Behçet’s Disease, Associated Large Vessel Thrombosis, and Coexistent Thrombophilia: A Distinct Nosological Entity?

Table 6

Prevalence of the procoagulant factors in the general population, in patients with BD, and in patients with BD and thrombosis.

Thrombophilic factors	Prevalence in the general population [5–7]	Prevalences of each thrombophilic factor in studies from literature review [8–16]
		All patients with BD	Patients with BD and thrombosis	Controls patients
FV leiden mutation	5% in Caucasians; (range: 0–15%); Rare or absent in black African and Far East Asian people.	Gül et al. [8]; 23% (15/64)^†,; Turkish patients	Gül et al. [8]; 37.5% (12/32)^†; Turkish patients	Gül et al. [8]; 10% (11/107)^†; Turkish patients
		≠	≠	≠
		Silingardi et al. [10]; 4.5% (8/118)^†; Italian patients	Silingardi et al. [10]; 7.4% (2/27)^†; Italian patients	Silingardi et al. [10]; 3.8% (5/132)^†; Italian patients
FII G20210A mutation	2-3%; Seen only in Caucasians.	Gül et al. [9]; 17% (11/64)^†; Turkish patients	Gül et al. [9]; 31.3% (10/32)^†; Turkish patients	No data
		≠	≠
		Silingardi et al. [10]; 5.8% (9/118)^†; Italian patients	Silingardi et al. [10]; 3.7% (1/27)^†; Italian patients	Silingardi et al. [10]; 3.8% (5/132)^†; Italian patients
MTHFR; polymorphisms	About 10% are homozygous carriers of the variant C677T.	Karakus et al. [11]: frequency of heterozygosity of C677T was nearly similar between two groups; the homozygosity of C677T was significantly higher in BD patients than healthy controls (P = 0.004; OR 5.05, 95% CI: 1.49–17.11).; Study group: 318 patients with BD and 207 healthy controls of Turkish origin.; ≠; Ricart et al. [12]: there was no difference between patients and controls in the prevalence of C677T polymorphism.; Study group: 79 patients with BD (23 with thrombosis and 56 withoutthrombosis) and 84 healthy control patients in eastern Spain.
Hyperhomocysteinemia; (>15 μmol/L)	Levels of homocysteine over 18 μmol/L are associated with an increased risk of thrombosis. Such levels are found in 5%–10%.	Shahram et al. [13]; 14.9 ± 13.9 μMol/L (SD)^‡; 96 Iranian patients	Shahram et al. [13]; 24.2 ± 13.2 μMol/L (SD)^‡; 49 Iranian patients	Shahram et al. [13]; 9.9 ± 6.7 μMol/L (SD)^‡; 49 Iranian patients
			≠
		No data	Leiba et al. [14]; 12.6 ± 3.9 μMol/L (SD)^‡; 33 Israeli patients	No data
Antiphospholipid; antibodies	1–5%; Usually detectable; in low titres.	Tokay et al. [15]: the frequency of IgG and IgM anticardiolipin antibodies (aCL) was 2.4% in BD, 50% in systemic lupus erythematosus (SLE), and 5.6% in healthy controls. No association was found between aCL titres and vascular involvement in BD.; Study group: 128 patients with BD, 20 patients with SLE, and 143 healthy control patients of Turkish origin.; ≠; Hull et al. [16]: statistically significant presence of aCL was found in 13/70 patients with BD and was connected with vascular pathology (8/13).; Study group: 70 patients with BD. Forty originated from Italy, 19 from the United Kingdom, 10 from Middle Eastern countries, and one from the West Indies.

†Percentage and number of the patients respectively, ^‡(SD): standard deviation.