Abstract Number: PB0823
Meeting: ISTH 2020 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Basic
Background: Factors affecting joint condition in severe haemophilia (SH) are still on debate. Variability on fibrin polymerization capacity might theoretically influence the bleeding tendency and thus, the joint condition in SH but studies on this regards are lacking.
Aims: To evaluate the correspondence between fibrin polymerization capacity and joint condition in SH patients.
Methods: 17 SH patients (3 inhibitors, median age [p25-p75]= 43.9 [36.0-52.2] years old) were recruited. Joint condition was evaluated using the HEAD-US score. Level of fibrinogen (Fib) (Clauss) and fibTEM (rotational thromboelastometry) was determined. Fibrinogen ability to increase the clot strength was evaluated by the quotient R= Fib/fibTEM maximum clot firmness (MCFfibTEM).
Results: Median value of Fib was 324 [278-351] mg/dl, MCFfibTEM = 15 [11-16] mm and R= 24.6 [20.6-26.6] mg/dl/mm. The patient group was divided into 2 groups taking into account the value of the 25th percentile of each variable. Thus, patients with Fib ≤ 278 mg/dl were considered patients with low fibrinogen level, patients with MCFfibTEM ≤ 11 mm were considered patients with low platelet independent maximum clot strength and patients with a R ≤ 20.6 mg/dl/mm were considered good responders to fibrinogen. Based on this analysis, neither Fib nor MCFfibTEM influenced the joint condition of the patients. However, patients with poor response to fibrinogen (R > 20.6 mg/dl/mm) had statistically significant greater joint damage than the good responders to fibrinogen (R ≤ 20.6 mg/dl/mm) (Table 1). Poor responders to fibrinogen had frequently joint damage in ankles followed by elbows and knees being cartilage the most commonly affected joint compartment. Good responders to fibrinogen only presented milder joint alterations in elbows and ankles.
Conclusions: Fibrin polymerization capacity may be variable between patients with SH and might influence their joint condition. However, larger studies are required to confirm this finding.
| VARIABLE | Good responders to fibrinogen | Poor responders to fibrinogen | p-value | |
| N | 4 | 13 | – | |
| Age (years) | 30.0 ± 18.2 | 44.9 ± 7.7 | 0.112 | |
| Inhibitors (%) | 2 (50) | 1 (7) | 0.121 | |
| HEAD-US | REC | 0.8 ± 1.5 | 3.3 ± 0.8 | < 0.010 |
| HEAD-US | REB | 0.3 ± 0.5 | 1.2 ± 0.7 | 0.020 |
| HEAD-US | LKC | 0 | 2.3 ± 2.0 | 0.037 |
| HEAD-US | RAC | 0 | 3.0 ± 1.5 | 0.010 |
| HEAD-US | RAB | 0 | 1.2 ± 0.7 | 0.011 |
| HEAD-US | LAC | 1.0 ± 2.0 | 3.0 ± 1.7 | 0.035 |
[Table 1: Patients’ characteristics. N: number of patients; R: right; E: elbow; C: cartilage; B: bone; L: left; K: knee; A: ankle]
To cite this abstract in AMA style:
Fernández-Bello I, De La Corte-Rodríguez H, Acuña-Butta P, Álvarez-Román M, Martín-Salces M, Rivas-Pollmar I, García-Barcenilla S, Cebanu T, Butta N, Jiménez-Yuste V. Fibrin Polymerization Ability Is Variable in Patients with Severe Haemophilia and Might Influence their Joint Condition [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/fibrin-polymerization-ability-is-variable-in-patients-with-severe-haemophilia-and-might-influence-their-joint-condition/. Accessed November 29, 2023.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/fibrin-polymerization-ability-is-variable-in-patients-with-severe-haemophilia-and-might-influence-their-joint-condition/