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原发性抗磷脂综合征伴脑缺血、血小板减少、贫血和蛋白尿,用华法林钾成功治疗。

Primary antiphospholipid syndrome presenting with cerebral ischemia, thrombocytopenia, anemia and proteinuria successfully treated with warfarin potassium.

作者信息

Miyata M, Sakata Y, Shibukawa G, Shishido H, Munakata O, Kazuta Y, Shio K, Sasajima T, Sato Y, Kasukawa R

机构信息

Department of Internal Medicine II, Fukushima Medical University School of Medicine.

出版信息

Intern Med. 2000 Sep;39(9):748-53. doi: 10.2169/internalmedicine.39.748.

DOI:10.2169/internalmedicine.39.748
PMID:10969908
Abstract

A 30-year-old woman with primary antiphospholipid syndrome (PAPS) presented with cerebral ischemia, thrombocytopenia, anemia and proteinuria. Administration of warfarin potassium, without concomitant corticosteroid administration, significantly improved all of these symptoms along with a decrease in the titers of antiCL-beta2-GP-I antibodies and a shortening of prolonged APTT. Therefore, the antiphospholipid antibodies in this patient could have been evoked by vitamin-K-dependent coagulation factors or plasma proteins which are assumed to undergo conformational changes exposing cryptic epitopes. This case report provides clues to the mechanisms underlying the production of antiphospholipid antibodies in patients with PAPS.

摘要

一名患有原发性抗磷脂综合征(PAPS)的30岁女性出现脑缺血、血小板减少、贫血和蛋白尿。在未同时给予皮质类固醇的情况下给予华法林钾,显著改善了所有这些症状,同时抗CL-β2-GP-I抗体滴度降低,延长的活化部分凝血活酶时间(APTT)缩短。因此,该患者体内的抗磷脂抗体可能是由维生素K依赖的凝血因子或血浆蛋白诱发的,这些因子或蛋白被认为会发生构象变化,暴露出隐蔽表位。本病例报告为PAPS患者抗磷脂抗体产生的潜在机制提供了线索。

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