Use of purified fibrinogen concentrate for dysfibrinogenemia and importance of laboratory fibrinogen activity measurement

Pediatr Blood Cancer. 2013 Mar;60(3):500-2. doi: 10.1002/pbc.24383. Epub 2012 Nov 5.

Abstract

We report a patient with dysfibrinogenemia treated with purified fibrinogen concentrate who had discrepant post-treatment laboratory values. The patient had mild bleeding symptoms and was diagnosed with dysfibrinogenemia based on fibrinogen activity of 51 mg/dl and antigen of 240 mg/dl. He was treated for an adenoidectomy with purified fibrinogen concentrate (RiaSTAP®) at a dose of 70 mg/kg. A discrepancy in post-treatment fibrinogen activity was observed between the hospital and reference laboratories. Investigation revealed differences in laboratory assay and calibration methods. Fibrinogen concentrate may be a treatment option for patients with dysfibrinogenemia, but accurate laboratory technique is critical for fibrinogen measurement.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Afibrinogenemia / drug therapy*
  • Fibrinogen / analysis*
  • Fibrinogen / metabolism
  • Fibrinogen / therapeutic use*
  • Hematologic Tests / standards*
  • Humans
  • Laboratories
  • Laboratories, Hospital
  • Male

Substances

  • Fibrinogen

Supplementary concepts

  • Dysfibrinogenemia, Congenital