Department of Laboratory Medicine, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, No.197 Ruijin Second Road, Shanghai, 200025, China.
Faculty of Medical Laboratory Science, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, No.197 Ruijin Second Road, Shanghai, 200025, China.
BMC Med Genet. 2018 Mar 27;19(1):50. doi: 10.1186/s12881-018-0557-1.
The contribution of moderate coagulation factor XII (FXII) deficiency to development of thromboembolism is still undetermined. We have tried to show the relevance of FXII deficiency to incidences of venous thrombosis by exploring the prevalence of F12 gene mutations in Chinese patients with thrombotic disorders.
One hundred and six patients with venous thromboembolism (VTE) and 220 healthy controls were enrolled in study. The coding region and flanking sequences of F12 gene were amplified and sequenced to identify genetic variances. Patients with F12 mutations were also screened for other thrombotic risk factors.
Heterozygous F12 gene mutations were identified in 6 individuals with VTE and 10 healthy controls. Q336X and R66W were found in two healthy individuals; D291E was identified in a patient with DVT; and A343P was a recurrent mutation with a prevalence of 4.7% (5/106) in patient group and 3.6%(8/220) in healthy control. The prevalence of heterozygous mutations between the two groups had no significant difference. The association of A343P mutations with VTE was weak with an OR of 1.31 (95% CI 0.42-4.11). No other thrombophilia risk factors screened were positive in patients harboring heterozygous F12 mutations.
There were conflicting theories about the relationship between FXII deficiency and thrombosis formation. Heterozygous F12 mutation decreases the plasma FXII activity approximately by half and cause moderate FXII deficiency. Although multiple mutations were identified in both groups, the link between F12 heterozygous mutation and development of thrombotic disorders is weak and further studies are warranted to clarify their relationship.
中度凝血因子 XII(FXII)缺乏症对血栓栓塞形成的影响仍不确定。我们试图通过探索中国血栓性疾病患者 FXII 基因缺失与静脉血栓形成发生率的相关性,来表明 FXII 缺乏症与静脉血栓形成的相关性。
研究纳入了 106 例静脉血栓栓塞症(VTE)患者和 220 例健康对照者。扩增并测序 F12 基因的编码区和侧翼序列,以确定遗传变异。还对存在 F12 突变的患者进行了其他血栓形成危险因素的筛查。
在 6 例 VTE 患者和 10 例健康对照者中发现了杂合性 F12 基因突变。在 2 例健康个体中发现了 Q336X 和 R66W 突变;D291E 突变见于 1 例 DVT 患者;A343P 是一种复发性突变,在患者组中的发生率为 4.7%(5/106),在健康对照组中的发生率为 3.6%(8/220)。两组之间杂合突变的发生率无显著差异。A343P 突变与 VTE 的相关性较弱,OR 值为 1.31(95%CI 0.42-4.11)。在携带杂合性 F12 突变的患者中,未发现其他血栓形成危险因素阳性。
FXII 缺乏症与血栓形成之间的关系存在矛盾的理论。杂合性 F12 突变使血浆 FXII 活性降低约一半,导致中度 FXII 缺乏症。尽管在两组中都发现了多种突变,但 F12 杂合突变与血栓性疾病的发生之间的联系较弱,需要进一步的研究来阐明它们之间的关系。