title: Transfusion management of factor V deficiency: three case reports and review of the literature.
abstract: Factor V (FV) deficiency may be inherited as an autosomal recessive disease or acquired as a result of autoantibody formation, either spontaneously or secondary to exposure to bovine thrombin or medications. Congenital FV deficiency has traditionally been treated with plasma transfusions. However, recent evidence has suggested that platelet (PLT) transfusions may be a better alternative as FV stored within PLT alpha granules has greater procoagulant potential and is released locally at sites of vascular injury. We report three cases of FV deficiency, one congenital and two acquired, and emphasize the different management approaches.
  
  
  
  Patient 1 was a 30-year-old man with congenital FV deficiency who presented with a trauma-induced hematoma of his lower extremity. He was treated with 5 PLT units over 48 hours. Patient 2 was a 64-year-old woman who presented with an upper-extremity thrombus and was discovered to have a FV inhibitor, likely secondary to antibiotics. Patient 3 was a 75-year-old woman with hepatitis C virus (HCV) who presented with minor ecchymosis and was found to have a FV inhibitor secondary to either HCV or antibiotic exposure. Corticosteroids alone were able to eradicate the inhibitors in both patients with acquired inhibitors.
  
  
  
  FV deficiency can present with a diverse range of symptoms. For bleeding patients, PLT transfusions should be the initial therapy. In patients with thrombosis, the risks and benefits of anticoagulation must be carefully assessed before treatment. For patients with minor bleeds, transfusions may be withheld, and elimination of the inhibitor should be the primary objective.
authors.affiliation: Division of Transfusion Medicine and Hemostasis, Department of Pathology, UT Southwestern Medical Center, Dallas, Texas.
authors.forename: Chakri
authors.initials: C
authors.lastname: Gavva
authors.affiliation: Division of Transfusion Medicine and Hemostasis, Department of Pathology, UT Southwestern Medical Center, Dallas, Texas.
authors.forename: Sean G
authors.initials: SG
authors.lastname: Yates
authors.affiliation: Division of Hematology, Department of Internal Medicine, UT Southwestern Medical Center, Dallas, Texas.
authors.forename: Siayareh
authors.initials: S
authors.lastname: Rambally
authors.affiliation: Division of Transfusion Medicine and Hemostasis, Department of Pathology, UT Southwestern Medical Center, Dallas, Texas.
authors.forename: Ravi
authors.initials: R
authors.lastname: Sarode
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood; D019468:Disease Management; D005165:Factor V / Q000037:antagonists & inhibitors / Q000627:therapeutic use; D005166:Factor V Deficiency / Q000150:complications / Q000209:etiology / Q000628:therapy*; D005260:Female; D006470:Hemorrhage / Q000209:etiology / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D017713:Platelet Transfusion; D013927:Thrombosis / Q000188:drug therapy / Q000209:etiology
chemical_list: D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D005165:Factor V