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

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.

Hybrid treatment of a case of obliterative hepatocavopathy (Budd-Chiari syndrome).

Progressive obliteration of the intrahepatic course of a inferior vena cava is an insidious disease that may lead to portal hypertension with progressive liver engorgement and ultimately to liver cirrhosis. Early diagnosis is extremely important so that therapeutic modalities can be offered that can favorably change the natural course of the disease. We present the case of a young woman whose obliterated vena cava could be successfully recanalized by a combined surgical and interventional technique.

[Two new very promising antithrombotic agents: pentasaccharide and ximelagatran]. / Deux nouveaux agents antithrombotiques très prometteurs: le pentasaccharide et le ximelagatran.

The main anticoagulants presently used have several drawbacks. Two new compounds (pentasaccharide and ximelagatran) have shown in recent studies several interesting properties and results. Both are of non animal origin, do not induce thrombocytopenia and do not require laboratory controls. Pentasaccharide has a better efficacy than low molecular weight heparin in major orthopaedic interventions; it will be registered soon in several countries. Ximelagatran, which can be given orally and is in phase III investigation, could replace vitamin K antagonist in the future.