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One in a Million: Post Partum Haemorrhage with multiple clotting factor deficiency
Abstract Number: F2C-6
Abstract Type: Case Report/Case Series
Introduction: Combined factor V & factor VIII (F5F8D) is an extremely rare, autosomal recessive inherited disorder with an incidence of ≈ 1:1,000,000.(1) We report the case of a patient who had a vaginal delivery with subsequent post-partum haemorrhage due to an unreported F5F8D.
Case report: A 29-year-old nulliparous parturient presented in labour at 39+1 weeks gestation. Previously, she suffered 3 miscarriages with no investigations or follow up. She reported no significant past medical history. Within 7 hours of arrival, she delivered a live male by vaginal delivery & episiotomy on the midwifery led unit. She had active management of the 3rd stage with Syntometrine leading to a well-contracted uterus, and subsequent perineal repair. The following day she reported lower abdominal pain, and later became acutely distressed, tachypnoeic and then collapsed. A cardiac arrest call was instigated but initial assessment revealed a heart rate of 145 and SaO2 of 94% on 15L O2. Intravenous access was obtained and fluid resuscitation started. She was transferred to theatre for further management where she became agitated with a GCS of 7. ABG analysis showed a severe metabolic acidosis with a Hb of 57. Approximately 1L of clot was removed during vaginal examination and bimanual compression was performed with 2g Tranexamic acid being administered. She recieved 2 units of blood, FFP & Cryoprecipitate. She was then transferred to ICU where she had a further 2 units of blood & FFP. She was successfully extubated the next morning. On day 4 post-natally, she was transferred back to the maternity unit, where her recovery was complicated by multiple further episodes of bleeding requiring further blood products. Despite multiple discussions with the patient & ongoing Haematology review, it wasn’t until day 11 post-natally when she volunteered a history of severe F5F8D, confirmed by an obstetrician's letter from Sri Lanka. This was confirmed on testing Factor V & VIII assays.
Conclusions: Both pregnancy & delivery carry high risks of bleeding. Data on F5F8D is rare, with no guidelines on the optimal management of women with F5F8D during pregnancy. Case reports have documented the increased risk of miscarriage & PPH with bleeding disorders. Management of F5F8D using a multidisciplinary team approach, with products before, during & after delivery have led the successful outcomes.(2) Women with known clotting abnormalities would benefit from a MDT birthing plan, including possible planned induction and care on labour ward throughout. Furthermore when no clear cause of PPH can be found, early investigation into clotting deficiencies should be instigated.
1. Spreafico M, Peyvandi F. Combined Factor V and Factor VIII Deficinecy. Semin Thromb Hemost. 2009;Jun 35(4):390-9.
2. Spiliopoulos D, Kadir RA. Congenital factor V and VIII deficiency in women: a systematic review of literature and report of two new cases. Blood Coagul Fibrinolysis. 2016;Apr 27(3):237-41.