Severe factor X deficiency and successful pregnancy

Abstract

Case reportA 22 year old Asian primigravida, known to have severe factor X deficiency with a factor X level 4 % of normal activity (normal range 50-150 YO), booked for antenatal care at 12 weeks of gestation. Her parents were first cousins and two siblings were heterozygous carriers of the factor X deficiency trait. Her husband had a normal factor X level. Factor X deficiency was first diagnosed in 1983 when she presented with prolonged bleeding from cuts. She also suffered from menorrhagia and was given fresh frozen plasma as prophylaxis against excessive bleeding before a dental extraction. An ultrasound scan confirmed the gestational age and showed a singleton pregnancy. Combined antenatal care by an obstetrician and a haematologist was planned.She was well at 18 weeks of gestation and an ultrasound scan showed apparently normal fetal anatomy. At 22 weeks she was admitted to hospital with vaginal bleeding, abdominal pain and tenderness over the fundus of the uterus. An ultrasound scan showed a fundal retroplacental haematoma measuring 55 x 25 mm. Fetal growth and the amniotic fluid volume were normal. A Kleihauer-Betke test was negative. Her factor X level was only 2 % of normal activity. After administration of two units of Bio-Product Laboratory (BPL) Factor IXA (composition per unit: 500 iu factor X, 500 iu antithrombin 111, 550 iu factor IXA, 600iu factor I1 and 5OOOiu heparin), her factor X level rose to 37% of normal activity.The day after BPL Factor IXA infusion she developed chest pain and shortness of breath. Her lungs were clinically clear, and there was no associated calf pain. Oxygen saturation (PaO,) was 97 %. There was concern about possible pulmonary embolism, but the clinical signs improved rapidly, and a chest X-ray and an electrocardiogram (ECG) were normal. After two days, the vaginal bleeding stopped. Four days later a repeat ultrasound scan showed that the haematoma had not increased in size, and she was discharged home with a follow up appointment in the antenatal clinic a week later. At antenatal visits at 23,27, 31 and 33 weeks fetal growth was satisfactory on ultrasound scans and the haematoma was getting smaller.At 34 weeks of gestation she was readmitted with a pyrexia of 37.8 "C, mild haemoptysis, shortness of breath, Correspondence: Dr J. C. Konje, Department of Obstetrics and Gynaecology, Clinical Sciences Building, Leicester Royal Infirmary, Leicester LE2 7LX, UK.and generalised lower abdominal tenderness. There was no vaginal bleeding, and fetal movements were normal. An ECG and a chest X-ray were normal, and a viral illness was diagnosed. After a few days the chest symptoms improved, but the abdominal pain increased and an ultrasound scan revealed another retroplacental haematoma, in a different site, measuring 48 x 56 mm. The old haematoma was still present and measured 15 x 12 mm. Later the same day she started having regular uterine contractions and was transferred to the labour ward where she was rehydrated and given two units of BPL Factor IXA. Four units of blood were cro...

Severe Factor X Deficiency in Pregnancy-An Obstetric Challenge

“…The coagulation parameters in our patient had improved during pregnancy when compared to non-pregnant levels. Konje et al 8 reported a case of FX deficiency which was complicated by recurrent retro placental bleeding in the antenatal period which was managed conservatively by the use of BPL factor IXA (composition per unit: 500 IU factor X, 500 IU ant thrombin III, 550 IU factor IXA, 600 IU factor II and 5000 IU Heparin). Kumar & Mehta 9 reported four pregnancies in one woman with FX deficiency, theorizing that prophylactic clotting factor replacement was instrumental in the favorable pregnancy outcome.…”

Congenital Factor X deficiency in women: A systematic review of the literature

“…The haematoma remained stable during the follow‐up, and the patient was delivered by c‐section at 34 weeks due to preterm labour . The third case (FX 0.02 IU/mL) presented with a retroplacental haematoma diagnosed on ultrasound scan at 22 and 34 weeks, and the patient had a successful delivery by c‐section at 39 weeks for foetal distress . The fourth case (FX 0.1 IU/mL) presented with vaginal bleeding at 6 and 13 weeks.…”

Two Episodes of Hemoperitoneum from Luteal Cysts Rupture in a Patient with Congenital Factor X Deficiency

“…Because treatment with high doses or prolonged administration of prothrombin complex concentrates rich in FX increases the risk of thromboembolism and disseminated intravascular coagulation, it is not recommended that the FX activity is raised more than 50% [2]. However, there are specific prothrombin complex concentrates containing antithrombin III and heparin as prophylaxis against thromboembolism [7].…”

“…The patient should receive an initial dose of 15-20 ml/kg body weight, followed by 3-6 ml/kg every 24 h. The rationale for such therapy is that the biological half-life of FX is 40 h. Also, prothrombin complex concentrates rich in FX have been used [2]. These concentrates are now the preferred therapy because they are virally inactivated, and the risk of volume overload during treatment is less compared with FFP [7]. Because treatment with high doses or prolonged administration of prothrombin complex concentrates rich in FX increases the risk of thromboembolism and disseminated intravascular coagulation, it is not recommended that the FX activity is raised more than 50% [2].…”