Abstract Number: PB0406
Meeting: ISTH 2021 Congress
Theme: Fibrinogen, Fibrinolysis and Proteolysis » Fibrinogen and Factor XIII
Background: The ISTH bleeding assessment tool (ISTH-BAT) was developed to aid the evaluation of suspected patients for bleeding disorders.
Aims: To assess the utility of ISTH-BAT in diagnosis, determining the bleeding severity in congenital fibrinogen disorders (CFDs) and diagnosing them according to the new ISTH classification.
Methods: A total of 70 (40 female, 30 male) Iranian patients with CFDs and 31 adult healthy controls (15 female, 16 male) were selected. Routine coagulation laboratory tests such as prothrombin time (PT), activated partial thromboplastin time (APTT), and thrombin time (TT) were performed. The fibrinogen antigen level (Fg:Ag) and its functional activity were measured using an immunoturbidimetric assay and the Clauss method (Fg:C), respectively. The ratio of Fg:C/Fg:Ag was calculated and ISTH-BAT was used to assess the bleeding score (BS) of patients and healthy controls.
Results: Laboratory and clinical parameters are summarized in table 1. Patients were identified according to the new classification: 1A (afibrinogenemia, n=37), 2B (moderate hypofibrinogenemia, n=6), 2C (mild hypofibrinogenemia, n=2), 3A (dysfibrinogenemia, n=19), 4B (moderate hypodysfibrinogenemia, n=2), 4C (mild hypodysfibrinogenemia, n=4). Sixty-seven (95.7%) patients experienced bleeding events and only 3 (4.3%) patients with dysfibrinogenemia were asymptomatic. Each group of patients showed a statistically higher median BS than healthy controls (Fig. 1). Overall median BS of all patients was 4 (range 0-9) and was statistically significantly higher than healthy controls (median 1, range 0-6, p <0.002).
The most common clinical symptoms in patients with CFDs were cutaneous bleeding (56%), menorrhagia (39%), and umbilical cord bleeding (29%). Umbilical cord bleeding was observed only in afibrinogenemia and one afibrinogenemia patient experienced eye bleeding. Miscarriage was reported in three patients (two dysfibrinogenemia, one hypodysfibrinogenemia), and two women (afibrinogenemia) had an ovarian cyst rupture.
| Classification of disorders | N (%) | Age | Sex (M/F) | Fg:C (mg/dl) | Fg:Ag (mg/dl) | PT (s) | PTT (s) | TT (s) | BS |
| Afibrinogenemia | 37 (53%) | 13 (1-39) | 20/17 | Undetectable | Undetectable | >60 | >120 | >60 | 5 (2-9) |
| Hypofibrinogenemia | 8 (11%) | 23 (2-34) | 3/5 | 89 (75-117) | 110 (73-134) | 17 (11-19) | 38 (31-47) | 23 (23-29) | 4.5 (1-6) |
| Dysfibrinogenemia | 19 (27%) | 34 (4-63) | 5/14 | 50 (25-78) | 312 (228-370) | 15 (12-20) | 36 (32-47) | 35 (19-46) | 3 (0-5) |
| Hypodysfibrinogenemia | 6 (9%) | 27 (8-61) | 2/4 | 43 (40-90) | 125 (76-156) | 14 (11-20) | 36 (30-42) | 25 (17-39) | 4 (2-7) |
| Normal range | – | – | – | 154-475 | 184-334 | 10-13 | 18-28 | 14-19 | male ≤3 female ≤5 children≤ 2 |
Laboratory and clinical characteristics of patients (median range)
Conclusions: This study showed that ISTH-BAT is a useful tool for the diagnosis of CFDs and identifies those patients who need further investigations.
To cite this abstract in AMA style:
Mohsenian S, Seidizadeh O, Azarkeivan A, Jazebi M, SM. Application of the ISTH-BAT In Congenital Fibrinogen Disorders [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/application-of-the-isth-bat-in-congenital-fibrinogen-disorders/. Accessed December 8, 2021.« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/application-of-the-isth-bat-in-congenital-fibrinogen-disorders/