Treatment tailoring for factor V deficient patients and perioperative management using global hemostatic coagulation assays

Sarina Levy-Mendelovich, Assaf Arie Barg, Nurit Rosenberg, Einat Avishai, Jacob Luboshitz, Mudi Misgav, Gili Kenet, Tami Livnat

Research output: Contribution to journalArticlepeer-review

Abstract

Introduction: Congenital factor V deficiency (FVD) is a rare bleeding disorder with an estimated incidence of 1 in 1000,000 in the general population. Since the common coagulation tests do not correlate with the bleeding tendency there is an unmet need to predict FVD patients’ bleeding hazard prior to surgical interventions. Aim: To optimize treatment prior to surgical interventions, using global coagulation assays, thrombin generation (TG) and rotating thromboelastogram (ROTEM). Methods: Our cohort included 5 patients with FVD, 4 severe and one mild. Two of them underwent TG and ROTEM prior to surgical interventions, including ex vivo spiking assays using bypass agents and platelets spiking. Results: All five patients exhibited prolonged PT and PTT, non-dependent on their bleeding tendency. Patient 1, who demonstrated severe bleeding phenotype, underwent surgery treated by combination of APCC (FEIBA) and platelet transfusion. Therapy was guided by global tests (TG as well as ROTEM) results. During the pre and post-operative period neither excessive bleeding nor any thrombosis was noted. In contrast, TG and ROTEM analysis of patient 4 has lead us to perform the surgery without any blood products’ support. Indeed, the patient did not encounter any bleeding. Conclusion: Global coagulation assays may be useful ancillary tools guiding treatment decisions in FVD patients undergoing surgical procedures.

Original languageEnglish
Pages (from-to)5-10
Number of pages6
JournalBlood Cells, Molecules, and Diseases
Volume71
DOIs
StatePublished - Jul 2018

Keywords

  • FEIBA
  • FV deficiency
  • Global coagulation assays
  • ROTEM
  • Thrombin generation
  • rFVIIa

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