Case Report: Effective management of adalimumab-induced acquired hemophilia A with the CyDRI protocol

Abstract

Introduction: Acquired Hemophilia A (AHA) is a rare autoimmune disorder characterized by the emergence of inhibitors that specifically target coagulation Factor VIII, frequently resulting in severe bleeding episodes.

Methods: We conducted a retrospective analysis of the medical records of a 68-year-old male patient who presented with adalimumab-induced AHA.

Results: The patient received adalimumab, a tumor necrosis factor inhibitor antibody, as part of his treatment for rheumatoid arthritis. The patient's clinical journey, characterized by intense bleeding and coagulopathy, was effectively managed with the application of recombinant Factor VIIa (rFVIIa) and the CyDRi protocol.

Discussion: The case emphasizes the importance of prompt coagulation assessment in patients with bleeding symptoms receiving disease-modifying therapy for rheumatoid arthritis that includes adalimumab therapy, considering the rare yet life-threatening nature of AHA. Additionally, this report provides an extensive review of the existing literature on drug-induced AHA, with a special emphasis on cases linked to immunomodulatory medications. Through this two-pronged approach, our report aims to enhance understanding and awareness of this severe complication among healthcare providers, promoting timely diagnosis and intervention.

Keywords: acquired hemophilia A; adalimumab; bleeding disorder; immunosuppression; rheumatoid arthritis.

FIGURE 1

Time course of Factor VIII dynamics following CyDRi treatment in a male patient with adalimumab-induced AHA. This figure displays the temporal correlation between the administration of the CyDRi immunosuppressive regimen (comprising cyclophosphamide, dexamethasone, and rituximab) and subsequent alterations in Factor VIII (FVIII) inhibitor levels (represented by the Bethesda titer in red) as well as FVIII activity (indicated in green). These changes are mapped over time to demonstrate the patient’s hematological response to the treatment. Key treatment intervals are marked by bars: the period of adalimumab administration is highlighted in red, while the initiation of the CyDRi regimen, after the discontinuation of adalimumab, is denoted in green. This visual representation aids in understanding the efficacy of the CyDRi regimen in altering FVIII parameters post-adalimumab therapy.