Severe Factor X Deficiency in Pregnancy-An Obstetric Challenge

Abstract

Abbreviations: FX, factor X; FX,C, factor X coagulant; FFP, fresh frozen plasma; PCC, prothrombin complex concentrate Case reportA 23year old lady with one previous abortion, presented to the antenatal outpatient department of St. John s Hospital, for the first time at 20weeks of gestation. She gave a history of prolonged bleeding following trivial trauma since childhood which stopped on compression. She also had hemarthrosis and spontaneous gum bleeds since childhood. At puberty, she gave history of menorrhagia which responded to oral Tranexamic acid. At 17years of age, patient had a severe episode of menorrhagia leading to hemorrhagic shock. She received multiple blood products and eventually required bilateral uterine artery ligation and a few haemostatic sutures on the uterus (exact details of which are not known and have not been documented). Soon after marriage, at 21years of age, she was diagnosed to have blighted ovum and presented later with incomplete abortion, for which she received Misoprostol. She had heavy bleeding leading to severe anemia and a provisional diagnosis of disseminated intravascular coagulation was made. Her investigations at that time were Prothrombin time 102s, Activated thromboplastin time 65.5 s, INR 8.5, Factor X levels were <1% with normal platelet count and morphology. Thus, a diagnosis of severe factor X deficiency was made. During the present pregnancy, she was referred to the antenatal outpatient department. She had developed gestational diabetes mellitus which was controlled with diet. The antenatal period was uneventful. As there was no significant bleeding in this pregnancy, the coagulation parameters were not repeated until term. Ultrasound done at 37weeks confirmed a flexed breech presentation. At 37weeks of gestation, patient received FFPs at 1 bag/10kg body weight to assess her laboratory haemostatic parameters and her investigations were as shown in Table 1. Elective cesarean delivery was planned at 38weeks of gestation and external cephalic version was not considered due to the previous scar on the uterus (i.e. haemostatic sutures on the uterus following an episode of menorrhagia at 17years of age). Patient underwent emergency cesarean delivery at 37weeks of gestation, as a spontaneous retro placental bleed of about 50ml was seen on ultrasound. Patient again received the same dose of FFP and 2g of intravenous Tranexamic acid preoperatively. A healthy baby boy of weight 2.5kg was delivered as flexed breech. A previous scar was noted on the uterus. Both ovaries were normal. Intraoperatively, a peritoneal drain was left in situ. Patient developed hematuria which cleared after 12hours. Postoperatively, patient received 3units of FFP and 1g intravenous Tranexamic acid 8 hourly for 48hours and then followed by 2units of FFP and 1g intravenous Tranexamic acid 12hourly till the seventh postoperative day. Sutures were removed on the seventh postoperative day. Postoperative period was uneventful. Patient has come for follow-up and has had no signs of postpartum hemorrhage til...

Factor X Deficiency Management for Elective Cesarean Delivery in a Pregnant Patient

“…Factor X deficiency is a rare inherited coagulopathy, manifested as various degrees of bleeding diathesis [3]. The amount of factor X required to accomplish hemostasis is unknown, but levels of 10-35% are thought to be sufficient [7]. In severe cases of factor X deficiency, bleeding complications can arise in the neonatal period in the form of intracranial hemorrhage or umbilical cord stump hemorrhage (usually 1-2 weeks after birth, when the stump dries and falls off) [7].…”

“…The amount of factor X required to accomplish hemostasis is unknown, but levels of 10-35% are thought to be sufficient [7]. In severe cases of factor X deficiency, bleeding complications can arise in the neonatal period in the form of intracranial hemorrhage or umbilical cord stump hemorrhage (usually 1-2 weeks after birth, when the stump dries and falls off) [7]. Less severe cases of factor X deficiency can result in bleeding complications in situations involving hemostatic challenge, such as surgery, tissue trauma, and menstrual cycle [7].…”

“…In severe cases of factor X deficiency, bleeding complications can arise in the neonatal period in the form of intracranial hemorrhage or umbilical cord stump hemorrhage (usually 1-2 weeks after birth, when the stump dries and falls off) [7]. Less severe cases of factor X deficiency can result in bleeding complications in situations involving hemostatic challenge, such as surgery, tissue trauma, and menstrual cycle [7]. Irrespective of the level of factor X, the most commonly reported symptom in factor X-deficient patients is epistaxis, which was reported to occur in 72% of patients [7].…”

“…Less severe cases of factor X deficiency can result in bleeding complications in situations involving hemostatic challenge, such as surgery, tissue trauma, and menstrual cycle [7]. Irrespective of the level of factor X, the most commonly reported symptom in factor X-deficient patients is epistaxis, which was reported to occur in 72% of patients [7]. Regarding laboratory investigations, in standard coagulation tests consisting of PT, INR, aPTT, fibrinogen concentration, and platelet count, patients with factor X deficiency usually have prolonged PT, INR, and aPTT [7].…”

“…Irrespective of the level of factor X, the most commonly reported symptom in factor X-deficient patients is epistaxis, which was reported to occur in 72% of patients [7]. Regarding laboratory investigations, in standard coagulation tests consisting of PT, INR, aPTT, fibrinogen concentration, and platelet count, patients with factor X deficiency usually have prolonged PT, INR, and aPTT [7]. However, to make a definite diagnosis, a functional assay for factor X has to be performed, with optional molecular genetic testing for variation in the factor X gene [7].…”