Abstract Title: A Rare Find: Diagnosis and Prophylactic Treatment of Factor VII Deficiency in a Pregnant Female Undergoing Cesarian Section

Background: Factor VII deficiency is a rare inherited autosomal recessive bleeding disorder which can vary greatly in its presentation and management. Factor VII plays a vital role in secondary hemostasis through its interaction with tissue factor in the extrinsic coagulation pathway activated in times of vascular injury. Phenotype can range from patients being asymptomatic to developing life-threatening bleeding involving the brain or gastrointestinal tract. Diagnosis is suspected in a bleeding patient with an isolated prolongation of the prothrombin time and confirmed with a serum factor VII level. Treatment consists of recombinant factor VIIa replacement therapy with the number of recommended doses dependent on the severity of bleeding.

Case Presentation: Our patient is a 32-year-old Hispanic female who presented at 37 weeks pregnancy for planned Cesarean and further evaluation of abnormal coagulation studies noted at an outside facility. She endorsed symptoms of easy bruising, bleeding gums with oral hygiene, occasional nosebleeds and an extensive history of heavy menstrual bleeding.  She had previously been hospitalized for blood transfusion five years earlier for heavy menstrual bleeding lasting one month.  Family history was significant for a sister who had recently been diagnosed with a coagulopathy though patient was unsure of her sister's diagnosis.   Lab work showed a prothrombin time (PT) greater than 169 seconds, INR >15, PTT 25.7 seconds and complete blood count demonstrated hematocrit 35% and platelet count of 145,000.  She was given one dose of oral vitamin K 10 mg without benefit.  Factor VII level was found to be less than 0.4 and PT correction was noted with one-to-one mix, confirming a diagnosis of factor VII deficiency. Initial dose of recombinant VIIa 20 mcg/kg was given before her planned C-section with the next dose at 15 mcg/kg 4 hours post-operatively and every 6 hours thereafter for a total of 5 doses in a 24-hour period. She underwent cesarean section without significant bleeding and her PT and INR normalized with the therapy outlined above. She was discharged from the hospital and at 1 month follow-up had no further episodes of bleeding.

Discussion: Our patient received factor VII replacement therapy and did not experience excessive bleeding with Cesarean section. This case demonstrates the phenotypic variation in the potential bleeding associated with Factor VII deficiency, with one-third of patients being asymptomatic during their life. Factor VII deficiency remains a disease to keep on one’s differential for an isolated elevation in PT and INR with a significant personal or family history of bleeding.