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

Authors: Lindahl, R; Nummi, V; Lehtinen, A-E; Szanto, T; Hiltunen, L; Olsson, A; Glenthoej, A; Chaireti, R; Vaide, I; Funding, E; Zetterberg, E

Affiliations: Malmo & Lund Univ, Skane Univ Hosp, Dept Haematol Oncol & Radiat Phys, Coagulat Unit, Lund, Sweden. Helsinki, Helsinki Univ Hosp, Comprehens Canc Ctr, Dept Haematol,Coagulat Disorders Unit, Helsinki, Finland. Finnish Red Cross Blood Serv, Dept Haemostasis, Helsinki, Finland. Sahlgrens Univ Hosp, Dept Med, Reg Vastra Gotaland, Gothenburg, Sweden. Rigshosp, Dept Haematol, Copenhagen, Denmark. Univ Copenhagen, Dept Clin Med, Copenhagen, Denmark. Karolinska Univ Hosp, Dept Haematol, Stockholm, Sweden. Univ Tartu, Inst Clin Med, Dept Haematol & Oncol, Tartu, Estonia. Fimlab Laboratoriot Oy Ltd, Haemostasis & Platelet Lab, Vantaa, Finland.] Skane Univ Hosp, Dept Haematol Oncol & Radiat Phys, Coagulat Unit, Jan Waldenstroms Gata 16, S-20502 Malmo, Sweden.

Publication: British Journal of Haematology; 2023

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). 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.