Combined factor V and VIII deficiency and pregnancy--need for an early protocol-based multidisciplinary management

We report the medical management of a 32-year-old primigravida, after she was found to have a combined factor V (FV) and factor VIII (FVIII) deficiency during pregnancy. A routine coagulation profile performed during the 6th month of pregnancy showed a prolonged activated partial thromboplastin time (aPTT) of 78 seconds, giving a patient/control ratio of 2.29, combined with a prothrombin time (PT) of 28 seconds. An investigation of the coagulation factors showed a combined FV and FVIII deficiency of 29% and 21% respectively. The bleeding risk was considered to be high. A multidisciplinary approach permitted a specific and individualized FVIII substitution protocol. At 39 weeks of amenorrhea, the patient was admitted to the labor room. An infusion of 2000IU of FVIII was implemented over 5 minutes; soon thereafter, PT was 17 seconds, aPTT patient/control ratio had decreased to 1.9 and FV and FVIII reached 38% and 36% respectively. Six hours later, the patient delivered an infant weighing 2850g who had an Apgar score of 10. No bleeding was detected. The patient was then closely monitored for 2 hours in the recovery room. Twelve hours after administration of the first dose of FVIII, another infusion of 2000IU of FVIII was administered. This substitution treatment was continued every 12 hours in ever-decreasing doses, allowing maintenance of FVIII level >50% for 5days. At D7 post-partum, the patient was discharged uneventfully.