Bernard-Soulier syndrome (BSS) with uncontrollable menorrhagia

Abstract

Menorrhagia is a common problem in women of reproductive age group and 5% of women between 30 and 49 years of age group consult their general practitioner for this problem. Menorrhagia occurring in adolescent age group and perimenopausal age group is chiefly due to anovulatory cycles. In perimenopausal age group, local pathology like leiomyomas can also contribute to menorrhagia. Recently, bleeding disorders like von Willebrand Disease, single coagulation factor deficiencies particularly factor XI, VIII, Factor V and platelet function disorders have been found to be prevalent in patients presenting with menorrhagia. Bernerd soulier syndrome is extremely rare haemostatic disorder due deficiency in GP IB/IX/V receptor complex present on platelet membrane which is necessary for platelet aggregation. As a result of the defect patients have prolonged bleeding time, which might vary in symptoms ranging from spontaneous self-limiting epistaxis to life threatening haemorrhage. Here we report a case of 31-year-old woman known case of Bernard soulier syndrome with severe menorrhagia treated with mirena insertion and course of complication and its management.