Lee J H, Lee K H, Choi J S, Zang D Y, Kim S B, Kim S W, Suh C, Lee J S, Kim W K, Lee Y S, Kim S H
Department of Medicine, Asan Medical Center, University of Ulsan, Seoul, Korea.
J Korean Med Sci. 1996 Apr;11(2):118-26. doi: 10.3346/jkms.1996.11.2.118.
Veno-occlusive disease (VOD) of the liver is a clinical syndrome characterized by hyperbilirubinemia, painful hepatomegaly, and fluid retention. In the bone marrow transplantation (BMT) setting, VOD is caused by dose-intensive chemotherapy and/or radiotherapy used to prepare patients for transplant. VOD occurs in up to 50% of the patients who undergo BMT and is usually associated with a high mortality rate. Until recently, there was no proven effective medical therapy for this condition once it was clinically apparent. We report here on the frequency and treatment result of VOD with rt-PA in our allogeneic BMT patients. Eight patients (median age 28.5 years) underwent allogeneic BMT from December, 1993 to June, 1995 in Asan Medical Center. Six leukemia patients were prepared for BMT with busulfan and cyclophosphmide, while two aplastic anemia patients received cyclophosphamide and antithymocyte globulin. VOD was defined as having two of the following features before day 20 posttransplant: jaundice (bilirubin > or = 2 mg/dL), tender hepatomegaly and/or right upper quadrant pain, ascites and/or unexplained weight gain (> 2% from baseline). All patients who were diagnosed with VOD received rt-PA (10-20 mg/day) and heparin (10,000 U/day). Three (37.5%) of the eight patients developed VOD that occurred between 6 and 10 days posttransplant. All three patients developed jaundice, weight gain, and tender hepatomegaly. Ascites and renal insufficiency occurred in two patients and pleural effusion in one patient. rt-PA and heparin were begun 6 to 26 days posttransplant and rt-PA was administered for 7 to 14 days. All three patients responded to the therapy; bilirubin levels began to decrease at 4 to 13 days from the start of therapy. They are all alive at day 111, 316, and 548 days posttransplant. None of the patients had significant hemorrhagic complications after rt-PA treatment. Prolonged administration of rt-PA was feasible without bleeding episode and it seems that rt-PA may alter the natural course of VOD.
肝静脉闭塞病（VOD）是一种以高胆红素血症、肝肿大疼痛和液体潴留为特征的临床综合征。在骨髓移植（BMT）情况下，VOD是由用于使患者为移植做准备的大剂量化疗和/或放疗引起的。VOD发生在高达50%接受BMT的患者中，并且通常与高死亡率相关。直到最近，一旦这种疾病在临床上显现出来，还没有经过证实的有效药物治疗方法。我们在此报告阿山医疗中心异基因BMT患者中VOD的发生率及rt-PA治疗结果。1993年12月至1995年6月期间，8例患者（中位年龄28.5岁）接受了异基因BMT。6例白血病患者接受白消安和环磷酰胺进行BMT预处理，而2例再生障碍性贫血患者接受环磷酰胺和抗胸腺细胞球蛋白治疗。VOD定义为移植后20天内出现以下两种特征：黄疸（胆红素≥2mg/dL）、肝肿大压痛和/或右上腹疼痛、腹水和/或无法解释的体重增加（比基线增加>2%）。所有被诊断为VOD的患者均接受rt-PA（10 - 20mg/天）和肝素（10,000U/天）治疗。8例患者中有3例（37.5%）在移植后6至10天发生VOD。所有3例患者均出现黄疸、体重增加和肝肿大压痛。2例患者出现腹水和肾功能不全，1例患者出现胸腔积液。rt-PA和肝素在移植后6至26天开始使用，rt-PA给药7至14天。所有3例患者对治疗均有反应；胆红素水平在治疗开始后4至13天开始下降。在移植后第111、316和548天，他们均存活。rt-PA治疗后，所有患者均未出现明显的出血并发症。长时间使用rt-PA是可行的，且无出血事件发生，似乎rt-PA可能改变VOD的自然病程。
