AI Article Synopsis
- Acquired haemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against factor VIII, with a population-based incidence of 1.78 cases per million per year found in a study in Manitoba, Canada.
- The study identified 34 patients over 15 years, most commonly presenting at an average age of 76, with 97% experiencing bleeding, often major, leading to delayed diagnoses and a need for haemostatic therapy.
- Treatment involved immunosuppressive therapy in 88% of cases, yielding an encouraging 79% remission rate, with relatively low treatment-related mortality and morbidity noted.
Article Abstract
Introduction: Acquired haemophilia A (AHA) is a rare bleeding disorder characterized by autoantibodies against coagulation factor VIII (FVIII). Estimates of AHA incidence are largely based on registry data, which may be prone to referral bias. Population-based studies can enhance our understanding of the epidemiology, presentation and outcomes of AHA.
Methods: We conducted a retrospective, population-based cohort study of all AHA diagnosed and treated in Manitoba, Canada over a 15-year period. Using records from the sole provincial reference laboratory, we identified all patients with FVIII inhibitors who did not have congenital haemophilia. Using a piloted case report form, patient data was ascertained from hospital and bleeding disorder clinic records.
Results: From 2006 to 2021, we identified 34 patients with AHA, corresponding to a population-based incidence rate of AHA of 1.78 cases per million per year. The median age at presentation was 76 years and most cases were idiopathic (79%). Almost all patients (97%) presented with bleeding, of which 58% were considered major bleeds and required haemostatic agents in 67%. Longstanding unexplained bleeding symptoms were commonly reported, suggesting delayed diagnosis. Immunosuppressive therapy (IST) was administered in 88% of patients. Remission was achieved in 79% of patients; median time to remission was 2.1 months. There were two deaths due to bleeding. No deaths due to IST were reported.
Conclusion: The population-based incidence of AHA in Manitoba is 1.78 cases/million/year. Bleeding is common and can be life-threatening. AHA outcomes are encouraging with the use of haemostatic agents and IST. Serious treatment-associated morbidity and mortality is uncommon.
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| http://dx.doi.org/10.1111/hae.14845 | DOI Listing |
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Hemophilia A is a clotting disorder due to factor VIII deficiency, leading to prolonged bleeding. Acquired hemophilia A results from the immune system attacking factor VIII, typically occurring later in life. Factor V Leiden is a genetic mutation causing abnormal blood clot formation, primarily in veins.
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