Palsson, Ragnar2; Vidarsson, Brynjar3; Gudmundsdottir, Brynja R3; Larsen, Ole H6; Ingerslev, Jorgen7; Sorensen, Benny8; Onundarson, Pall T3
1 Department of Clinical Medicine - Department of clinical biochemistry, Department of Clinical Medicine, Health, Aarhus University2 Department of Medicine, Duke University Medical Center , Durham, North Carolina , USA .3 unknown4 Department of Clinical Medicine - Clinical Biochemistry, Department of Clinical Medicine, Health, Aarhus University5 HE Administrative Centre - Health Byg Drift, HE Administrative Centre, Health, Aarhus University6 Department of Clinical Medicine - Clinical Biochemistry, Department of Clinical Medicine, Health, Aarhus University7 Department of Clinical Medicine - Department of clinical biochemistry, Department of Clinical Medicine, Health, Aarhus University8 HE Administrative Centre - Health Byg Drift, HE Administrative Centre, Health, Aarhus University
Women with Bernard-Soulier syndrome (BSS) are considered to be at high risk of serious bleeding during childbirth. Due to the frequently occurring platelet transfusion refractoriness, alternative prophylactic therapy is required. Using rotational thromboelastometry, we evaluated the whole blood coagulation profile of a pregnant woman with BSS before and after spiking ex vivo with different concentrations of recombinant activated factor VII (rFVIIa) and fibrinogen. As experiments suggested improved clotting with clinically applicable concentrations of both agents in a complementary manner, the findings were confirmed on blood from a non-pregnant woman and three men suffering from BSS. During delivery, bleeding refractory to platelets occurred and immediately following delivery she received both rFVIIa and fibrinogen intravenously. Immediate cessation of bleeding occurred, and no postpartum hemorrhage was seen. Another woman with BSS later also received the same rFVIIa and fibrinogen treatment prophylactically after delivery without any postpartum bleeding. Eventually, the first woman during her second delivery received the same treatment again prophylactically without any bleeding. No side effects were observed during these three deliveries. Our observations suggest that rFVIIa combined with fibrinogen may provide a beneficial clinical hemostatic effect partly by separate but complementary mechanisms.