Low plasma levels of tissue factor pathway inhibitor in patients with congenital factor V deficiency
Low plasma levels of tissue factor pathway inhibitor in patients with congenital factor V deficiency
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DOI：
10.1182/blood-2008-06-162453
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发表时间：
2008-11-01
期刊：
影响因子：
20.3
通讯作者：
Castoldi, Elisabetta
中科院分区：
文献类型：
--
作者：
Duckers, Connie;Simioni, Paolo;Castoldi, Elisabetta
重度凝血因子V（FV）缺乏与轻度至重度出血素质相关，但许多FV水平低于1%的患者出血少于预期。我们使用校准的自动血栓描记术来筛选严重FV缺乏的患者的保护性促凝血缺陷。FV缺陷血浆中的凝血酶生成仅在高组织因子浓度下可测量。在用纯化的FV重建FV缺陷血浆后，凝血酶生成随FV浓度急剧增加，在约10%FV时达到平台。用100%FV重建的FV缺乏血浆产生的凝血酶比正常血浆多几倍，特别是在低组织因子浓度（1.36 pM）或存在活化蛋白C的情况下，表明FV缺乏血浆中组织因子途径抑制物（TFPI）水平降低。血浆TFPI抗原和活性水平确实较低（P
Severe factor V (FV) deficiency is associated with mild to severe bleeding diathesis, but many patients with FV levels lower than 1% bleed less than anticipated. We used calibrated automated thrombography to screen patients with severe FV deficiency for protective procoagulant defects. Thrombin generation in FV-deficient plasma was only measurable at high tissue factor concentrations. Upon reconstitution of FV-deficient plasma with purified FV, thrombin generation increased steeply with FV concentration, reaching a plateau at approximately 10% FV. FV-deficient plasma reconstituted with 100% FV generated severalfold more thrombin than normal plasma, especially at low tissue factor concentrations (1.36 pM) or in the presence of activated protein C, suggesting reduced tissue factor pathway inhibitor ( TFPI) levels in FV-deficient plasma. Plasma TFPI antigen and activity levels were indeed lower (P