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静脉闭塞性疾病是一种特定的综合征,还是造血干细胞移植(HSCT)中生理病理止血变化的加剧?

Is veno-occlusive disease a specific syndrome or the exacerbation of physiopathologic hemostatic changes in hematopoietic stem cell transplantation (HSCT)?

作者信息

Villalón L, Avello A G, César J, Odriozola J, López J, Oteyza J P, Laraña J G, Cantalapiedra A, Navarro J L

机构信息

Department of Hematology, Hospital Ramón y Cajal, Madrid, Spain.

出版信息

Thromb Res. 2000 Sep 1;99(5):439-46. doi: 10.1016/s0049-3848(00)00269-3.

Abstract

The objective of the present study was to analyze whether veno-occlusive disease (VOD) is based on specific findings or whether this syndrome is the exacerbation of changes in hemostatic parameters that develop following hematopoietic stem cell transplantation (HSCT). 40 patients undergoing HSCT were enrolled (6 allogeneic bone marrow transplantation and 34 peripheral stem cell rescue-2 allogeneic, 32 autologous). Measurements of hemostatic parameters (endothelial, hypercoagulability and fibrinolytic markers) were obtained prior to chemotherapy and weekly thereafter for 3 weeks. The incidence of VOD was 15%. HSCT showed a state of moderate hypercoagulability (increase of thrombin-antithrombin complex and fibrinogen, and decrease of Factor VII, Protein C, and antithrombin-III), probably as a consequence of marked endothelial damage (increase of von Willebrand Factor and tissue plasminogen activator). All these alterations create a potentially prothrombotic state, more pronounced in VOD. The decreasing incidence of VOD and the moderate disease in all patients suggest that increasing improvements in transplant strategies have reduced the risk and severity of a syndrome that at the beginning of the transplantation era was a leading cause of morbidity/mortality.

摘要

本研究的目的是分析静脉闭塞性疾病(VOD)是否基于特定的表现,或者该综合征是否是造血干细胞移植(HSCT)后发生的止血参数变化的加剧。纳入了40例接受HSCT的患者(6例同种异体骨髓移植和34例外周干细胞救援——2例同种异体,32例自体)。在化疗前以及此后3周每周测量止血参数(内皮、高凝和纤溶标记物)。VOD的发生率为15%。HSCT显示出中度高凝状态(凝血酶-抗凝血酶复合物和纤维蛋白原增加,因子VII、蛋白C和抗凝血酶III减少),这可能是明显内皮损伤(血管性血友病因子和组织纤溶酶原激活物增加)的结果。所有这些改变都造成了潜在的血栓形成状态,在VOD中更为明显。VOD发病率的降低以及所有患者病情的中度表明,移植策略的不断改进降低了一种综合征的风险和严重程度,在移植时代初期,该综合征是发病/死亡的主要原因。

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