Acquired hemophilia A: when an overlooked autoimmune disorder causes significant bleeding

ABSTRACT

Introduction: Acquired hemophilia A (AHA) is a rare disease caused by the presence of autoantibodies directed against factor VIII (FVIII) resulting in spontaneous bleeding. AHA is associated with high risk of mortality from bleeding, treatment complications, and the presence of underlying diseases. Because of its rarity, AHA diagnosis can be challenging and sometimes missed in the emergency setting. Management consists of bleeding control and prevention, inhibitor eradication using immunosuppressive therapy, as well as treatment of the underlying disease.

Areas covered: An overview of AHA including epidemiology, pathophysiology, clinical presentation, workup, and management; as well as a review of the literature in order to better understand the disease.

Expert opinion: A prompt recognition of AHA is mandatory for the initiation of early and aggressive treatment. Optimal management of AHA necessitates a multidisciplinary approach in collaboration between hematologists and physicians from various specialties. Future studies are needed to identify prognostic factors for remission and survival in patients with AHA. Clinicians who treat patients with AHA are encouraged to actively contribute to the available registries. International associations and societies are also invited to assist in the establishment of hemophilia centers and networks worldwide for the standardization of clinical practice when dealing with patients with AHA.

KEYWORDS:

• Acquired hemophilia A
• epidemiology
• pathophysiology
• clinical presentation
• workup
• management
• hemorrhage
• inhibitor eradication
• immunosuppressive therapy

Article highlights

• Acquired hemophilia is an uncommon autoimmune disease that affects most frequently young adults, mainly females with autoimmune diseases in the postpartum period or the elderly population.

• Bleeding is the most common clinical presentation, including spontaneous bleeding, trauma-related bleeding, surgery-related bleeding, and others.

• Diagnosis is based on the presence of an isolated prolonged activated partial thromboplastin time (aPTT), failing to correct after mixing study, with a normal prothrombin (PT) and thrombin time (TT) and a negative lupus anticoagulant (LA).

• Management of AHA is a three-way directed approach: control of bleeding (through the use of bypassing agents mainly), elimination of the inhibitor (through immunosuppressive therapy) and treatment of the underlying disease.

• Frequent monitoring especially during first 6 weeks of disease control is essential with patient and clinician education being of utmost importance.

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Declaration of Interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer Disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.