Successful eradication of acquired factor VIII inhibitors with rituximab: a report of two cases.

OBJECTIVES

Acquired hemophilia A (AHA) is a rare autoimmune disorder that presents with spontaneous bleeding due to the development of autoantibodies against coagulation factor VIII. This study aims to highlight the challenges in diagnosing and treating AHA, particularly through presenting two cases managed with rituximab, an anti-CD20 antibody, to demonstrate its safety and efficacy as a treatment option.

METHODS

Two male patients, aged 38 and 68, with significant bleeding episodes and prolonged activated partial thromboplastin time (aPTT), were evaluated. Both patients received standard care, including factor VIII replacement and corticosteroids. Due to persistent symptoms, rituximab was administered weekly for four weeks, and follow-up assessments were performed to monitor factor VIII levels, aPTT, and clinical symptoms.

RESULTS

Both cases showed improvement with rituximab. In Case 1, a 38-year-old male with idiopathic AHA achieved normal factor VIII levels and aPTT, with complete resolution of symptoms and no recurrence. In Case 2, a 68-year-old male with congenital hemophilia A and superimposed AHA responded positively to rituximab, showing stabilized factor VIII levels and no further bleeding episodes at a six-month follow-up.

DISCUSSION: The management of AHA is complex due to its rarity and severe bleeding risks. Although corticosteroids and bypassing agents are primary treatments, rituximab is emerging as a promising therapeutic option. Current literature supports rituximab for patients with contraindications to first-line agents or poor prognosis, yet further studies are required to assess its potential as a first-line treatment.

CONCLUSION

These cases emphasize the efficacy and safety of rituximab in managing AHA. Given its potential benefits, further randomized controlled trials are warranted to evaluate rituximab's role as a first-line treatment. A structured monitoring protocol is recommended to ensure safe administration and manage potential side effects associated with immunosuppressive therapy.