Bernard Soulier syndrome in pregnancy and delivery – a case report

K. Liebscher1, I. Niemann2, I. Bogner2, A. Siegemund3, M. Krause2, U. Scholz2 (1Leipzig, Germany, 2Leipzig, DE, 3Leipzog, DE)

Women issues in thrombosis and hemostasis
Date: 17.02.2017,
Time: 17:15 - 18:15

Objective: Background: The Bernard Soulier syndrome (BSS) is a rare bleeding disorder, inherited as an autosomal recessive trait. The main characteristics are thrombozytopenia, giant platelets and a quantitative defect of platelet membrane glycoprotein Ib-IX-V complex. BSS is associated with varying bleeding symptoms (hypermenorrhoea, epistaxis and hematoma). We report our experience about the management of a pregnancy and delivery of a patient with Bernard Soulier syndrome


Results: Case report: A 27-year-old woman with known BSS was referred due to first pregnancy. Despite the patient received platelet concentrates in its medical history, no HLA-antibodies or HPA-antibodies could be proved until now. Ex-vivo testing of recombinant factor VIIa (spiked plasma) showed a sufficient effect in coagulation. During pregnancy two bleeding episodes (gestation week 9: retroplacental hematoma, gestation week 11: epistaxis and hematoma) appeared with administration of HLA-matched platelet concentrates. Primary caesarean was at gestation week 37 due to a former laparotomic and inherited bleeding disorder with transfusion of two HLA-matched platelet concentrates without clinical complications. Monitoring of the therapy was possible by platelet count and thrombelastography.

Conclusion: This case shows that BSS can be managed in a cross-functional cooperation of obstetric, hemostaseology and transfusion medicine to minimize the bleeding risk of patient with rare bleeding disorders.