Successful perioperative management using prothrombin complex concentrates in patients with severe congenital protein C deficiency

Pediatr Blood Cancer. 2022 Jan;69(1):e29380. doi: 10.1002/pbc.29380. Epub 2021 Oct 19.

Abstract

Perioperative management of severe congenital protein C deficiency remains unestablished. This deficiency is often treated with anticoagulants, such as warfarin. Although anticoagulants need to be perioperatively discontinued, there are few methods for the management of such patients. We adopted a method for administering prothrombin complex concentrates (PCC), which includes intermittent administration of inactive protein C (PPSB-HT), and examined its outcome as a perioperative management approach for severe congenital protein C deficiency. Three patients underwent our perioperative management six times. We monitored activity levels of protein C, factor IX, and so forth. These patients could be perioperatively managed with PCC treatment.

Keywords: congenital protein C deficiency; perioperative management; prothrombin complex concentrates (PPSB-HT).

MeSH terms

  • Anticoagulants
  • Blood Coagulation Factors
  • Humans
  • Protein C
  • Protein C Deficiency* / drug therapy
  • Prothrombin

Substances

  • Anticoagulants
  • Blood Coagulation Factors
  • Protein C
  • prothrombin complex concentrates
  • Prothrombin