Successful Long Term Eradication of Factor Viii Inhibitor in Patients With Acquired Haemophilia a in Saudi Arabia

Abstract

Acquired haemophilia A is a serious and potentially fatal bleeding disorder. Diagnosis is difficult and maybe delayed due to its rarity. The high mortality rate and the complex nature of treatment necessitate patient management at a haemophilia centre, where the required expertise and resources are available. Prompt diagnosis is crucial and early initiation of therapy could be life saving. Management includes initial control of bleeding followed by an approach to eradicate the coagulation factor inhibitor. In this paper we describe our local experience with acquired haemophilia A, which resulted in the successful control of major bleeding at presentation and eradication of inhibitors.

Cyclophosphamide Treatment for Acquired Factor VIII Inhibitor in a Patient with AIDS-Associated Progressive Multifocal Leukoencephalopathy

“…Those with substantial bleeding and high inhibitor levels require more aggressive interventions such as administration of activated prothrombin complex concentrate Factor 8 inhibitor bypassing activity (FEIBA). 4 For those patients who fail to respond and for whom bleeding remains problematic, human recombinant factor VIIa (rfVIIa) may be efficacious as a salvage therapy in as many as 80% of patients. 5,24,25 While there are no randomized trials comparing FEIBA and rVIIa effectiveness, results from an uncontrolled European registry of 501 patients with acquired FVIII deficiency suggest that the 2 agents have similar efficacy.…”

“…1,2 Patients typically present with hemorrhages in the skin, subcutaneous tissues, and muscles as well as mucosal bleeds. 3,4 The hemorrhages are frequently severe and can be accompanied by life-threatening retroperitoneal hematomas and compartment syndromes. Joint bleeds, a characteristic feature of classical congenital hemophilia, are rare.…”