فهرست نشریات علمی دسترسی آزاد ایران

Background & aim: Bernard-Soulier syndrome (BSS) is known as a rare congenital bleeding disorder, characterized by thrombocytopenia and the presence of giant platelets (PLTs). The treatment process is controversial. Data on the clinical course and outcome of pregnancy in women with Bernard Soulier syndrome is scattered in individual case reports. From this perspective, this case report aimed to present pregnancy associated with this syndrom.Case report: A 23-year-old primigravid woman by clinical manifestations of frequent episodes of epistaxis and thrombocytopenia hospitalized in the 38th weeks of pregnancy. She was undergoing frequent PLT transfusions, had a vaginal delivery with episiotomy through which a baby girl was born with an Apgar score of 8-9 and a normal birth weight. The patient was then discharged after six days in good condition. Due to the rarity of the disease, it may not appear foremost on the clinician's differential. Affected individuals tend to bleed excessively.Conclusion: Pregnancy in such cases is unpredictable, so its management is still controversial and merely based on multi-specialty consultation and practice. In this line, PLT transfusion, recombinant activated factor VII (rFVIIa) administration, and prescription of desmopressin should be considered to shorten bleeding time, but anti-PLT medications must be avoided.