetate injection is used 2 The use of desmopressin acetate injection should be guided by the patient's factor VIII coagulant activity levels.
From the Research
Management of Acute Bleeding in Hemophilia A
The management of acute bleeding in Hemophilia A involves various treatment options, including:
- Replacement therapy with blood products, such as plasma-derived cryoprecipitate and factor VIII concentrates 3
- Desmopressin, a synthetic vasopressin analogue that increases plasma concentrations of coagulation factor VIII and von Willebrand factor, which is an attractive therapeutic alternative due to its lack of risk of transmitting infectious diseases 3
- Recombinant factor VIII replacement therapy, which has significantly improved the life expectancy and quality of life of individuals with severe Hemophilia A, but requires tremendous resources and is expensive 4
- Tranexamic acid, which may be used as an adjunct to factor VIII concentrate or bypassing agents to enhance clot stability and allow for effective treatment at lower doses of factor concentrate 5
Treatment Options
The following treatment options are available for managing acute bleeding in Hemophilia A:
- Desmopressin: optimal hemostatic effect is achieved with a dosage of 0.3 microg/kg given intravenously, and an intranasal desmopressin spray is suitable for home treatment 3
- Recombinant factor VIII: current optimal treatment should aim to provide sufficient factor VIII to prevent spontaneous bleeding and to have normal coagulation function after any trauma 4
- Factor VIII replacement therapies: the dose and frequency required to reduce bleeds varies amongst individuals and may change over time, making personalized therapy essential 6
- Tranexamic acid: may be used in combination with factor concentrates to treat individuals with Hemophilia A, resulting in normalized clot stability even at low doses of factor concentrate 5
Challenges and Future Directions
The management of acute bleeding in Hemophilia A faces several challenges, including:
- Development of neutralizing antibodies to factor VIII, which can render replacement therapy ineffective 4
- High cost and limited availability of factor VIII concentrates 3, 4
- Need for personalized therapy to optimize treatment outcomes 6
- Limited evidence on the optimal hemostatic therapies for acquired Hemophilia A, highlighting the need for further research 7