Swiss Medical Weekly (Mar 2023)

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

  • Andrea Ruberti,
  • Johanna A. Kremer Hovinga,
  • Federico Nappi,
  • Aurora Vettese,
  • Elena Bianchi,
  • Eliana Fernandes,
  • Elena Galfetti,
  • Rita Monotti,
  • Pamella Paul,
  • Stefano Regazzoni,
  • Daniela Valente,
  • Davide Rossi,
  • Georg Stussi,
  • Bernhard Gerber

DOI
https://doi.org/10.57187/smw.2023.40048
Journal volume & issue
Vol. 153, no. 3

Abstract

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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.