Abstract Number: PB2572
Meeting: ISTH 2020 Congress
Background: Severe Factor V deficiency is a rare disorder and literature on management of pregnancy is limited. We report on four pregnancies in two such women registered at our Centre.
Aims: The two patients with severe factor V deficiency had very different phenotypes and presentation. They both required very different approaches to the management of their pregnancies.The aim of this abstract is to shed light on the varying presentation and management-strategies that were required in four pregnancies in the two women.
Methods: Both women had no clear family history of factor V deficiency.
The first woman presented in her mid-20s and had three consecutive-pregnancies, none of which were complicated by antepartum-haemorrhage. In order to cover delivery she was given virally inactivated octaplas at onset of labour, and a pool of platelets for intra-platelet content of factor V. Epidural/spinal analgesia was not permitted. She also required tranexamic acid postpartum for 2 weeks.
The second woman who was in her late 20s had no previous history of bleeding and no surgical challenges. However, she presented on multiple occasions with significant antepartum haemorrhage which required hospital admission. On a couple of occasions the bleeding was severe enough to require cover with octaplas and tranexamic acid. On three other occasions the antepartum-haemorrhage episodes were managed with tranexamic acid alone.
Results: The three pregnancies in the first woman resulted in three vaginal deliveries of normal infants. Although she did not have bleeding she had severe back pain and symphysis pubis pain which were unrelated. The sole pregnancy in the second woman resulted in a normal vaginal delivery.
Conclusions: The clinical phenotypes of pregagncies can differ in severity despite similar factor V levels. Individualised treatment plans and close liaison is required between obstetricians, haematologists and gynaecologists to manage these complex pregnancies.
To cite this abstract in AMA style:Madan B, Gray G, Holloway D. Management of Pregnancy in Severe Factor V Deficiency in Women with Varying Phenotypes [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/management-of-pregnancy-in-severe-factor-v-deficiency-in-women-with-varying-phenotypes/. Accessed May 20, 2022.
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