factor V deficiency (Owren parahemophilia)

Etiology

• inherited
• acquired inhibitor of factor V
  • post partum
  • treatment with a certain type of fibrin glue
  • surgery
  • autoimmune diseases
    • certain cancers

Genetics

• associated with defects in coagulation factor V

Clinical manifestations

• similar to hemophilia, except bleeding into joints is less common
• bleeding into the skin
• bleeding of the gums
• excessive bruising
• excessive menstrual bleeding
• epistaxis
• prolonged or excessive loss of blood with surgery or trauma
• umbilical stump bleeding

Laboratory

• factor V assay shows decreased activity
• thrombin time is normal
• partial thromboplastin time (PTT) is prolonged
• prothrombin time is prolonged
• bleeding time (slightly prolonged in some people)

Management

• fresh frozen plasma for bleeding or after surgery

More general terms

• coagulation factor deficiency

Database

• OMIM: https://mirror.omim.org/entry/227400