Prognostic factors for recurrence in acquired hemophilia A-results from a long-term observational study
Objectives: Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against factor (F)VIII (FVIII), potentially leading to life-threatening bleeding. While predictors for remission have been analyzed, data on recurrence is lacking. Methods: This study investigated predictor...
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Elsevier
2025-02-01
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| Series: | Research and Practice in Thrombosis and Haemostasis |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2475037925000317 |
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| author | Lisa Reich Florian Gatzke Steffen Rauchfuss Stefanie Roth Wolfgang Miesbach |
| author_facet | Lisa Reich Florian Gatzke Steffen Rauchfuss Stefanie Roth Wolfgang Miesbach |
| author_sort | Lisa Reich |
| collection | DOAJ |
| description | Objectives: Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against factor (F)VIII (FVIII), potentially leading to life-threatening bleeding. While predictors for remission have been analyzed, data on recurrence is lacking. Methods: This study investigated predictors of AHA recurrence in 41 patients. Patients were divided into 2 groups: those with recurrence (n = 18) and those in stable long-term remission (n = 23) with at least 1 year of follow-up. Results: All relapses occurred within 1 year of initial remission. The median follow-up period was 3.8 years (IQR, 1.8-6.4) for all included patients. Multivariate Cox regression analysis revealed that initial FVIII activity <1 IU/dL and failure to achieve initial complete remission (CR) were significant predictors of relapse. Kaplan–Meier curves showed significantly different relapse-free survival rates for patients with initial FVIII activity <1 IU/dL vs ≥1 IU/dL (χ2[1] = 5.950, P = .015), and for those achieving initial CR vs partial remission (χ2[1] = 6.570, P = .010).Other factors such as inhibitor titer, gender, age, World Health Organization scale, underlying disorder, controlled disorder, initial immunosuppressive therapy, immunosuppressive therapy escalation, and partial remission at day 21 showed no significant relation to recurrences. Overall survival did not differ significantly between relapsing and nonrelapsing patients (χ2[1] = .896, P = .344). Conclusion: Initial FVIII <1 IU/dL and failure to achieve initial CR are identified as risk factors for recurrence in AHA. Patients with these characteristics should be closely monitored for at least 1 year after initial remission due to increased recurrence risk. |
| format | Article |
| id | doaj-art-a80e3b84b73c4b81a886493fb72f6ba8 |
| institution | OA Journals |
| issn | 2475-0379 |
| language | English |
| publishDate | 2025-02-01 |
| publisher | Elsevier |
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| series | Research and Practice in Thrombosis and Haemostasis |
| spelling | doaj-art-a80e3b84b73c4b81a886493fb72f6ba82025-08-20T02:18:23ZengElsevierResearch and Practice in Thrombosis and Haemostasis2475-03792025-02-019210270710.1016/j.rpth.2025.102707Prognostic factors for recurrence in acquired hemophilia A-results from a long-term observational studyLisa Reich0Florian Gatzke1Steffen Rauchfuss2Stefanie Roth3Wolfgang Miesbach4Medical Clinic 2, University Hospital Frankfurt, GermanyMedical Clinic 2, University Hospital Frankfurt, GermanyMedical Clinic 2, University Hospital Frankfurt, GermanyMedical Clinic 2, University Hospital Frankfurt, GermanyCorrespondence Wolfgang Miesbach, Medical Clinic 2, University Hospital Frankfurt, Theodor-Stern-Kai 7, 60590 Frankfurt, Germany.; Medical Clinic 2, University Hospital Frankfurt, GermanyObjectives: Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against factor (F)VIII (FVIII), potentially leading to life-threatening bleeding. While predictors for remission have been analyzed, data on recurrence is lacking. Methods: This study investigated predictors of AHA recurrence in 41 patients. Patients were divided into 2 groups: those with recurrence (n = 18) and those in stable long-term remission (n = 23) with at least 1 year of follow-up. Results: All relapses occurred within 1 year of initial remission. The median follow-up period was 3.8 years (IQR, 1.8-6.4) for all included patients. Multivariate Cox regression analysis revealed that initial FVIII activity <1 IU/dL and failure to achieve initial complete remission (CR) were significant predictors of relapse. Kaplan–Meier curves showed significantly different relapse-free survival rates for patients with initial FVIII activity <1 IU/dL vs ≥1 IU/dL (χ2[1] = 5.950, P = .015), and for those achieving initial CR vs partial remission (χ2[1] = 6.570, P = .010).Other factors such as inhibitor titer, gender, age, World Health Organization scale, underlying disorder, controlled disorder, initial immunosuppressive therapy, immunosuppressive therapy escalation, and partial remission at day 21 showed no significant relation to recurrences. Overall survival did not differ significantly between relapsing and nonrelapsing patients (χ2[1] = .896, P = .344). Conclusion: Initial FVIII <1 IU/dL and failure to achieve initial CR are identified as risk factors for recurrence in AHA. Patients with these characteristics should be closely monitored for at least 1 year after initial remission due to increased recurrence risk.http://www.sciencedirect.com/science/article/pii/S2475037925000317acquired hemophiliableedingfactor VIIIrecurrenceremission |
| spellingShingle | Lisa Reich Florian Gatzke Steffen Rauchfuss Stefanie Roth Wolfgang Miesbach Prognostic factors for recurrence in acquired hemophilia A-results from a long-term observational study Research and Practice in Thrombosis and Haemostasis acquired hemophilia bleeding factor VIII recurrence remission |
| title | Prognostic factors for recurrence in acquired hemophilia A-results from a long-term observational study |
| title_full | Prognostic factors for recurrence in acquired hemophilia A-results from a long-term observational study |
| title_fullStr | Prognostic factors for recurrence in acquired hemophilia A-results from a long-term observational study |
| title_full_unstemmed | Prognostic factors for recurrence in acquired hemophilia A-results from a long-term observational study |
| title_short | Prognostic factors for recurrence in acquired hemophilia A-results from a long-term observational study |
| title_sort | prognostic factors for recurrence in acquired hemophilia a results from a long term observational study |
| topic | acquired hemophilia bleeding factor VIII recurrence remission |
| url | http://www.sciencedirect.com/science/article/pii/S2475037925000317 |
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