Inhibitor eradication and treatment for acquired hemophilia A

ABSTRACT

Introduction

Acquired hemophilia A (AHA) is a rare hemorrhagic autoimmune disorder characterized by autoantibodies against coagulation factor VIII (FVIII). In approximately half of the cases AHA does not recognize any cause (idiopathic form), while in the other cases it may be triggered by autoimmune disorders, cancers, drugs, infections, or pregnancy. Besides treating the underlying disorder, specific AHA treatment includes management of bleeding, if necessary, and inhibitor eradication.

Areas covered

This narrative review summarizes the main epidemiological, clinical, laboratory, and therapeutic characteristics of AHA. In particular, it is focused on the current therapeutic options for the inhibitor eradication, also showing the latest findings on the innovative therapies. A literature search strategy was performed, without temporal limits, through Medline and PubMed electronic databases.

Expert opinion

Various first-line and second-line immunosuppressive agents are currently available for the management of AHA. Among the latter, the anti-CD20 monoclonal antibody rituximab has been the object of intense research during the last years from investigators as innovative promising eradicating therapy for AHA. Preliminary data from the studies support the use of this drug as a first-line option for newly diagnosed AHA cases.

KEYWORDS:

• Acquired hemophilia A
• inhibitors
• FVIII
• emicizumab
• bleeding

Article highlights

• Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against coagulation factor VIII.

• Not rarely AHA is characterized by a sudden serious hemorrhage, which represents an emergency medical condition.

• The mainstay of treatment of AHA is based on anti-hemorrhagic therapy with bypassing agents and inhibitor eradication using a combination of steroids and cytotoxic agents.

• The use of the monoclonal antibody emicizumab as hemostatic therapy in AHA is promising, although more safety and efficacy data are needed.

• Among immunosuppressive therapies aimed at long-term eradication of anti-FVIII autoantibody, rituximab is the most promising.

Abbreviations

AHA	=	acquired hemophilia A.
IST	=	immunosuppressive therapy.
FVIII	=	factor VIII.
BU	=	Bethesda unit.

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.

Author contributions

Conceptualization was performed by M Franchini, the methodology by D Focosi, the writing of the original draft by M Franchini, and the writing, reviewing, and editing by D Focosi. All authors have read and agreed to the final published version of the manuscript.

Additional information

Funding

This paper was not funded.