Acquired haemophilia A in southern Switzerland from 2013 to 2019: a case series

Swiss Med Wkly. 2023 Mar 31:153:40048. doi: 10.57187/smw.2023.40048.


Aims of the study: Acquired haemophilia A is a rare disease with an annual incidence of 1.48 per million. Based on clinical observations, we suspect a higher incidence in southern Switzerland, and aimed at providing local epidemiological data, and clinical information regarding diagnosis, treatment and outcome in our region.

Methods: All adult patients with acquired haemophilia A treated between 2013 and 2019 in our facility were included in the present retrospective analysis.

Results: We treated 11 patients with acquired haemophilia A between 2013 and 2019, resulting in an annual incidence of 4.5 per million (95% confidence interval [CI] 0-9.0). Median delay from first symptoms to diagnosis was 4.5 days, and the median age at diagnosis was 79 years (range 23-87). Possible causative conditions were: pregnancy (n = 1), polyarteritis nodosa (n = 1), myelodysplastic syndrome (n = 1), chronic human immunodeficiency virus (HIV) (n = 1), and HIV postexposure prophylaxis (n = 1). In five patients no underlying or associated condition was identified. Median activated partial thromboplastin time (aPTT)) at baseline was 79 seconds (65-117; ref. value <38 sec), and FVIII:C 2.15% (<1-3.75%). A FVIII:C <1% was present in 4/10 patients. Median FVIII-inhibitor titre was 10.3 BU/ml (2.4-75.0 BU/ml). 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. FVIII levels of ≥50% were achieved after a median of 40 days (8-62). One patient had a severe immunosuppressive therapy-related infection. An 87-years-old woman died for reasons not related to acquired haemophilia A or immunosuppressive therapy.

Conclusions: 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.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Female
  • Hemophilia A* / complications
  • Hemophilia A* / drug therapy
  • Hemorrhage
  • Humans
  • Middle Aged
  • Pregnancy
  • Rare Diseases / complications
  • Retrospective Studies
  • Switzerland
  • Young Adult

Supplementary concepts

  • Factor 8 deficiency, acquired