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严重再生障碍性贫血异基因骨髓移植后发生的肝静脉闭塞病

Veno-occlusive disease of the liver after allogeneic bone marrow transplantation for severe aplastic anemia.

作者信息

Lee J H, Lee K H, Choi S J, Min Y J, Kim J G, Kim S, Lee J S, Kim S H, Park C J, Chi H S, Kim W K

机构信息

Department of Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

出版信息

Bone Marrow Transplant. 2000 Sep;26(6):657-62. doi: 10.1038/sj.bmt.1702583.

Abstract

There are few reports about the occurrence of hepatic VOD after BMT for severe aplastic anemia (SAA). We prospectively studied 17 patients with SAA after allogeneic BMT for the occurrence and severity of VOD. Plasma levels of protein C, protein S, antithrombin III, vWF, t-PA and PAI-1 were determined before preparative chemotherapy, on the day of marrow infusion, and on days 7, 14 and 21. VOD occurred in seven patients (41.2%) at a median of day 1 (range, day -2 to 15). Five had mild, and two moderate VOD. Platelet transfusion requirements were higher in the patients with VOD. The plasma levels of natural anticoagulants such as protein C, free protein S and antithrombin III decreased significantly on day 0 from the baseline levels. Plasma levels of t-PA, PAI-1 and vWF increased significantly in the early post-transplant period compared to the baseline levels. The mean plasma levels of t-PA on day 7 (P = 0.016) and PAI-1 on days 0 and 7 (P = 0.016, 0.032) were higher in the patients with VOD. In summary, we observed hypercoagulability and a high incidence of VOD after allogeneic BMT for SAA. Levels of t-PA and PAI-1 were significantly higher in the patients with VOD after BMT.

摘要

关于重型再生障碍性贫血(SAA)患者接受骨髓移植(BMT)后发生肝静脉闭塞病(VOD)的报道较少。我们前瞻性地研究了17例接受异基因BMT的SAA患者VOD的发生情况及严重程度。在预处理化疗前、骨髓输注当天、以及第7、14和21天测定血浆蛋白C、蛋白S、抗凝血酶III、血管性血友病因子(vWF)、组织型纤溶酶原激活物(t-PA)和纤溶酶原激活物抑制剂-1(PAI-1)的水平。7例患者(41.2%)发生了VOD,中位发生时间为第1天(范围为-2至15天)。5例为轻度VOD,2例为中度VOD。发生VOD的患者血小板输注需求更高。天然抗凝剂如蛋白C、游离蛋白S和抗凝血酶III的血浆水平在第0天较基线水平显著降低。与基线水平相比,移植后早期血浆t-PA、PAI-1和vWF水平显著升高。发生VOD的患者第7天t-PA的平均血浆水平(P = 0.016)以及第0天和第7天PAI-1的平均血浆水平(P = 0.016,0.032)更高。总之,我们观察到异基因BMT治疗SAA后存在高凝状态以及VOD的高发生率。BMT后发生VOD的患者t-PA和PAI-1水平显著更高。

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