The role of recombinant factor VIIa for obstetric block in women with severe factor XI deficiency

Abstract

Regional blocks like spinal, epidural and combined spinal epidural (CSE) are relatively contraindicated in individuals with bleeding disorders. Consequently pregnant women with severe factor XI (FXI) deficiency are often denied this option during labour and caesarean section. We describe three women with severe FXI deficiency in whom regional block was performed with low-dose recombinant factor VIIa (rFVIIa) for their operative procedures during delivery. All women achieved haemostasis and had uncomplicated regional block, delivery and surgical procedures. The point of care device--rotational thromboelastometry (ROTEM) was used to monitor the patients' coagulation and determine the dose of rFVIIa to achieve in vitro haemostasis in these women that was then subsequently used in vivo. Low-dose rFVIIa seems to be effective and safe in the management of delivery and enables provision of regional blocks in women with severe FXI deficiency.

The impact of thrombin generation and rotation thromboelastometry on assessment of severity of factor XI deficiency

“…Yet, no relation of thromboelastometry parameters to FXI concentrations and genotypes was investigated. Nevertheless, ROTEM has been found useful for monitoring rFVIIa therapy and anti-fibrinolytic agents in patients with severe FXI deficiency undergoing surgery [11,14,26]. The disadvantage of ROTEM in clinical practice is that standardization of the results is difficult since the assay is best done on fresh whole blood, thus not enabling to ensure reproducibility between laboratories.…”

Gynaecological and obstetrical bleeding in women with factor XI deficiency – a systematic review

“…None of the included studies used recombinant FVIIa. A case series describes the use of recombinant FVIIa associated with uncomplicated obstetric block in women with severe FXI . The rise in FXI activity is unpredictable when using plasma therapy.…”

Why factor XI deficiency is a clinical concern

“…In patients with fXI antibody inhibitors, a strategy based on a single dose of recombinant factor VIIa (15–30 μg/kg) followed by antifibrinolytic therapy has maintained hemostasis in patients undergoing major surgery, including one case of aortic dissection repair [13]. Factor VIIa (15–30 μg/kg) every two to four hours in conjunction with antifibrinolytic therapy has been used successfully during surgery [57,58] or epidural anesthesia [59] in lieu of factor replacement in patients without inhibitors who wish to avoid plasma exposure. This strategy may be preferable in patients with very low plasma fXI levels (<1% of normal), such as homozygotes for the type II mutation, ~30% of who develop neutralizing antibodies after exposure to fXI [52].…”