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...
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SMW supporting association (Trägerverein Swiss Medical Weekly SMW)
2023-03-01
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| Series: | Swiss Medical Weekly |
| Online Access: | https://smw.ch/index.php/smw/article/view/3309 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Andrea Ruberti |
| collection | DOAJ |
| description |
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.
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| format | Article |
| id | doaj-art-b9cd8e6b1af4463c9eb5e55afec7ae86 |
| institution | OA Journals |
| issn | 1424-3997 |
| language | English |
| publishDate | 2023-03-01 |
| publisher | SMW supporting association (Trägerverein Swiss Medical Weekly SMW) |
| record_format | Article |
| series | Swiss Medical Weekly |
| spelling | doaj-art-b9cd8e6b1af4463c9eb5e55afec7ae862025-08-20T02:23:08ZengSMW supporting association (Trägerverein Swiss Medical Weekly SMW)Swiss Medical Weekly1424-39972023-03-01153310.57187/smw.2023.40048Acquired haemophilia A in southern Switzerland from 2013 to 2019: a case seriesAndrea RubertiJohanna A. Kremer HovingaFederico NappiAurora VetteseElena BianchiEliana FernandesElena GalfettiRita MonottiPamella PaulStefano RegazzoniDaniela ValenteDavide RossiGeorg StussiBernhard Gerber 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. https://smw.ch/index.php/smw/article/view/3309 |
| spellingShingle | 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 Acquired haemophilia A in southern Switzerland from 2013 to 2019: a case series Swiss Medical Weekly |
| title | Acquired haemophilia A in southern Switzerland from 2013 to 2019: a case series |
| title_full | Acquired haemophilia A in southern Switzerland from 2013 to 2019: a case series |
| title_fullStr | Acquired haemophilia A in southern Switzerland from 2013 to 2019: a case series |
| title_full_unstemmed | Acquired haemophilia A in southern Switzerland from 2013 to 2019: a case series |
| title_short | Acquired haemophilia A in southern Switzerland from 2013 to 2019: a case series |
| title_sort | acquired haemophilia a in southern switzerland from 2013 to 2019 a case series |
| url | https://smw.ch/index.php/smw/article/view/3309 |
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