Hamostaseologie 2019; 39(S 01): S1-S92
DOI: 10.1055/s-0039-1680124
SY13 Acquired Haemophilia
Georg Thieme Verlag KG Stuttgart · New York

Experience in the Treatment of Acquired Haemophilia A (AHA) in 26 Patients in Relation to Bleeding Control and Induction of Immune Tolerance

G. Di Prinzio
1   Institut für Experimentelle Hämatologie und Transfusionsmedizin (IHT), Uniklinikum Bonn, Bonn, Germany
,
G. Goldmann
1   Institut für Experimentelle Hämatologie und Transfusionsmedizin (IHT), Uniklinikum Bonn, Bonn, Germany
,
S. Horneff
1   Institut für Experimentelle Hämatologie und Transfusionsmedizin (IHT), Uniklinikum Bonn, Bonn, Germany
,
C. Klein
1   Institut für Experimentelle Hämatologie und Transfusionsmedizin (IHT), Uniklinikum Bonn, Bonn, Germany
,
N. Marquardt
1   Institut für Experimentelle Hämatologie und Transfusionsmedizin (IHT), Uniklinikum Bonn, Bonn, Germany
,
H. Zeitler
2   Med. Klinik I, Uniklinikum Bonn, Bonn, Germany
,
J. Nadal
3   Institut für Medizinische Biometrie, Informatik und Epidemiologie, Bonn, Germany
,
J. Oldenburg
1   Institut für Experimentelle Hämatologie und Transfusionsmedizin (IHT), Uniklinikum Bonn, Bonn, Germany
› Author Affiliations
Further Information

Publication History

Publication Date:
13 February 2019 (online)

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Acquired Haemopophilia A (AHA) is a rare and difficult-to-treat disease, with high mortality. For more than 25 years we have treated patients with AHA in our hospital. By most of them high dose FVIII concentrates have been employed to control bleeding.

Patients and Methods: We conducted a retrospective review on 26 patients with AHA who had been treated between 2011 and 2017 at our institution. In most patients the disease presented with bleeding symptoms, that were treated with high dose FVIII infusion. We evaluated the effectivity of FVIII in controlling bleeding, as well as other therapeutic interventions, in lowering bleeding rates and in achieving more rapidly complete/partial remission. Subgroup analysis was performed according to different patients base-characteristics (age, inhibitor titer, FVIII concentrations at diagnosis, comorbidities) and combination of therapeutic interventions.

Results: 19 patients presented a high antibody titer (> 5 Bethesda Units, BU) while in 7 patients the antibody titer was < 5 BU. The average antibody titer was 32,95 BU and complete remission was achieved after a median of 43 days. In our preliminary analysis the patient-group with a low inhibitor titer (< 5 BU) and a high residual FVIII activity presented less bleeding events, a shorter FVIII substitution time and hospital stay as well as a reduced number of infections and episodes of leukopenia. The application of Rituximab, Immunoadsorption and continuous infusion of FVIII did not seem to be associated with a better outcome. Employment of activated Factor VII (FVIIa) was transitory required in 6 patients. The median value of the time needed for FVIII increase to > 10% was 4 days.

Conclusion: Our experience primarily suggests that replacement with high-dose FVIII in AH allows a rapid FVIII-level increase and a rapid bleeding-control.

First both authors contributed equally to this article.