Document Type : Case Report
Authors
1
MSc Student, Department of Midwifery, School of Nursing and Midwifery, Mashhad University of Medical Sciences, Mashhad, Iran
2
Assistant Professor, Department of Obstetrics and Gynecology, Mashhad University of Medical Sciences, Mashhad, Iran
3
a) Assistant Professor, Nursing and Midwifery Care Research Center, Mashhad University of Medical Sciences, Mashhad, Iran b) Department of Midwifery, School of Nursing and Midwifery, Mashhad University of Medical Sciences, Mashhad, Iran
10.22038/jmrh.2024.73245.2165
Abstract
Background & aim: Bernard-Soulier syndrome (BSS) is known as a rare congenital bleeding disorder, characterized by thrombocytopenia and the presence of giant platelets (PLTs). From this perspective, this case report is to illustrate this illness in pregnancy.
Case report: A 23-year-old woman in the 38th weeks of pregnancy, undergoing frequent PLT transfusions, had a vaginal delivery with episiotomy and a baby girl was born with an Apgar score of 8-9 and a normal weight. The patient was then discharged after six days in good condition.
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 desmopressin use should be considered to shorten bleeding time, but anti-PLT medications must be avoided. As the postpartum period is the most critical time due to the likelihood of secondary bleeding, it is concluded that the placental transfer of maternal anti-PLT antibodies entails the increased risk of fetal and neonatal alloimmune thrombocytopenia (FNAIT) and severe bleeding, such as intracranial hemorrhage (ICH).
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