Treatments for acute bleeds in people with acquired hemophilia A

Acquired hemophilia A is a rare but severe bleeding disorder caused by the autoantibody directed against factor VIII (FVIII, a blood clotting protein) in patients with no previous history of a bleeding disorder. This bleeding disorder occurs more frequently in the elderly and may be associated with several other conditions, such as solid tumours, autoimmune diseases, or with drugs; it is also sometimes associated with pregnancy. However, in about half of the cases the causes are unknown. Bleeding occurs in the skin; mucous membranes; and muscles; with joint bleeds being unusual. Therapies for acquired hemophilia A include treatments to stop acute bleeds and to destroy the FVIII autoantibodies. Acute bleeds can be treated with recombinant activated FVII or activated prothrombin complex concentrate (aPCC). If these are unavailable, FVIII concentrates or 1-desamino-8-D-arginine-vasopressin (DDAVP) fresh frozen plasma can be attempted; however, these last two options are not usually effective, and it is not known if one of these products is better than the other.

We conducted a review of any treatment used in people with acquired hemophilia A during episodes of acute bleeding. After searching for all relevant trials, no randomised controlled trials could be identified, and no definitive conclusion on the best available treatment could be drawn.

Prospective randomised controlled trials are needed to evaluate the precise role of different drugs for acute bleeds in acquired hemophilia A, but the rarity of the condition is hindering their planning and execution. While waiting for better evidence, patients and doctors need to base treatment decisions on the largest and better conducted observational studies, which are referenced in the background of our review.

Authors' conclusions: 

No randomised clinical trials of hemostatic therapies for acquired hemophilia A were found. Thus, we are not able to draw any conclusions or make any recommendations on the optimal hemostatic therapies for acquired hemophilia A based on the highest quality of evidence. GIven that carrying out randomized controlled trials in this field is a complex task, the authors suggest that, while planning randomised controlled trials in which patients can be enrolled, clinicians treating the disease continue to base their choices on alternative, lower quality sources of evidence, which hopefully, in the future, will also be appraised and incorporated in a Cochrane Review.

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Background: 

Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation factor VIII (FVIII). In most cases, bleeding episodes are spontaneous and severe at presentation. The optimal hemostatic therapy is controversial.

Objectives: 

To determine the efficacy of hemostatic therapies for acute bleeds in people with acquired hemophilia A; and to compare different forms of therapy for these bleeds.

Search strategy: 

We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library 2014, Issue 4) and MEDLINE (Ovid) (1948 to 30 April 2014). We searched the conference proceedings of the: American Society of Hematology; European Hematology Association; International Society on Thrombosis and Haemostasis (ISTH); and the European Association for Haemophilia and Allied Disorders (EAHAD) (from 2000 to 30 April 2014). In addition to this we searched clinical trials registers.

Selection criteria: 

All randomised controlled trials and quasi-randomised trials of hemostatic therapies for people with acquired hemophilia A, with no restrictions on gender, age or ethnicity.

Data collection and analysis: 

No trials matching the selection criteria were eligible for inclusion.

Main results: 

No trials matching the selection criteria were eligible for inclusion.

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