Abstract
Objective Bernard-Soulier syndrome (BSS) is one of the rare inherited
platelet disorders that is characterized by macrothrombocytopenia and adhesion
abnormality due to the absence or malfunctioning of the membrane GPIb-IX-V
complex. There is no high-quality evidence on obstetric management of BSS owing
to its rarity. Here we report an uncomplicated delivery of an adolescent with
BSS and review the literature on the topic of BSS and pregnancy.
Methods PUBMED, EMBASE, COCHRANE, and Google Scholar databases were
searched up to April 2022 without language and year restriction using the terms
“Bernard Soulier” and “Pregnancy”. The primary
objectives were to evaluate maternal and fetal outcomes. The secondary
objectives were to analyze pregnancy complications, gestational age at delivery,
mode of delivery, administered prophylaxis, treatment approaches, duration of
postpartum hospitalization, and the postpartum requirement of blood and blood
product.
Results The patient was a 19-year-old and 39-week pregnant woman who was
diagnosed with BSS at the age of 10 by flow cytometry and genetic analysis.
Single donor platelet transfusions and oral tranexamic acid were administered as
prophylaxis at the peripartum period. She was delivered by cesarean section due
to failure of labor. The postpartum period was uneventful for both mother and
neonate. In the literature review, postpartum hemorrhage (PPH) was found in
52.9% (27/51) of deliveries. Late PPH occurred more frequently
than early PPH (35.3 and 31.4%, respectively). 49%
(25/51) of pregnancies had severe thrombocytopenia, and antepartum
hemorrhage was observed in 11.8% (6/51) of those. The platelet
count was in close relation to antenatal complications. 64.7%
(33/51) of the patients were delivered via cesarean section. PPH and
late PPH were found to be more common in those who delivered vaginally compared
to those who delivered by caesarean section. It was observed that PPH was less
common in women who were given prophylaxis in the peripartum period.
Conclusion BSS is an inherited macro-thrombocytopathy that may cause
adverse maternal and neonatal outcomes. The optimal mode and timing of delivery
remain unclear. A multidisciplinary approach with prophylaxis at the peripartum
period should be applied.
Key words
bernard soulier syndrome - management - postpartum hemorrhage - pregnancy - prophylaxis
