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

Acquired hemophilia A is a severe bleeding disorder caused by an autoantibody to factor VIII. Previous reports have focused on referral center patients and it is unclear whether these findings are generally applicable. To improve understanding of the disease, a 2-year observational study was established to identify and characterize the presenting features and outcome of all patients with acquired hemophilia A in the United Kingdom. This allowed a consecutive cohort of patients, unbiased by referral or reporting practice, to be studied. A total of 172 patients with a median age of 78 years were identified, an incidence of 1.48/million/y. The cohort was significantly older than previously reported series, but bleeding manifestations and underlying diseases were similar. Bleeding was the cause of death in 9% of the cohort and remained a risk until the inhibitor had been eradicated. There was no difference in inhibitor eradication or mortality between patients treated with steroids alone and a combination of steroids and cytotoxic agents. Relapse of the inhibitor was observed in 20% of the patients who had attained first complete remission. The data provide the most complete description of acquired hemophilia A available and are applicable to patients presenting to all centers.

Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against coagulation factor VIII and characterized by spontaneous hemorrhage in patients with no previous family or personal history of bleeding. Although data on several AHA cohorts have been collected, limited information is available on the optimal management of AHA.

Acquired hemophilia A (AHA) is a rare disease resulting from autoantibodies (inhibitors) against endogenous factor VIII (FVIII) that leads to bleeding, which is often spontaneous and severe. AHA tends to occur in elderly patients with comorbidities and is associated with high mortality risk from underlying comorbidities, bleeding, or treatment complications. Treatment, which consists of hemostatic management and eradication of the inhibitors, can be challenging to manage. Few data are available to guide the management of AHA-related bleeding and eradication of the disease-causing antibodies. An extensive literature review was conducted with the aim to build on and complement already existing guidelines since the emergence of a newly approved hemostatic agent for this condition. An international panel of 8 experts in AHA was convened in 2015. A comprehensive literature search of PubMed and Embase was conducted; duplicate records and single-patient case studies were removed; and outputs were evaluated by at least 2 reviewers. Key questions were identified and analyzed; evidence was weighted; and consensus was formed. The resulting guidance for the management of AHA, presented here, was endorsed by the Hemostasis and Thrombosis Research Society of North America. AHA is rarely encountered by most physicians, and is likely to be underdiagnosed and misdiagnosed in real-world clinical practice. Data for AHA are limited and mainly restricted to registries, case reports, and clinical expertise. Recommendations for the management of AHA are summarized here based on the available data, integrated with the clinical experience of panel participants. This article is protected by copyright. All rights reserved.