PB0215 - Assessment of global hemostatic assay by ROTEM in 63 patients with congenital dysfibrinogenemia

Sunday, June 25, 2023

18:30 – 19:30 ET

Room: Exhibition

Presenting Author(s)

Miroslava Drotarova, MD

PhD. student
National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, Slovakia
Martin, Zilina, Slovakia

Co-Author(s)

Tomas Simurda, MD, PhD.

physician
National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, Slovakia
Martin, Zilina, Slovakia
ZK

Zuzana Kolkova

Laboratory diagnostician
Biomedical Center Martin, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin
Martin, Zilina, Slovakia
DL

Dusan Loderer

Laboratory diagnostician
Biomedical Center Martin, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin
Martin, Zilina, Slovakia
PK

Peter Kubisz

university professor
National Center of Hemostasis and Thrombosis, Department of Hematology and Transfusion Medicine, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, Martin, Slovakia
Martin, Zilina, Slovakia
Jan Stasko, MD, PhD (he/him/his)

university professor
National Center of Hemostasis and Thrombosis, Department of Hematology and Transfusion Medicine, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, Martin, Slovakia
Martin, Zilina, Slovakia
Monika Brunclikova, MD

physician
National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, Slovakia
Martin, Zilina, Slovakia
Jana Zolkova, DSc, PhD (she/her/hers)

laboratory researcher
National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, Slovakia
Martin, Zilina, Slovakia
Ingrid Skornova, Ing.

Laboratory diagnostician
National Center of Hemostasis and Thrombosis, Department of Hematology and Transfusion Medicine, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, Martin, Slovakia
Martin, Zilina, Slovakia
Rita Marchi

PhD
UNIGE
Geneva, Geneve, Switzerland
Alessandro Casini, MD

MD
Division of Angiology and Hemostasis, Faculty of Medicine, Geneva University Hospitals, Geneva, Switzerland
Geneva, Geneve, Switzerland

Background: Rotational thromboelastometry (ROTEM) is a global hemostasis assay increasingly used in the perioperative and emergency assessment of bleeding diathesis. The diagnosis and predictive values of ROTEM in congenital dysfibrinogenemia remains to be established.

Aims: The aim of this study was to analyze clot formation by ROTEM in a dysfibrinogenemic cohort and to establish correlations with clinical features, coagulation parameters at diagnosis.

Methods: The clinical phenotype was assesed by the ISTH bleeding assessment tool (BAT). Coagulation parameters were measured by standard hemostasis asssays, fibrinogen activity and antigen. EXTEM, INTEM, FIBTEM tests were used to measure selected ROTEM parameters, including clotting time (CT), clot formation time (CFT), maximal clot firmness (MCF) and amplitude 10 min after CT (A10). The study included genetically confirmed congenital dysfibrinogenemia (n=63) and healthy controls (n=50).

Results: Bleeding phenotype with at least one bleeding symptoms was observed in 36.5 % of patients. The median BAT was 1 (range 0-5). A10 in FIBTEM was statistically significantly lower than control. MCF in FIBTEM, EXTEM and INTEM were similar to controls. CT in INTEM was statistically significantly shorter than controls and the CFT in EXTEM was prolonged. Fibrinogen activity was positively correlated with fibrinogen antigen, A10 in FIBTEM, A10 and MCF in FIBTEM, EXTEM and INTEM.

Conclusion(s): We observed that in ouh cohort the ROTEM seems not able to distinguish dysfunctional fibrinogen from controls. Additional studies based in genotype are needed to evaluate the role of ROTEM in such patients.