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Use of TEG trace in the management of homozygous factor X deficiency for successful vaginal delivery
Abstract Number: T-45
Abstract Type: Case Report/Case Series
Isolated Factor X deficiency is one of the rarest forms of inherited coagulation disorders; with an autosomal recessive pattern and an incidence of 1:1,000,000 (1) in the general population. Factor X plays a crucial role in the coagulation cascade, as the first enzyme in the common pathway. Pregnancy in a homozygous patient with factor X deficiency is often complicated by recurrent miscarriages, uterine bleeding and premature labor. We present a successful vaginal delivery of a parturient with severe Factor X deficiency whose coagulation status was assessed with thromboelastography (TEG).
Case: 22 yr old G1 P0, 36 week parturient with factor X deficiency presented in labor for planned vaginal delivery. She was diagnosed with factor X deficiency at the age of 2 with history of spontaneous intra-abdominal bleeding which was treated by ex-laporotomy (x2) as a teenager. She also had a history of spontaneous joint bleeding treated by Factor IX Complex. On admission her laboratory values included Hb: 11.4, Platelets 240, PT: 54.7, INR: 6.2, PTT: 55 and Factor X Assay: 1%; with significantly elevated TEG R value of 12.1. Since previous use of factor IX complex was successful, the plan included administering 3 units of Factor IX Complex just before the birth to be repeated after delivery, if there was significant bleeding. Cross-matched RBC and FFP were readily available; with a plan to proceed to general anesthesia for caesarean section due to higher risk associated with neuraxial anesthesia based on TEG and other values. Patient delivered a healthy female baby vaginally; received 3 units of Factor IX Complex just prior to delivery and 3 units Factor IX Complex in the post partum period; with the total blood loss of 600ml and discharged home uneventfully on day 3.
Discussion: Clinical management of parturients with coagulopathies are challenging with no routine laboratory values that assess full coagulation status. TEG is useful in providing additional clinical information to assess the risk of bleeding in these patients and whether to consider neuraxial anesthesia. Due to the rarity of the disorder, very few published data regarding the outcome of pregnancy is available (2) and we believe that our experience about this rare coagulation disorder adds valuable information from use of the TEG for future reference.
1. Romagnolo C, Burati S, Ciaffoni S, Fattori E, Franchi M, Zanon E, Girolami A. Severe factor X deficiency in pregnancy: case report and review of the literature. Haemophilia. 2004 Sep;10(5):665-8.
2. Mamopoulos A, Vakalopoulou S, Lefkou E, Fileli A, Garipidou V, Mavromatidis G, Dinas K, Karagiannis V. Pregnancy in a patient with severe factor X deficiency. Haemophilia. 2009 Nov;15(6):1351-3.