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Acquired Haemophilia A in four north European countries : survey of 181 patients

Lindahl, Rickard LU ; Nummi, Vuokko LU ; Lehtinen, Anna Elina ; Szanto, Timea ; Hiltunen, Leena ; Olsson, Anna ; Glenthoej, Andreas ; Chaireti, Roza LU ; Vaide, Ines and Funding, Eva LU , et al. (2023) In British Journal of Haematology 201(2). p.326-333
Abstract

Acquired haemophilia A (AHA) is a rare bleeding disorder caused by acquired antibodies against coagulation factor VIII. In the Nordic countries, treatment and outcomes have not been studied in recent times. To collect retrospective data on patients diagnosed with AHA in the Nordic countries between 2006 and 2018 and compare demographic data and clinical outcomes with previously published reports, data were collected by six haemophilia centres: three Swedish, one Finnish, one Danish and one Estonian. The study included 181 patients. Median age at diagnosis was 76 (range 5–99) years, with even gender distribution. Type and severity of bleeding was comparable to that in the large European Acquired Haemophilia Registry study (EACH2).... (More)

Acquired haemophilia A (AHA) is a rare bleeding disorder caused by acquired antibodies against coagulation factor VIII. In the Nordic countries, treatment and outcomes have not been studied in recent times. To collect retrospective data on patients diagnosed with AHA in the Nordic countries between 2006 and 2018 and compare demographic data and clinical outcomes with previously published reports, data were collected by six haemophilia centres: three Swedish, one Finnish, one Danish and one Estonian. The study included 181 patients. Median age at diagnosis was 76 (range 5–99) years, with even gender distribution. Type and severity of bleeding was comparable to that in the large European Acquired Haemophilia Registry study (EACH2). Bleedings were primarily treated with activated prothrombin complex concentrate (aPCC) with a high success rate (91%). For immunosuppressive therapy, corticosteroid monotherapy was used most frequently and this may be the cause of the overall lower clinical remission rate compared to the EACH2 study (57% vs. 72%). Survey data on 181 patients collected from four north European countries showed similar demographic and clinical features as in previous studies on AHA. aPCC was used more frequently than in the EACH2 study and the overall remission rate was lower.

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organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
acquired haemophilia A, diagnosis, haemostatic treatment, immunosuppression, outcome
in
British Journal of Haematology
volume
201
issue
2
pages
326 - 333
publisher
Wiley-Blackwell
external identifiers
  • scopus:85144340768
  • pmid:36541135
ISSN
0007-1048
DOI
10.1111/bjh.18611
language
English
LU publication?
yes
id
9664c0e7-c2d2-43e1-a01a-291010aa7c57
date added to LUP
2023-01-23 14:31:31
date last changed
2024-06-25 19:33:51
@article{9664c0e7-c2d2-43e1-a01a-291010aa7c57,
  abstract     = {{<p>Acquired haemophilia A (AHA) is a rare bleeding disorder caused by acquired antibodies against coagulation factor VIII. In the Nordic countries, treatment and outcomes have not been studied in recent times. To collect retrospective data on patients diagnosed with AHA in the Nordic countries between 2006 and 2018 and compare demographic data and clinical outcomes with previously published reports, data were collected by six haemophilia centres: three Swedish, one Finnish, one Danish and one Estonian. The study included 181 patients. Median age at diagnosis was 76 (range 5–99) years, with even gender distribution. Type and severity of bleeding was comparable to that in the large European Acquired Haemophilia Registry study (EACH2). Bleedings were primarily treated with activated prothrombin complex concentrate (aPCC) with a high success rate (91%). For immunosuppressive therapy, corticosteroid monotherapy was used most frequently and this may be the cause of the overall lower clinical remission rate compared to the EACH2 study (57% vs. 72%). Survey data on 181 patients collected from four north European countries showed similar demographic and clinical features as in previous studies on AHA. aPCC was used more frequently than in the EACH2 study and the overall remission rate was lower.</p>}},
  author       = {{Lindahl, Rickard and Nummi, Vuokko and Lehtinen, Anna Elina and Szanto, Timea and Hiltunen, Leena and Olsson, Anna and Glenthoej, Andreas and Chaireti, Roza and Vaide, Ines and Funding, Eva and Zetterberg, Eva}},
  issn         = {{0007-1048}},
  keywords     = {{acquired haemophilia A; diagnosis; haemostatic treatment; immunosuppression; outcome}},
  language     = {{eng}},
  number       = {{2}},
  pages        = {{326--333}},
  publisher    = {{Wiley-Blackwell}},
  series       = {{British Journal of Haematology}},
  title        = {{Acquired Haemophilia A in four north European countries : survey of 181 patients}},
  url          = {{http://dx.doi.org/10.1111/bjh.18611}},
  doi          = {{10.1111/bjh.18611}},
  volume       = {{201}},
  year         = {{2023}},
}