Abstract Number: PB0706
Meeting: ISTH 2021 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Rare Bleeding Disorders
Background: Routine coagulation screening tests are not useful in the diagnosis of Factor XIII (FXIII) deficiency. Though, clot solubility test can be used as a screening test, the test is time consuming and lacks sensitivity. Global viscoelastic test like Rotational thromboelastometry (ROTEM) is the only rapid point of care test to assess hyperfibrinolysis.
Aims: To determine the diagnostic performance of bleeding symptoms, ISTH bleeding assessment tool (BAT) and ROTEM parameters in the diagnosis of FXIII deficiency.
Methods: This was a retrospective study wherein patients with FXIII deficiency among patients referred with a suspected bleeding disorder from October 2016 – October 2020 were included. Eighty consecutive patients with no intrinsic abnormality were recruited as controls. Diagnosis was confirmed after a detailed coagulation workup.
Results: 34 patients with FXIII deficiency were identified during the study period. The median (IQR) of FXIII activity and antigen levels of patients were 5.9 (4.3-8) and 0 (0-2.5) respectively. All patients had significant BAT score with a median (IQR) of 8 (5-11). The mean maximum clot firmness (MCF) mm was significantly lower [48.5 ± 10.1 vs 63.2 ± 4.8; p=<0.0001], and Maximum Lysis (ML)% was significantly higher [20.7 ± 9.1 vs 8.3 ± 3.9; p=<0.0001] in patients than controls. FXIII deficiency patients with ML <15% had significantly higher Fibrinogen levels (mg/dL) compared to those with ML≥15% [Mean: 325 ± 83 vs 250.5 ± 71; p = 0.024] which could have contributed to the lower ML%. The sensitivity and specificity of different variables are shown in Figure. Presence of either increased lysis in ROTEM or umbilical stump bleeding had a sensitivity of 97.1%. When both umbilical stump bleeding and increased lysis was present, the specificity was 100%.
Conclusions: A combination of bleeding history, specifically umbilical stump bleeding and increased lysis in ROTEM can serve as a useful screening test for FXIII deficiency.
To cite this abstract in AMA style:
Geevar T, Dave RG, David V T, K C G, Vijayan R, Samuel A, Singh S, John Mammen J, Chandran Nair S. ROTEM in Combination with Bleeding History – Can it Serve as a Screening Test for Factor XIII Deficiency in Patients with Suspected Bleeding Disorder? [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/rotem-in-combination-with-bleeding-history-can-it-serve-as-a-screening-test-for-factor-xiii-deficiency-in-patients-with-suspected-bleeding-disorder/. Accessed March 22, 2024.« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/rotem-in-combination-with-bleeding-history-can-it-serve-as-a-screening-test-for-factor-xiii-deficiency-in-patients-with-suspected-bleeding-disorder/