Challenges in management of unusual acquired factor V deficiency: A case report

Medicine (Baltimore). 2019 Apr;98(17):e15259. doi: 10.1097/MD.0000000000015259.

Abstract

Rationale: Acquired inhibitors of coagulation are antibodies that either inhibit the activity or increase the clearance of a clotting factor. Acquired factor V deficiency is a rare coagulation disorder, and it can sometimes be life threatening.

Patient concerns: We describe a case of a 90-year-old Japanese male with acquired factor V deficiency. He was previously misdiagnosed with congenital factor V deficiency when he presented with hemoptysis and a negative factor V inhibitor test result at a different hospital 5 years earlier. Coagulopathy recurred with ecchymosis when he sustained a bruise after falling on a bush.

Diagnosis: Although the factor V inhibitor test result was negative and a mixing study suggested a deficiency pattern, we diagnosed the patient with acquired factor V deficiency on the basis of no history of bleeding diathesis, a lack of response to multiple fresh frozen plasma transfusion, and clinical response to corticosteroid therapy.

Interventions: Intravenous methylprednisolone was administered at 500 mg/day for 3 days, followed by oral prednisolone at 1 mg/kg/day.

Outcomes: Coagulation test results improved and symptoms resolved 2 weeks after corticosteroid administration.

Lessons: This case report suggests that clearance-facilitating antibodies exist without the presence of neutralizing inhibitors. When patients present with coagulation factor V deficiency in the absence of coagulation inhibitors, acquired factor V deficiency should also be considered.

Publication types

  • Case Reports

MeSH terms

  • Aged, 80 and over
  • Factor V Deficiency / diagnosis
  • Factor V Deficiency / drug therapy*
  • Glucocorticoids / therapeutic use*
  • Humans
  • Male
  • Methylprednisolone / therapeutic use*
  • Partial Thromboplastin Time

Substances

  • Glucocorticoids
  • Methylprednisolone