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[抗凝血酶III缺乏所致家族性复发性静脉炎。附1例报告]

[Familial recurrent phlebitis due to deficiency of antithrombin III. Apropos of a case].

作者信息

Caillé J P, Sarrade-Loucheur C, Ferrer F, Vergoz D

出版信息

Phlebologie. 1980 Jan-Mar;33(1):131-7.

PMID:7375519
Abstract

The authors report the case of a young woman aged 29, mother of 2 children, presenting a post-phlebitic syndrome with hyperalgic capillaritic ulcer, a 10 year history of serious thrombophlebitic episodes with repeated pulmonary embolism despite heparin therapy, and reports of similar episodes in the family, some of which proved fatal. Haematological study showed a clear diminution in progressive antithrombin activity. A discussion follows on the clinical and haematological aspects of this constitutional and familial deficiency, which has been known since 1965 (Egelberg). The relative rarity of this condition should not prevent the routine search (by combined functional, immunological and chromogenic estimation) in the presence of repeated thrombosis, and when the thrombosis is biologically resistant to heparin : in such cases, the indication for long term treatment with vitamin K antagonists is undeniable.

摘要

作者报告了一名29岁的年轻女性病例,她是两个孩子的母亲,患有伴有痛性毛细血管炎溃疡的静脉炎后综合征,有10年严重血栓性静脉炎发作史,尽管接受了肝素治疗仍反复发生肺栓塞,且家族中有类似发作的报告,其中一些已证实是致命的。血液学研究显示进行性抗凝血酶活性明显降低。随后对这种自1965年(埃格尔伯格)以来已知的体质性和家族性缺陷的临床和血液学方面进行了讨论。这种情况相对罕见,但在反复出现血栓形成以及血栓对肝素具有生物学抗性时,不应妨碍通过功能、免疫和显色联合评估进行常规筛查:在这种情况下,使用维生素K拮抗剂进行长期治疗的指征是不可否认的。

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