Glueck C J, McMahon R E, Bouquot J, Stroop D, Tracy T, Wang P, Rabinovich B
Cholesterol Center, Jewish Hospital, Cincinnati, Ohio, USA.
Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1996 May;81(5):557-66. doi: 10.1016/s1079-2104(96)80047-3.
Our specific aim in 49 patients (42 women, 7 men) with osteonecrosis of the jaw was to determine whether thrombophilia (increased tendency to intravascular thrombosis) or hypofibrinolysis (reduced ability to lyse thrombi) were associated with this regional avascular necrosis.
Determinants of thrombosis and fibrinolysis were compared in healthy controls and in 42 women and 7 men who had biopsy-proven idiopathic osteonecrosis of the jaw with severe chronic jaw or facial pain syndromes and failure to respond to conventional medical and dental treatments.
Of the 49 patients, 35 (71%) had thrombophilia or hypofibrinolysis and only 14 were normal. Thrombophilia as a sole coagulation defect was found in 10 patients, 7 with resistance to activated protein C and 3 with low protein C (deficiency of an antithrombotic protein). Hypofibrinolysis with low stimulated tissue plasminogen activator activity and high lipoprotein (a) (an atherogenic, hypofibrinolytic lipoprotein) were found as sole coagulation defects in seven and eight patients, respectively. Ten patients had mixed defects; 7 of these 10 had thrombophilia with resistance to activated protein C. Sinusoidal dilatation was a constant feature in maxillary and mandibular bone biopsies, suggesting venous occlusion with intramedullary hypertension. Marrow fibrosis and occasional fibrin plugs were additional microscopic features believed to impair venous drainage and to contribute to ischemic necrosis of the alveolar bone.
Primary thrombophilia and hypofibrinolysis appear to be common, heritable, pathophysiologic risk factors for idiopathic osteonecrosis of the jaws. These coagulation defects may also contribute to alveolar neuralgia, atypical odontalgia and facial neuralgia, idiopathic trigeminal neuralgia, and to treatment failures so often encountered in patients with alveolar osteonecrosis and disabling chronic facial and jawbone pain syndromes.
我们对49例颌骨骨坏死患者（42例女性，7例男性）进行研究的具体目的是确定血栓形成倾向（血管内血栓形成倾向增加）或纤维蛋白溶解功能减退（溶解血栓能力降低）是否与该区域的缺血性坏死相关。
比较健康对照组以及42例女性和7例男性的血栓形成和纤维蛋白溶解的决定因素，这些患者经活检证实患有特发性颌骨骨坏死，伴有严重的慢性颌面部疼痛综合征，且对传统医学和牙科治疗无反应。
49例患者中，35例（71%）有血栓形成倾向或纤维蛋白溶解功能减退，只有14例正常。仅10例患者存在血栓形成倾向这一单一凝血缺陷，其中7例对活化蛋白C有抵抗，3例蛋白C水平低（一种抗血栓蛋白缺乏）。分别有7例和8例患者仅存在纤维蛋白溶解功能减退这一单一凝血缺陷，表现为刺激的组织纤溶酶原激活物活性低和脂蛋白(a)水平高（一种致动脉粥样硬化、纤维蛋白溶解功能减退的脂蛋白）。10例患者有混合缺陷；这10例中的7例有对活化蛋白C抵抗的血栓形成倾向。窦状隙扩张是上颌骨和下颌骨活检中的一个常见特征，提示静脉阻塞伴髓内高压。骨髓纤维化和偶尔出现的纤维蛋白栓是另外的微观特征，被认为会损害静脉引流并导致牙槽骨缺血性坏死。
原发性血栓形成倾向和纤维蛋白溶解功能减退似乎是特发性颌骨骨坏死常见的、可遗传的病理生理风险因素。这些凝血缺陷也可能导致牙槽神经痛、非典型牙痛和面部神经痛、特发性三叉神经痛，以及牙槽骨坏死患者和致残性慢性面部和颌骨疼痛综合征患者中经常遇到的治疗失败情况。
