Consensus recommendations for the diagnosis and treatment of acquired hemophilia A

Peter Collins, Francesco Baudo, Angela Huth-Kühne, Jørgen Ingerslev, Craig M Kessler, Maria E Mingot Castellano, Midori Shima, Jean St-Louis, Hervé Lévesque, Peter Collins, Francesco Baudo, Angela Huth-Kühne, Jørgen Ingerslev, Craig M Kessler, Maria E Mingot Castellano, Midori Shima, Jean St-Louis, Hervé Lévesque

Abstract

Background: Acquired hemophilia A (AHA) is a rare bleeding disorder caused by an autoantibody to coagulation factor (F) VIII. It is characterized by soft tissue bleeding in patients without a personal or family history of bleeding. Bleeding is variable, ranging from acute, life-threatening hemorrhage, with 9-22% mortality, to mild bleeding that requires no treatment. AHA usually presents to clinicians without prior experience of the disease, therefore diagnosis is frequently delayed and bleeds under treated.

Methods: Structured literature searches were used to support expert opinion in the development of recommendations for the management of patients with AHA.

Results: Immediate consultation with a hemophilia center experienced in the management of inhibitors is essential to ensure accurate diagnosis and appropriate treatment. The laboratory finding of prolonged activated partial thromboplastin time with normal prothrombin time is typical of AHA, and the diagnosis should be considered even in the absence of bleeding. The FVIII level and autoantibody titer are not reliable predictors of bleeding risk or response to treatment. Most patients with AHA are elderly; comorbidities and underlying conditions found in 50% of patients often influence the clinical picture. Initial treatment involves the control of acute bleeding with bypassing agents. Immunosuppressive treatment to eradicate the FVIII inhibitor should be started as soon as the diagnosis is confirmed to reduce the time the patient is at risk of bleeding.

Conclusions: These recommendations aim to increase awareness of this disorder among clinicians in a wide range of specialties and provide practical advice on diagnosis and treatment.

Figures

Figure 1
Figure 1
Extensive subcutaneous ecchymoses of the limbs, thorax and abdomen.
Figure 2
Figure 2
Algorithm to guide the management of patients with suspected acquired hemophilia. AH, acquired hemophilia; LA, lupus anticoagulant; F, coagulation factor; aPTT, activated partial thromboplastin time.

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