Suggested abnormalities in clotting, coagulation, and fibrinolysis in patients with Behçet's disease (BD) with thrombotic complications—comparison with other patients with BD and with controls. (Only significant data are represented)
| Analysed factor | Prevalence among control patients | Prevalence among all patients with BD | Prevalence among patients with thrombosis | Prevalence among patients without thrombosis | Reference | Comments | |
| Anticoagulant factors | |||||||
| Leiden factor (% (No)) | 10 (11/107) | 23 (15/64) | 37.5 (12/32) | 9 (3/32) | Gulet al (4) | Results revalidated in few clinical trials | |
| APCR1-152 (% (No)) | 5 (16/320) | 29 (17/58) | 67 (6/9) | 22 (11/49) | Kosar et al (19) | Another study confirmed these results. | |
| Thrombomodulin (mean values) (ng/ml) | 26.7 | 35 | Espinosa et al (32) | Increased plasma concentration in patients. May point to decreased concentration on endothelial cells and decreased function | |||
| Procoagulant factors | |||||||
| Homocysteine (% (No)) | 9 (4/43) | 28 (12/43) | Altinbaset al (50) | No significant difference between patients groups | |||
| Prothrombin mutation (PT20210) (% (No)) | 31 (10/32) | 3 (1/32) | Gul et al (56) | Comparison between patients. No controls | |||
| Anticardiolipin antibody (% (No)) | 5 (1/20 patients with RA) | 40 (10/25) | Mader et al(29) | Most studies confirmed these results, but specific antibody assays were not significant50 | |||
| Fibrinolytic pathway | |||||||
| Lp(a)1-152 (mean value) (g/l) | 0.18 | 0.3 | 0.52 | 0.21 | Orem et al (25) | Same study shows other fibrinolytic anomalies: increased PAI-1 and decreased tPA in patients with Behçet's disease | |
| (normal = 3.1 g/l) | 30 Pt1-152 | 33 Pt | 5 Pt | 28 | |||
| tPA1-152 (mean (SD) value) (ng/ml) | 5.8 | 4.21-150 | 4.781-150 | 4.6 | (25) | ||
| 30 Pt | 33 Pt | 5 Pt | 28 Pt | ||||
| 5.25 (1.84) | 7.72 (3.52) | 8.16 (2.89) | 7.57 (3.81) | Demirer et al(24) | |||
| 24 Pt | 127 Pt | 34 | 93 Pt | ||||
| PAI-11-151(mean value) (ng/ml) | 58.11-151 | 63.81-151 | 83 | 59.4 | (25) | ||
| 24 Pt | 33 Pt | 5 Pt | 28 Pt | ||||
| Endothelial injury | |||||||
| Mean (SD) vWF1-152 (50%–160% scale) (%) | 88.2 (30.7) | 108.9 (40.2) | 121.8 (54.8) | 104.3 (32.4) | (24) | Same patient group showed increased vWF and tPA and decreased thrombomodulin indicators of endothelial damage | |
↵1-150 Differences between patients with BD with and without thrombosis were not significant.
↵1-151 Differences between patients with BD and controls were not significant.
↵1-152 APCR = activated protein C resistance; Lp(a) = lipoprotein (a); tPA = tissue plasminogen activator; PAI-1 = plasminogen activator inhibitor; vWF = von Willebrand factor; Pt = patients.