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Diagnostic utility of bleeding assessment tools in congenital fibrinogen deficiencies

Samin Mohsenian1, Omid Seidizadeh1, Roberta Palla1

  • 1Università degli Studi di Milano, Department of Pathophysiology and Transplantation, Milan, Italy.

Haemophilia : the Official Journal of the World Federation of Hemophilia
|March 10, 2023
PubMed

Insights

Two bleeding assessment tools, the ISTH-BAT and EN-RBD-BSS, effectively identify patients with congenital fibrinogen deficiencies (CFDs). Both tools demonstrate significant sensitivity in detecting fibrinogen deficiency and classifying bleeding severity.

Area of Science:

  • Hematology
  • Rare Diseases
  • Diagnostic Tools

Background:

  • Clinical history assessment is vital for laboratory test referrals.
  • Bleeding Assessment Tools (BATs) standardize clinical evaluation.
  • Congenital Fibrinogen Deficiencies (CFDs) require effective diagnostic tools.

Purpose of the Study:

  • To compare the diagnostic adequacy of the ISTH-BAT and EN-RBD-BSS for identifying CFD patients.
  • To analyze the correlation between BAT scores, fibrinogen levels, and clinical bleeding severity.

Main Methods:

  • Inclusion of 100 Iranian patients diagnosed with CFDs.
  • Performance of routine coagulation and fibrinogen-specific tests (Fg:Ag, Fg:C).
  • Assessment of bleeding scores using both the ISTH-BAT and EN-RBD-BSS.

Main Results:

  • A moderate, statistically significant correlation was observed between the ISTH-BAT and EN-RBD-BSS (r = .597, P < .001).
  • Both tools showed a negative correlation with fibrinogen activity (Fg:C), indicating higher scores with lower activity.
  • The ISTH-BAT and EN-RBD-BSS correctly identified 70% and 72% of patients with fibrinogen deficiencies, respectively.

Conclusions:

  • The EN-RBD-BSS, alongside the ISTH-BAT, shows utility in identifying patients with CFDs.
  • Both BATs exhibit significant sensitivity for detecting fibrinogen deficiency.
  • Bleeding severity was accurately classified in approximately two-thirds of patients by these tools.
Abstract

Keywords:
EN-RBD-BSSISTH-BATbleeding assessment toolsfibrinogenfibrinogen deficiencies

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