Acquired haemophilia A is a rare disease with an annual incidence of 1.48 per million. Based on clinical observations, in this article a higher incidence in southern Switzerland is suspected, and its aim is to provide local epidemiological data, and clinical information regarding diagnosis, treatment and outcome in this region. All adult patients with acquired haemophilia A treated between 2013 and 2019 in a medical establishment in southern Switzerland are included in the present retrospective analysis.
11 patients with acquired haemophilia A between 2013 and 2019 are treated, resulting in an annual incidence of 4.5 per million. Median delay from first symptoms to diagnosis was 4.5 days, and the median age at diagnosis is 79 years. Possible causative conditions were: pregnancy, polyarteritis nodosa, myelodysplastic syndrome, chronic human immunodeficiency virus (HIV), and HIV postexposure prophylaxis. In five patients no underlying or associated condition was identified. All patients had bleeding symptoms, 5/10 patients had major bleedings, and 7/10 patients were treated with bypassing agents. All patients received corticosteroids; 7/10 patients received immunosuppressive combination therapy.
Acquired haemophilia A is a rare disease, but manageable despite the advanced patient age and comorbidities. Its incidence in Southern Switzerland is higher than previously suspected. Read the full article here.