Seminars in thrombosis and hemostasis
-
Semin. Thromb. Hemost. · Nov 2009
ReviewTwelve years of experience of acquired hemophilia A: trials and tribulations in South Australia.
Acquired hemophilia A (AH) is a rare and serious acquired bleeding disorder where prompt and correct diagnosis is crucial, and immune suppression is often required for factor VIII (FVIII) autoantibody eradication. The acquired FVIII deficiency usually manifests as bruises and bleeding, and treatment such as FVIII has limited efficacy because of the neutralizing FVIII inhibitor. Expensive bypassing agents such as recombinant activated factor VII (rFVIIa) may be required to treat clinically significant bleeding. ⋯ Overall mortality was 25% ( N = 6), five of whom were not treated. Advanced age and lack of treatment were predictive of poor survival outcomes. The very elderly (>75 years of age) may warrant a different treatment modality such as rituximab, which is potentially more tolerable and efficacious.
-
Semin. Thromb. Hemost. · Nov 2009
ReviewRecent improvements in the clinical treatment of coagulation factor inhibitors.
The mainstay of therapy in patients with congenital hemophilia is factor replacement. However, the development of inhibitors in these patients is a major complication that represents an important challenge in hemophilia care. ⋯ The management of severe bleeding episodes and the definitive eradication of the autoantibody are also the two main options of the clinical management of patients with acquired hemophilia, a rare but life-threatening hemorrhagic condition. The most recent options available for treating patients with acquired hemophilia and congenital hemophilia with inhibitors are addressed in this review.