PB1229 - Haemostatic control with recombinant porcine factor VIII in acquired haemophilia A

Tuesday, June 27, 2023

18:30 – 19:30 ET

Room: Exhibition

Presenting Author(s)

AB

Anna Maria Buczma, PhD (she/her/hers)

assistant
Department of Hemostasis Disorders and Internal Medicine, Institute of Hematology and Transfusion Medicine, Warsaw, Poland
warsaw, Mazowieckie, Poland

Co-Author(s)

BB

Beata Baran

PhD, Laboratory assistant
Laboratory of Hemostasis and Metabolic Diseases, Institute of Hematology and Transfusion Medicine, Warsaw, Poland
warsaw, Mazowieckie, Poland
PP

Piotr Paluszkiewicz

Professor
Department of General and Oncologic Surgery, Institute of Hematology and Transfusion Medicine, Warsaw, Poland
warsaw, Mazowieckie, Poland
MK

Magdalena Korwin

assistant
Public Clinical Eye Hospital, Warsaw, Poland
Warsaw, Mazowieckie, Poland
AG

Adela Gwozdowska

assistant
Department of Hemostasis Disorders and Internal Medicine, Institute of Hematology and Transfusion Medicine, Warsaw, Poland
Warsaw, Mazowieckie, Poland
SJ

Slawomir Jurek

assistant
Department of Hemostasis Disorders and Internal Medicine, Institute of Hematology and Transfusion Medicine, Warsaw, Poland
Warsaw, Mazowieckie, Poland
Jerzy Windyga, Prof. dr med. (he/him/his)

Head Department of Hemostasis Disorders and Internal Medicine
Institute of Hematology and Transfusion Medicine
Warsaw, Mazowieckie, Poland

Background: Acquired haemophilia A(AHA) is an autoimmune bleeding disorder, caused by autoantibodies reducing factor VIII activity (FVIII:C). AHA manifests as spontaneous hemorrhage or bleeding induced by invasive procedures. Several treatment options for the management of bleeding episodes are available.

Aims: To describe our recent experience with novel haemostatic agent in AHA - recombinant porcine factor VIII (rpFVIII)

Methods: Four AHA patients, aged 61-89 (median 70) years were treated with rpFVIII. All patients indentified with AHA and presence of anti human FVIII (anti-hFVIII) inhibitors were also tested for anti-porcine FVIII (anti-pFVIII) inhibitors during the initial diagnostic process. Peak (30min post infusion) and trough FVIII levels were monitored to guide ongoing doses.

Results: Anti-hFVIII inhibitor titers ranged 2.5-59BU/ml. Two patients with acute bleeding and anti-pFVIII ( < 0.5UB/ml and 0.52UB/ml), initially commenced on by-passing agent (BPA), due to inadequate hemostasis, were switched to rpFVIII. The loading dose of 50U/kg and the same maintenance doses were repeated every 6 hours to achieve minimal target FVIII:C (61-105 U/dl). The bleeding was stoped in 24-48h and intervals of doses were prolonged to 8-12h. In two cases rpFVIII was used to control bleeding during surgical treatment. First patient with anti-pFVIII inhibitor titer 0.68 BU/ml was treated with loading dose of rpFVIII at 100 U/kg and this increased FVIII:C to 170%. Second patient with anti-pFVIII 7.7BU/ml obtained initially 200U/kg of rpFVIII, which increased FVIII:C to 120%. Both patients continued rpFVIII treatment with subsequent doses of 100U/kg every 8h and 200 U/kg every 4h respectively. Targeting FVIII trough levels were achieved and ranged 70-110% during 72h after surgery.

Conclusion(s): Presented data confirm that rpFVIII is effcacious to treat acute bleeds even refractory to BPA. Use of rpFVIII is an effective treatment option to control perioperative hemostasis in AHA. Presense of porcine inhibitor titer > 5BU/ml demands increased dose and frequency of rpFVIII infusion.