Abstract
To conduct a systematic review of the literature reporting efficacy and safety of recombinant factor VIIa (rFVIIa) for the treatment of bleeding in acquired haemophilia and, if data permitted, undertake a meta-analysis of the current evidence. MEDLINE®, Embase®, and the Cochrane Central Register of Controlled Trials (CENTRAL) databases were searched for all studies on rFVIIa treatment in acquired haemophilia. Heterogeneity of included studies was measured using the inconsistency index (I2). Of the 2353 publications screened, 290 potentially relevant references were identified: 12 studies published in 32 publications met inclusion criteria. In total, 1244 patients and 1714 bleeds were included (671 patients received rFVIIa treatment for 1063 bleeds). In seven of 12 studies, the initial dose of Recombinant FVIIa was 90 ± 10 μg/kg. Recombinant FVIIa was used as first-line therapy in the majority of cases. Median number of doses administered ranged from 10 to 28. Between 68 and 74% of bleeds were spontaneous, whereas 4–50% were traumatic. Thirty-nine to 90% of bleeds were severe. Haemostatic effectiveness was > 90% in 5/6 studies for both patient and bleed level. Recombinant FVIIa had a favourable safety profile with low risk of general adverse events and thromboembolic-associated events. The heterogeneity of the studies and data precluded a meta-analysis. Recombinant FVIIa demonstrated effectiveness for the treatment of bleeds and had a good safety profile. It is apparent from these data that there is a need for more standardised measures of clinical effectiveness in acquired haemophilia to enable comparison and pooling of results in the future.
Highlights
Acquired haemophilia A is a rare bleeding disorder that affects approximately 1.48 people per million per year [1]
The incidence of acquired haemophilia increases with age [1], and there is a small peak among women of childbearing age [2, 3]
Acquired haemophilia is caused by autoantibodies against coagulation factor VIII (FVIII) that partially or completely
Summary
Acquired haemophilia A is a rare bleeding disorder that affects approximately 1.48 people per million per year [1]. Acquired haemophilia is caused by autoantibodies against coagulation factor VIII (FVIII) that partially or completely. Bleeding presentation in acquired haemophilia can be mild but is more frequently severe (> 67% of cases) [3], and not all types of bleeding may require intervention (ecchymosis and subcutaneous lesions), the immediate treatment priority is generally to control acute bleeding with bypassing agents [6, 7]. Haemostatic treatment is recommended in patients with acquired haemophilia and active severe bleeding, irrespective of inhibitor titre and residual FVIII activity [8]. Recombinant factor VIIa (rFVIIa) was first approved in 1996 and is indicated for both the treatment of bleeds and prevention of bleeding in patients with acquired haemophilia undergoing surgery or invasive procedures [9]
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