Acquired Haemophilia A in four north European countries: survey of 181 patients
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Acquired Haemophilia A in four north European countries : survey of 181 patients. / Lindahl, Rickard; Nummi, Vuokko; Lehtinen, Anna Elina; Szanto, Timea; Hiltunen, Leena; Olsson, Anna; Glenthoej, Andreas; Chaireti, Roza; Vaide, Ines; Funding, Eva; Zetterberg, Eva.
I: British Journal of Haematology, Bind 201, Nr. 2, 2023, s. 326-333.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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TY - JOUR
T1 - Acquired Haemophilia A in four north European countries
T2 - survey of 181 patients
AU - Lindahl, Rickard
AU - Nummi, Vuokko
AU - Lehtinen, Anna Elina
AU - Szanto, Timea
AU - Hiltunen, Leena
AU - Olsson, Anna
AU - Glenthoej, Andreas
AU - Chaireti, Roza
AU - Vaide, Ines
AU - Funding, Eva
AU - Zetterberg, Eva
N1 - Publisher Copyright: © 2022 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.
PY - 2023
Y1 - 2023
N2 - 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.
AB - 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.
KW - acquired haemophilia A
KW - diagnosis
KW - haemostatic treatment
KW - immunosuppression
KW - outcome
U2 - 10.1111/bjh.18611
DO - 10.1111/bjh.18611
M3 - Journal article
C2 - 36541135
AN - SCOPUS:85144340768
VL - 201
SP - 326
EP - 333
JO - British Journal of Haematology
JF - British Journal of Haematology
SN - 0007-1048
IS - 2
ER -
ID: 335054819