title: Thromboelastography as a Surrogate Marker of Perisurgical Hemostasis in Gaucher Disease.
abstract: Thromboelastography (TEG) has long been available for routine monitoring of perisurgical and postpartum hemostasis, especially at point of care. The purpose of this study is to retrospectively compare TEG parameters to concomitant standard clotting test results in an unselected cohort of patients with Gaucher disease to ascertain whether TEG values are specific and sensitive enough to substitute for classic coagulation tests for decision making. This remains a cogent concern because of high incidence of thrombocytopenia in patients with Gaucher disease. Thromboelastography values were compared to concomitant platelet counts, partial thromboplastin time, international normalization ratio, and plasma fibrinogen. Demographic characteristics were collected from patients' files. There were 22 patients with Gaucher disease (2 children; 12.5%) for whom there were 24 TEG results at the same time as classic coagulation test results and 30% performed platelet function tests. The current study shows linear and/or monotonic relationships between platelet counts and several TEG values that were significant over a range of platelet counts including severe thrombocytopenia. The fibrinogen component, correlating only with the rate of clot lysis, played a lesser role. Based on these preliminary results albeit in a small cohort with only 1 case of hemorrhage, there is putative support for the intention to treat patients with Gaucher disease based on TEG results using the same TEG protocol as for other patients undergoing comparable procedures in our institution.
authors.affiliation: Department of Anesthesiology, Perioperative Medicine & Pain Treatment, Shaare Zedek Medical Center, Jerusalem, Israel.
authors.forename: Alexander
authors.initials: A
authors.lastname: Ioscovich
authors.affiliation: Department of Anesthesiology, Perioperative Medicine & Pain Treatment, Shaare Zedek Medical Center, Jerusalem, Israel.
authors.forename: Dmitri
authors.initials: D
authors.lastname: Fadeev
authors.affiliation: National Hemophilia Center, Sheba Medical Center, Tel Hashomer affiliated with the Sackler Medical School of the Tel Aviv University, Tel Aviv, Israel.
authors.forename: Gili
authors.initials: G
authors.lastname: Kenet
authors.affiliation: Blood Bank, Shaare Zedek Medical Center, Jerusalem, Israel.
authors.forename: Mira
authors.initials: M
authors.lastname: Naamad
authors.affiliation: Department of Anesthesiology, Perioperative Medicine & Pain Treatment, Shaare Zedek Medical Center, Jerusalem, Israel.
authors.forename: Gal
authors.initials: G
authors.lastname: Schtrechman
authors.affiliation: Gaucher Clinic, Shaare Zedek Medical Center, Jerusalem, Israel.
authors.forename: Ari
authors.initials: A
authors.lastname: Zimran
authors.affiliation: Gaucher Clinic, Shaare Zedek Medical Center, Jerusalem, Israel elstein@szmc.org.il.
authors.forename: Deborah
authors.initials: D
authors.lastname: Elstein
mesh_terms: D015331:Cohort Studies; D005260:Female; D005776:Gaucher Disease / Q000097:blood*; D006487:Hemostasis; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D013916:Thrombelastography / Q000379:methods*; D013921:Thrombocytopenia / Q000097:blood*
keywords: Gaucher disease; coagulopathy; enzyme replacement therapy; surgery; thrombocytopenia; thromboelastography