Bernard-Soulier syndrome in pregnancy.
Bernard-Soulier syndrome (BSS) is a rare autosomal recessively inherited bleeding disorder. Pregnancy in patients with BSS is characterized by ante-, intra-, or postpartum haemorrhage, which may be delayed and severe. There is no consensus in the management of BSS in pregnancy and so far only 16 pregnancies in nine patients have been described. We report a further three pregnancies in two women with the syndrome. We also outline our management of pregnant patients with BSS.
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Bradford Teaching Hospitals NHS Foundation Trust, Duckworth Lane, Bradford BD9 6RJ, UK.
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D000293:Adolescent; D000328:Adult; D001606:Bernard-Soulier Syndrome / Q000150:complications* / Q000628:therapy; D001792:Blood Platelets / Q000473:pathology; D003241:Consanguinity; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D017707:Erythrocyte Transfusion; D005260:Female; D006801:Humans; D010974:Platelet Aggregation; D017713:Platelet Transfusion; D006473:Postpartum Hemorrhage / Q000628:therapy; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000097:blood / Q000209:etiology* / Q000628:therapy; D014148:Tranexamic Acid / Q000627:therapeutic use
D014148:Tranexamic Acid; D003894:Deamino Arginine Vasopressin