PB0168 - Acquired Hemophilia in four north European countries, a survey of 181 patients

Sunday, July 10, 2022

6:30 PM – 7:30 PM

Location: Exhibition

Presentation Format: In-Person

Presenting Author(s)

Rickard Lindahl, MA

Medical doctor
Lund University
Malmö, Skane Lan, Sweden

Co-Author(s)

AL

Anna-Elina Lehtinen

Adjunct Professor, Head of the Unit
Coagulation Disorders Unit, Department of Hematology, Helsinki University Hospital Comprehensive Cancer Center and University of Helsinki, Helsinki, Finland
Helsinki, Uusimaa, Finland
VN

Vuokko Nummi

consultant
Coagulation Disorder Unit, Helsinki University Hospital
Helsinki, Uusimaa, Finland
Timea Szanto

consultant
Coagulation Disorders Unit, Department of Hematology, Helsinki University Hospital Comprehensive Cancer Center and University of Helsinki, Helsinki, Finland
Helsinki, Uusimaa, Finland
LH

Leena Hiltunen

consultant
Department of Haemostasis, Finnish Red Cross Blood Service, Helsinki, Finland
Helsinki, Uusimaa, Finland
AO

Anna Olsson

Senior Consultant/Medical Doctor
Sahlgrenska University Hospital, Department of Medicine
Gothenburg, Vastra Gotaland, Sweden
AG

Andreas Glenthoej

Consultant
Copenhagen University Hospital/Copenhagen University
Copenhagen, Hovedstaden, Denmark
RC

Roza Chaireti

Senior Consultant/Associate Professor
(1) Department och Molecular Medicine and Surgery, Karolinska Institute och (2) Department of Haematology, Karolinska University Hospital,
Stockholm, Stockholms Lan, Sweden
IV

Ines Vaide

MD, PhD student
Department of Hemato-Oncology, Institute of Clinical Medicine, University of Tartu, Estonia
Tartu, Tartumaa, Estonia
Eva Funding, MD

Senior consultant, associate professor
Rigshospitalet
Copenhagen, Hovedstaden, Denmark
EZ

Eva Zetterberg, MD PhD

Senior Consultant/Associate Professor
Department of Hematology, oncology and radiation physics, Coagulation Unit, Skane University Hospital, Malmö, and Lund University, Sweden
Malmö, Skane Lan, Sweden

Background: Acquired haemophilia A (AHA) is a rare bleeding disorder due to antibodies against coagulation factor VIII. Treatment involves the use of coagulation concentrates as well as immunosuppressive treatment (IST). Current guidelines on AHA are based on retrospective data, derived from studies conducted before 2015. No studies have been performed in the Nordic countries in recent times.

Aims: To collect retrospective data on patients diagnosed with AHA in the Nordic countries 2006-2018 and compare demographic data and clinical outcomes with previously published reports.

Methods: In the Nordic Acquired Haemophilia A study, data was collected in 2020-2021 by six haemophilia centers: three Swedish, one Finnish, one Danish and one Estonian. Data on demographics, underlying conditions, bleeding episodes, haemostatic and immunosuppressive therapy (IST), response to therapy, adverse events, relapse and death, were collected.

Results: The study included 181 patients. Median age at diagnosis was 76 years, with even sex distribution, 48% females and 52% males. Type of and severity of bleeding was comparable to the large EACH2 study (Table 1). Bleedings were primarily treated with recombinant FVIIa (rFVIIa) and activated prothrombin complex concentrate (APCC). Treatment of bleeds with rFVIIa was less efficient than in the EACH2 study (55% vs. 91% of treatments considered efficient). As IST, most patients were treated with corticosteroids as monotherapy, but the highest success rate was seen in patients treated with rituximab and corticosteroids in combination (82%) (Table 2).

Conclusion(s): Data on 181 patients collected from 6 centers in 4 north European countries show similar demographic and clinical features as in previous studies on AHA. However, the success rate of rFVIIa in treatment of bleeds was lower than in previous studies. Rituximab based regimens were the most efficient IST.