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[凝血障碍的诊断与治疗]

[Diagnosis and treatment of coagulation disorders].

作者信息

Esnaola-Rojas M M

机构信息

Centro Neurológico de Tratamiento y Rehabilitación, Hospital Francés, Buenos Aires, Argentina.

出版信息

Rev Neurol. 1999;29(12):1290-300.

Abstract

INTRODUCTION

Approximately 5% of all cerebrovascular events (CVE), and 10% of those occurring in young patients, are due to hematological disorders. Hypercoagulability states are related to CVE in young patients, deep vein thrombosis, recurrent thromboses, pulmonary embolism, a family history of thrombosis and unusual venous and arterial thromboses.

DEVELOPMENT

The conditions related to increased risk of thrombosis are: the congenital thrombophilias due to deficiency of protein C, protein S or antithrombin III, resistance to protein C activated by Leiden's factor V--cofactor of protein C with genetic mutation--; the primary antiphospholipid syndrome with anticardiolipin antibodies and lupus inhibitor; platelet disorders, deficit of heparin cofactor II, deficit of plasminogen and plasminogen tissue activator (t-PA) and increase in the inhibitor of plasminogen tissue activator (PAI-I); alterations in factors of coagulation such as deficits of factor VII and factor XIII, mutation of prothrombin 20210-->A, increase in factor VIII. Hyperfibrinogenemia and hyperhomocysteinemia are also independent risk factors for CVE.

CONCLUSION

The patients, especially young patients, with recurrent thrombosis or thrombosis of unknown origin should be assessed seeking clinical and serological signs of the primary antiphospholipid syndrome or other coagulopathies. Although we still have no results of controlled prospective studies regarding these conditions, long term anticoagulation is recommended on the findings of small-scale retrospective studies.

摘要

引言

在所有脑血管事件(CVE)中,约5%以及在年轻患者中发生的CVE的10%是由血液系统疾病引起的。高凝状态与年轻患者的CVE、深静脉血栓形成、复发性血栓形成、肺栓塞、血栓形成家族史以及不寻常的静脉和动脉血栓形成有关。

进展

与血栓形成风险增加相关的情况有:因蛋白C、蛋白S或抗凝血酶III缺乏导致的先天性血栓形成倾向,对由莱顿因子V激活的蛋白C产生抵抗——蛋白C的辅因子且存在基因突变——;伴有抗心磷脂抗体和狼疮抗凝物的原发性抗磷脂综合征;血小板疾病、肝素辅因子II缺乏、纤溶酶原和纤溶酶原组织激活剂(t-PA)缺乏以及纤溶酶原组织激活剂抑制剂(PAI-I)增加;凝血因子改变,如因子VII和因子XIII缺乏、凝血酶原20210→A突变、因子VIII增加。高纤维蛋白原血症和高同型半胱氨酸血症也是CVE的独立危险因素。

结论

对于患有复发性血栓形成或不明原因血栓形成的患者,尤其是年轻患者,应评估其是否存在原发性抗磷脂综合征或其他凝血病的临床和血清学迹象。尽管我们仍没有关于这些情况的对照前瞻性研究结果,但根据小规模回顾性研究结果,建议进行长期抗凝治疗。

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