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Peripartum Management of Severe Factor XI Deficiency – Role for ROTEM in Guiding Therapy
Abstract Number: SU-38
Abstract Type: Case Report/Case Series
Introduction: Factor XI deficiency has long presented a clinical challenge to the obstetric anesthesiologist. It has been well established that factor XI levels in these patients do not correlate with their risk of bleeding. While there is no standard of practice regarding their management, it is recommended that those with severe factor XI deficiency (activity level <15%) undergo factor replacement therapy in the peripartum period. The question of how to adequately assess the effects of this replacement therapy towards effective hemostasis remains unanswered. The adjunct of viscoelastic testing may be an important tool in providing a more complete picture of the coagulation process in these patients.
Case Report: A 34 year-old G2P1 with history of severe factor XI deficiency (baseline factor XI <1% and 2% at 35 weeks gestation) who experienced postpartum hemorrhage (PPH) after her first cesarean delivery two years prior now presents for repeat cesarean section. For her first section she received a total of 10U of FFP to normalize her factor XI level. On this admission, baseline coagulation and rotational thromboelastometry (ROTEM) were measured the night before her scheduled section. A total of 5U of FPP (15cc/kg) were then transfused. The aPTT normalized from 61.4 to 27.6 and on ROTEM, the clotting time (CT) on INTEM also normalized from 264 to 146 seconds. Additionally, on both the baseline and post-transfusion TEMograms, the rest of the parameters (A10, MCF) reflected the expected hypercoaguable changes seen with pregnancy. Given the normalization of coagulation studies with evidence of strong clot formation on ROTEM analysis, the patient received spinal anesthesia and cesarean section was performed without incident. Factor XI was measured immediately postoperatively showing an increase from 2% to 25%. The EBL was 800cc and the postoperative course was unremarkable with no evidence of PPH or spinal hematoma.
Discussion: Although previous recommendations include factor replacement therapy titrated to a level of activity of 30-40% normal, in this case, this would have required excessive plasma transfusion beyond what available studies showed the patient needed to form an effective clot. With the risk of infection, adverse transfusion reactions, and alloimmunization, therapy should be guided in a way to achieve effective hemostasis while limiting potential harm. There is great potential for using ROTEM, at least adjunctively, to demonstrate effective hemostasis following peripartum replacement therapy in patients with severe factor XI deficiency. Adapting ROTEM testing on labor and delivery units is an important tool in the assessment of global coagulation in the pregnant patient.
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