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Factor XI Deficiency in Pregnancy: A Case Series
Abstract Number: 28
Abstract Type: Case Report/Case Series
Introduction: Factor XI deficiency, also known as Hemophilia C, is a rare, inherited, autosomal bleeding disorder that affects approximately 1:1,000,000 people in the general population, but can affect as many as 9% of the Ashkenazi Jewish population(1). Bleeding after surgery or trauma is a worry for patients with Factory XI deficiency and is a risk factor for postpartum hemorrhage (PPH) in the laboring parturient(2).
Case Presentation: Case one is a 32-year-old female, gravida 2, cesarea 1, at 37 weeks gestation, admitted for a non-urgent repeat cesarean section. There was a history of bleeding after surgery, including a prior cesarean section, but no pre-existing diagnosis of any bleeding disorder. Repeat c-section was uneventful, with an estimated blood loss (EBL) of 550 mL. The patient became hypotensive in the PACU and was found to have a hemoglobin concentration of 3.7g/dL. The patient was transfused with 6 units packed red blood cells and 2 units fresh frozen plasma (FFP). Hysterectomy was required to control bleeding. Postoperative investigations revealed a prolonged activated partial thromboplastin time (aPTT) of 45s with normal PT and a Factor XI activity of 30% (reference range 50-150%). Case two is a 33 year-old female, gravida 3 para 0, who presented at 40 4/7 weeks gestation for induction of labor and was requesting epidural analgesia. The patient was known to be Factor XI deficient with a Factor XI activity of 32%. Two units of FFP were administered before placement of a lumbar epidural in the L3-4 interspace, using an 18g Touhy needle. Epidural placement was uneventful and there was normal progression of labor ending in delivery of a 3795g female infant. The epidural catheter was removed 24 hours after delivery and recovery was uneventful. Case three is a 30 year-old female, gravida 3 cesarea 1, who presented to labor and delivery at 37 1/7 weeks twin gestation for repeat cesarean section. Preoperatively, aPTT was measured to be 43s (reference range 23-34) and four units of FFP were administered. After administration of three units, aPTT was 34s. The patient was taken to the operating room and received spinal anesthesia using a 25g Whitacre needle in the L4-5 interspace. Two healthy female infants were delivered with an EBL of 800mL. Two additional units of FFP were administered postoperatively. Recovery from surgery was uneventful and no further interventions were required.
Discussion: Women with Factor XI deficiency pose a unique challenge for the obstetric anesthesiologist, as these women may be at an increased risk for PPH. However, we have shown here that when diagnosed before labor, prophylactic treatment of Factor XI deficiency at the time of delivery appears to be an effective treatment in reducing the risk of postoperative bleeding.
1. Blood 1995 Jan 15;85(2):429-32.
2. Br J Obstet Gynaecol 1998 Mar;105(3):314-21.