Miller N, Hultin M B, Gounder M, Zarrabi M H
Am J Hematol. 1986 Feb;21(2):215-21. doi: 10.1002/ajh.2830210211.
We report on a newly diagnosed family with hereditary antithrombin III deficiency, with thromboembolic complications in the propositus. Both the propositus and his asymptomatic sister had decreased plasma levels of antithrombin III antigen and activity (28-52% of normal with good agreement between functional and immunologic assays). The propositus developed deep venous thrombosis, followed by massive pulmonary emboli despite heparin therapy and was treated with streptokinase and heparin with excellent results. Shortly thereafter, small bowel obstruction required surgical intervention, and antithrombin III concentrate, recently available in the United States as an investigational new drug (I.N.D.), was administered with no postoperative thrombotic complications. He was subsequently asymptomatic while on warfarin prophylaxis but twice developed venous thrombosis when he failed to take warfarin. The addition of danazol therapy led to a sustained rise in the antithrombin III level. Each of these therapeutic approaches is discussed and the literature reviewed with emphasis on the newer agents--streptokinase, antithrombin III concentrate, and danazol.
我们报告了一个新诊断出的遗传性抗凝血酶III缺乏症家族，先证者出现了血栓栓塞并发症。先证者及其无症状的妹妹血浆抗凝血酶III抗原和活性水平均降低（为正常水平的28 - 52%，功能检测和免疫检测结果高度一致）。先证者发生了深静脉血栓形成，尽管接受了肝素治疗，随后仍出现了大面积肺栓塞，接受链激酶和肝素治疗后效果良好。此后不久，小肠梗阻需要手术干预，当时美国刚作为研究性新药（IND）上市的抗凝血酶III浓缩物被用于治疗，术后未出现血栓并发症。在接受华法林预防治疗期间他随后无症状，但在未服用华法林时两次发生静脉血栓形成。达那唑治疗使抗凝血酶III水平持续升高。本文讨论了每种治疗方法，并回顾了相关文献，重点关注新型药物——链激酶、抗凝血酶III浓缩物和达那唑。
