Bernard-Soulier syndrome in pregnancy

Abstract

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.

Antifibrinolytic therapy with tranexamic acid in pregnancy and postpartum

“…The reports dated from 1976 [25] to 2010 [26], and the age of patients ranged from 16 to 39 years [27,28]. The majority of the cases reported management of postpartum hemorrhage with tranexamic acid [29,30,27,[31][32][33][34][35][36][37]26]. Seven studies reported antepartum hemorrhage [25,[38][39][40][41][42][43].…”

“…Seven studies reported antepartum hemorrhage [25,[38][39][40][41][42][43]. Hemorrhage associated with hematological disorders was reported in nine cases [44,29,30,31,28,[33][34][35][36][37]. The most common hematological disorder mentioned was Bernard Soulier syndrome [29,[34][35][36], followed by hemophilia [30,33], leukemia [28], other platelet disorders such as Glanzmann's thrombasthenia [44], Evan's syndrome [31], and platelet storage pool disease [37].…”

“…Hemorrhage associated with hematological disorders was reported in nine cases [44,29,30,31,28,[33][34][35][36][37]. The most common hematological disorder mentioned was Bernard Soulier syndrome [29,[34][35][36], followed by hemophilia [30,33], leukemia [28], other platelet disorders such as Glanzmann's thrombasthenia [44], Evan's syndrome [31], and platelet storage pool disease [37]. Tranexamic acid was given as treatment of induced coagulopathy amphetamine intoxication [45], postpartum hemorrhage [32], and amniotic fluid embolism [42,26].…”

“…Treatment was provided from 21 weeks to 36 weeks of gestation [25,41]. Nine studies provided information about the amount of blood loss (range 1000 --19 000 ml) [27,46,41,42,33,[35][36][37]43]. However, information about how blood loss was estimated was unclear in all the reported studies.…”

“…However, information about how blood loss was estimated was unclear in all the reported studies. In 13 women (56.5% of all case reports), the authors reported that hemorrhage 'gradually stopped' with additional management or prolonged treatment [25,38,44,29,39,27,28,[40][41][42]36,26]. In five women (21.7%) the hemorrhage 'stopped' [30,45,34,35,37] and in five women the hemorrhage was defined as 'not stopped' (21.7%) [46,32,33,37,43].…”