Onkologie. 2024:18(5):330-333 | DOI: 10.36290/xon.2024.069

Emicizumab in treating acquired haemophilia A: our initial experience

Eva Drbohlavová

Oddělení klinické hematologie, Krajská nemocnice Liberec

Acquired haemophilia A (AHA) is a rare bleeding disorder occurring particularly in the elderly. It is caused by the presence of autoantibodies against coagulation factor VIII (FVIII). It may be associated with an autoimmune disease, malignancy, occur after birth, or arise idiopathi­cally. Early diagnosis and treatment are important, which involves treatment of bleeding and immunosuppressive therapy to induce disease remission or eradicate the inhibitor. We report a case of a female patient with rheumatoid arthritis with bleeding manifestations from the gastrointestinal tract (GIT), skin suffusions, and prolonged bleeding after tooth extraction. The patient was diagnosed with AHA and had to be started on treatment with recombinant activated factor VII (rFVIIa). Initially, a high inhibitor level was found that decreased very slowly after treatment with combined immunosuppression (corticoids, cyclophosphamide, rituximab). Due to newly detected bleeding into the psoas muscle, rFVIIa replacement therapy was increased and emicizumab was indicated to prevent further bleeding in the patient with persistent inhibitor. Thanks to the subcutaneous route of administration, the patient could be discharged to outpatient care.

Keywords: acquired haemophilia A, bypassing agents, immunosuppression, emicizumab.

Accepted: November 18, 2024; Published: November 28, 2024  Show citation