First-line immunosuppressive therapies for acquired hemophilia A: A 25-year cohort experience and network meta-analysis
Issued Date
2024-09-01
Resource Type
ISSN
00493848
eISSN
18792472
Scopus ID
2-s2.0-85197555105
Journal Title
Thrombosis Research
Volume
241
Rights Holder(s)
SCOPUS
Bibliographic Citation
Thrombosis Research Vol.241 (2024)
Suggested Citation
Rungjirajittranon T., Suwanawiboon B., Nakkinkun Y., Leelakanok N., Kaokunakorn T., Chinthammitr Y., Owattanapanich W., Ruchutrakool T. First-line immunosuppressive therapies for acquired hemophilia A: A 25-year cohort experience and network meta-analysis. Thrombosis Research Vol.241 (2024). doi:10.1016/j.thromres.2024.109067 Retrieved from: https://repository.li.mahidol.ac.th/handle/123456789/99650
Title
First-line immunosuppressive therapies for acquired hemophilia A: A 25-year cohort experience and network meta-analysis
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Corresponding Author(s)
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Abstract
Acquired hemophilia A (AHA) presents a significant bleeding risk. Management involves bleeding control and immunosuppressive therapy (IST) to eliminate inhibitors. This study, encompassing a retrospective cohort of 76 newly diagnosed AHA patients (1997–2022), evaluated IST outcomes such as complete remission (CR), relapse, and mortality rates, alongside influencing factors. Supplementing these findings, a systematic review and network meta-analysis compared CR and relapse rates across ISTs, sourcing from Embase, Scopus, and ScienceDirect up to November 2023. In our cohort, demarcated by a 20 Bethesda-unit titer threshold, cyclophosphamide plus prednisolone (CP; n = 64) was the predominant initial IST. Lower inhibitor levels significantly correlated with higher CR rates (86.8 % vs 62.2 %; P = .014) and showed an odds ratio of 0.26 for CR (P = .021). Median relapse-free survival (RFS) extended to 37.13 months, significantly enhanced by CP (hazard ratio, 0.24; 95 % confidence interval, 0.10–0.60; P = .002). Our network meta-analysis, including 1476 CR and 636 relapse patients, indicated CP and rituximab-based ISTs significantly outperformed steroid monotherapy in terms of CR and lower relapse rates (risk differences of 0.15 and −0.13/−0.15, respectively; P < .05), without significant differences between CP and rituximab. Moreover, adding rituximab to the front-line treatment did not produce superior outcomes compared to the CP regimen alone, positioning CP as a viable first-line choice, particularly where rituximab is less accessible. The consideration of IST toxicity remains critical in treatment decisions.