Cornudella R, Puente F, Hortells J L, Gutiérrez M
Sangre (Barc). 1989 Apr;34(2):144-6.
The interrelationship between factor XII deficiency (Hageman trait) and thrombosis is well known. A case of moderate factor XII deficiency (activity, 30%) associated to deep vein thrombosis, which occurred in the popliteal region of the left lower limb after abdominal surgery, is reported. The deficit was found in 4 family members of the three generations studied, and all of them showed a close interrelationship between factor XII activity and kallikrein levels. Prolonged APTT was found in 3 of the 4 affected subjects. A multiallelic model is suggested to explain the genetic transmission of this impairment.
凝血因子XII缺乏症(哈格曼特征)与血栓形成之间的相互关系已为人熟知。本文报告了一例腹部手术后发生于左下肢腘窝区域的深静脉血栓形成伴中度凝血因子XII缺乏症(活性为30%)的病例。在所研究的三代家族中,有4名家族成员存在该缺陷,并且他们所有人的凝血因子XII活性与激肽释放酶水平之间都显示出密切的相互关系。4名受影响的受试者中有3名发现活化部分凝血活酶时间(APTT)延长。有人提出一个多等位基因模型来解释这种缺陷的遗传传递。