PB0712 - The utility of bleeding assessment tools in fibrinogen deficiencies

Monday, July 11, 2022

6:30 PM – 7:30 PM

Location: Exhibition

Presentation Format: In-Person

Presenting Author(s)

Samin Mohsenian

Master of Hematology and blood banking
Angelo Bianchi Bonomi Hemophilia and Thrombosis Center .
Milan, Lombardia, Italy

Co-Author(s)

Omid Seidizadeh, MSc, Phd student

Phd Student
Fondazione IRCCS Ca’Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy
Milan, Lombardia, Italy
RP

Roberta Palla

Biologist
Università degli Studi di Milano, Department of Pathophysiology and Transplantation, Milan, Italy
MILAN, Lombardia, Italy
MJ

Mohammad Jazebi

Senior laboratory scientist
Iranian Comprehensive Hemophilia Care Center
Tehran, Tehran, Iran
AA

Azita Azarkeivan

Professor of Pediatric Hematology and Oncology
Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine
Tehran, Tehran, Iran
SM

Samira Moazezi

Medical laboratory technician
Iranian Comprehensive Hemophilia Care Center
Tehran, Tehran, Iran
Mb

Mohammad Reza baghaipour

MD
Iranian Comprehensive Hemophilia Care Center
Tehran, Tehran, Iran
Marzia menegatti, PhD

Biologist
Fondazione IRCCS Ca’Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa, Milan, Italy
MILAN, Lombardia, Italy
Flora Peyvandi, MD, PhD

Professor of Internal Medicine; Director of the Angelo Bianchi Bonomi Hemophilia & Thrombosis Center
Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy; Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy
Milan, Lombardia, Italy

Background: Bleeding-assessment-tools (BAT) or bleeding-score-systems (BSS) are developed to address the challenges associated with evaluation of patients. A small number of patients with congenital fibrinogen deficiencies (CFD) are evaluated with these tools without definitive results.

Aims: To compare the results of the ISTH-BAT with the EN-RBD-BSS in patients with CFD. We further evaluated the correlation between bleeding scores (BS) and fibrinogen levels.

Methods: In total, 100 (55% females) Iranian patients with CFD were included. Routine coagulation and fibrinogen-specific tests including antigen (Fg:Ag) and its functional activity (Fg:C) were performed. Patients were classified using Fg:C/Fg:Ag ratio and they were divided into four categories of bleeding severity (asymptomatic, grade I, grade II, grade III). The ISTH-BAT and the EN-RBD-BSS were used to assess the BS of all patients. Pearson’s correlation was performed using IBM-SPSS Statistics 26.

Results: Patients’ characteristics are summarized in Table 1. The median (range) of the ISTH-BAT and EN-RBD-BSS were 4 (0-16) and 2.21 (-1.49-6.71), respectively. In general, a moderate positive correlation (r=0.597, P< 0.001) was found between the ISTH-BAT score and EN-RBD-BSS(Figure 1-A). The correlation between Fg:Ag and the ISTH-BAT scores was weak negative (r= -0.410, P< 0.001), comparable to the correlation between Fg:C and ISTH-BAT scores (r= -0.419, P< 0.001). There was a weak negative correlation between Fg:Ag and EN-RBD-BSS (r= -0.460, P< 0.001), and between Fg:C and EN-RBD-BSS scores (r= -0.405, P< 0.001, Figure 1-B). Hypodysfibrinogenemic patients had the strongest correlation between the EN-RBD-BSS and ISTH-BAT (r= -0.916, P=0.001). Excluding dysfibrinogenemic patients, there was a significant association between bleeding severity and Fg:C (r= -0.399, P< 0.001), and Fg:Ag (r= -0.322, P= 0.005).

Conclusion(s): Based on the good correlation between the two systems, it appears that in addition to the ISTH-BAT score, the EN-RBD-BSS could also be useful in CFD patients. The BS showed a significant negative correlation with fibrinogen antigen/activity levels.