Bernard Soulier syndrome is a rare autosomal recessive disorder of platelets, was first described by Bernard and Soulierin1948(.1,2) It is characterized by platelet dysfunction, prolonged bleeding time, abnormal consumptionof prothrombin,normal to decreased number of unusually large platelets.
Pregnacy in BSS ischaractrised by bleeding episodes , especially during postpartum period.
22 year old primigravida with BSS was refered to our hospital at 34 weeks of gestation. She was diagnosed to have BSS at the age of 13 years, when she was evaluated for menorrhagia with severe anemia.
She conceived spontaneously, nine years after the diagnosis of BSS was made. I admitted her to our hospital at 9 months with pain abdomen and low platelet count [16,000] .
We successfully managed with timely platelet transfusions and casearean section as fetus was in distress and she was having bleeding per vaginum.
I thank the whole team and patient's family for their continuous support and trust in me !