Abstract Number: PB0434
Meeting: ISTH 2021 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Acquired Hemorrhagic Coagulation Disorders
Background: Acquired hemophilia A (AHA) is a disease characterized by the presence of autoantibody against the factor VIII. Bleeding control and inhibitor eradication are major goals for the treatment of acquired hemophilia A. There is limited data on the rituximab used as an inhibitor eradication.
Aims: To determine the efficiency and safety of rituximab for treating acquired hemophilia A.
Methods: We conducted a systematic review and individual patient-data analysis by searching PubMed, EMBASE, and Scopus databases from Inception to February 2021. Studies were included if they were case reports, case series, cross-sectional studies, case-control studies, or cohort studies concerning the utilization of rituximab on the treatment of acquired hemophilia A. We excluded studies that lacked individual patient data. Primary outcomes were a response rate and all-cause mortality.
Results: A total of 105 studies involving 186 patients with acquired hemophilia A were included in the analysis. Males were 56.6%. The median age was 71 years (interquartile range; IQR, 56-78). Median factor VIII clotting activity (FVIII: C) was 0.02% (IQR 0-2), and the median FVIII inhibitor was 31 Bethesda unit/mL (IQR 10-128). Rituximab was used as a single agent in 23 patients (12.4%). The rest of the patients received rituximab in combination with either corticosteroid, cytotoxic agents, mycophenolate mofetil, or others. Overall response rate was 85.0% (complete remission 72.6%, partial remission 12.4%). Rituximab treated as first-line therapy was associated with a higher rate of complete remission as comparing to second-line therapy, 82.1% and 64.7%, respectively (Table 1). The median time to respond was 76 days (IQR 30-149). Twenty-seven patients (14.4%) died. Of these, 10 patients died from infectious complications.
| Line of rituximab therapy | Response | |
|---|---|---|
| Complete remission | Partial remission | |
| First-line therapy | 69 (82.14%) | 5 (5.95%) |
| Second-line therapy | 66 (64.71%) | 18 (17.65%) |
Response comparing line of rituximab therapy
Conclusions: This study showed that rituximab monotherapy or in combinations were effective for the inhibitor eradication therapy in patients with acquired hemophilia A. Further prospective studies investigating the efficacy of rituximab in acquired hemophilia are urgently needed.
To cite this abstract in AMA style:
Moonla S, Suriyasathaporn A, Kiatsoonthon K, Chai-Adisaksopha C. Efficacy of Rituximab for the Treatment of Acquired Hemophilia A: A Systematic Review [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/efficacy-of-rituximab-for-the-treatment-of-acquired-hemophilia-a-a-systematic-review/. Accessed November 29, 2023.« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/efficacy-of-rituximab-for-the-treatment-of-acquired-hemophilia-a-a-systematic-review/