Background: Bernard Soulier Syndrome is a rare inherited platelet disorder with mainly autosomal- recessive inheritance. The frequency is 1 in one-million people. It is characterised by macro-thrombocytopenia, reduced expression of platelet-glycoprotein Ib-IX-V  and genetic mutations. Literature on obstetric-management is limited in this rare disorder. We report on two pregnancies in a single patient.

Aims: This patient had a life-long history of menorrhagia, gastrointestinal and urinary-tract bleeding. She required a multidisciplinary-approach to the management of her pregnancies and deliveries. The aim of this report is to shed light on challenges of obstetric management-strategies that are required.

Methods: The patient received counselling regarding increased risk of bleeding associated with pregnancy. However, she made an informed decision to proceed with a planned first pregnancy. The second pregnancy occurred due to failure of contraception and she decided to proceed with this. Due to maternal co-morbidities, an elective caesarean section was planned for both the pregnancies. Close liaison with the National Blood Service was essential as she had anti-D antibodies and for monitoring of HPA and HLA platelet antibodies.

Results: The antepartum-course in both pregnancies was complicated by episodes of gastrointestinal-bleeding necessitating hospital admissions. During the first delivery by caesarean section the blood loss was 600ml . However, it was complicated post-operatively by an infected pelvic haematoma which required laparotomy and drainage.  The second delivery by elective caesarean section was complicated by 1.5 litre post-partum haemorrhage. In both deliveries, intrapartum and post-partum haemorrhage was managed with blood product support requiring HLA matched platelets, red cell transfusions, recombinant VIIa and tranexamic acid. The two pregnancies resulted in delivery of two healthy female infants.

Conclusions: Pregnancy, especially labour and delivery are complicated by a significantly increased risk in bleeding in Bernard Soulier Syndrome. Individualised treatment-plans and close liaison is required between obstetricians, haematologists, gynaecologists and blood transfusion service to manage these complex pregnancies.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/bernard-soulier-syndrome-in-pregnancy-management-of-two-pregnancies-in-a-patient-with-variant-bernard-soulier-syndrome/