Acquired haemophilia and postpartum haemorrhage treated with internal pudendal embolisation

Abstract

Case report A 32 year old healthy nulliparous woman had a normal delivery at term. Primary postpartum haemorrhage of 500 mL occurred within minutes of delivery and settled with an infusion of syntocinon and suturing of a vaginal tear. Bleeding recurred within hours and she underwent examination in theatre with evacuation of clots and placental tissue and resuturing. The estimated blood loss was 2600 mL and she had a three-unit blood transfusion. On the following two days, she was transfused a further six units. She went home on day three. She had further bleeding and was commenced on antibiotics and readmitted on day six when she underwent general anaesthesia and resuturing. By day seven, her clotting results showed an activated partial thromboplastin time (APPT) of 78.2, ratio 2.8 and a prothrombin time (PT) of 10.9, ratio 0.9. Tests revealed her factor VIII level at <1% of normal. A diagnosis of acquired factor VIII inhibitor was made at a level of 15 Bethesda units. She was commenced on prednisolone 1 mg/kg/day and had two doses of factor VIIa 360 IU. Subsequently, her loss fluctuated and she had factor VIIa and blood transfused. By day 16, loss increased and she was given desmopressin (DDAVP) 0.3 Ag/kg, giving initial improvement. Continued bleeding, despite further DDAVP and factor VIIa, prompted transfer to the regional unit. At the time of transfer, 19 days since delivery, she had received more than 20 units of packed cells, five doses of factor VIIa and two doses of DDAVP. She was on tranexa-mic acid 1 g qds, prednisolone 60 mg od and ferrous sul-phate. Over the subsequent three days, repeated attempts to control the bleeding with vaginal packing and resuturing had failed. She was transferred to the angiography department and selective bilateral internal pudendal artery embolisation was performed via a right common femoral artery catheter under local anaesthetic. Arterial blushing was seen in the vaginal wall and both internal pudendal arteries were embolised with gelatine sponge particles. Local pressure was applied to the puncture site in the groin and factor VII infusions were needed both at the end of the embo-lisation procedure and on day three to control local oozing. The following day, she started mobilising and the aprotinin was discontinued. Inspection of the perineal area showed no evidence of ischaemic damage. She was discharged home, 31 days after she had given birth, on prednisolone 60 mg od and tranexamic acid 500 mg tds. Discussion Acquired factor VIII deficiency has an incidence of approximately one per million. 1 It is due to development of antibodies against factor VIII. Most cases occur in healthy individuals without discernible risk factors, but it is also associated with autoimmune conditions such as rheumatoid arthritis and systemic lupus erythematosus, inflammatory bowel disease, multiple sclerosis and malignancies. 2 In up to 11% of cases, the associated factor is a recent or ongoing pregnancy. 2,3 Acquired haemophilia may occur in relation to any pregnancy but the risk appears to...

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].…”

“…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.…”

“…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].…”

Management of severe postpartum haemorrhage: an updated protocol

“…Selective angiographic embolisation requires only local anaesthesia. It may be carried out even on patients with a severe coagulopathy [22], although clotting abnormalities are preferably corrected before the procedure. If the blood loss originates from the uterus, both uterine arteries should be occluded because of the existence of innumerable vascular anastomoses between the left and the right side of the uterus.…”