A rare bleeding disorder with severe haemorrhagic manifestation

Abstract

Factor V is a major part of the coagulation system and maintains a balance in pro-coagulant and anticoagulant pathways. Any deficiency or mutation can lead to disequilibrium in coagulation. Factor V deficiency can be congenital or acquired. Congenital factor V deficiency (parahemophilia or Owren’s disease) is a rare autosomal recessive condition. We present a case of an adolescent unmarried girl presenting with an acute abdomen and deranged coagulation parameters. It was confirmed that the bilateral haemorrhagic ovarian cysts and hemoperitoneum were due to factor V deficiency. In her case, the bleeding was likely due to a ruptured corpus luteum during ovulation. She was successfully managed conservatively with red cells and fresh frozen plasma transfusions, tranexamic acid and combined oral contraceptive pills (COCP) with instructions to continue the use of COCP to prevent such bleeding manifestations.

Keywords

• Congenital factor V deficiency
• Ruptured corpus luteum
• Hemoperitoneum
• Acute abdomen