دورية أكاديمية
Acquired haemophilia A in southern Switzerland from 2013 to 2019: a case series.
| العنوان: | Acquired haemophilia A in southern Switzerland from 2013 to 2019: a case series. |
|---|---|
| المؤلفون: | Ruberti, Andrea Alberto, Kremer Hovinga, Johanna A, Federico, Nappi, Aurora, Vettese, Elena, Bianchi, Eliana, Fernandes, Elena, Galfetti, Rita, Monotti, Pamella, Paul, Stefano, Regazzoni, Daniela, Valente, Davide, Rossi, Georg, Stussi, Bernhard, Gerber |
| المصدر: | Ruberti, Andrea Alberto; Kremer Hovinga, Johanna A; Federico, Nappi; Aurora, Vettese; Elena, Bianchi; Eliana, Fernandes; Elena, Galfetti; Rita, Monotti; Pamella, Paul; Stefano, Regazzoni; Daniela, Valente; Davide, Rossi; Georg, Stussi; Bernhard, Gerber (2023). Acquired haemophilia A in southern Switzerland from 2013 to 2019: a case series. Swiss medical weekly, 153, p. 40048. EMH Schweizerischer Ärzteverlag 10.57187/smw.2023.40048 |
| بيانات النشر: | EMH Schweizerischer Ärzteverlag |
| سنة النشر: | 2023 |
| المجموعة: | BORIS (Bern Open Repository and Information System, University of Bern) |
| مصطلحات موضوعية: | 610 Medicine & health |
| الوصف: | 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. |
| نوع الوثيقة: | article in journal/newspaper |
| وصف الملف: | application/pdf |
| اللغة: | English |
| العلاقة: | https://boris.unibe.ch/181593Test/ |
| الإتاحة: | https://doi.org/10.57187/smw.2023.40048Test https://boris.unibe.ch/181593/1/18632521221144060.pdfTest https://boris.unibe.ch/181593Test/ |
| حقوق: | info:eu-repo/semantics/openAccess |
| رقم الانضمام: | edsbas.2B1A9600 |
| قاعدة البيانات: | BASE |
| الوصف غير متاح. |