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[严重肝静脉闭塞病(VOD)经支持治疗成功治愈,但随后出现晚期复发]

[Severe hepatic veno-occlusive disease (VOD) which was successfully treated with supportive therapy, but subsequently developed late-recurrence].

作者信息

Matsuoka S, Okamoto S, Ishida A, Wakui M, Watanabe R, Moriki T, Ikeda Y, Hirabayashi N

机构信息

Department of Medicine, Keio University School of Medicine, Tokyo.

出版信息

Rinsho Ketsueki. 1998 Feb;39(2):139-45.

PMID:9545827
Abstract

A 40-year-old man with chronic myelogenous leukemia in chronic phase received an allogeneic marrow graft from his HLA identical brother. He was conditioned with busulfan (16 mg/kg) and cyclophosphamide (120 mg/kg). Graft-versus-host disease (GVHD) prophylaxis was attempted with cyclosporine A (CYA) and methotrexate. On day 30, weight gain, ascites and hepatomegaly developed in addition to an elevation of total bilirubin (TB). He was diagnosed as having veno-occlusive disease (VOD) and treated conservatively. The TB level increased up to 20.1 mg/dl on day 66, then reduced to 2.1 mg/dl on day 129. By that time ascites and hepatomegaly also had completely resolved. However, on day 134. The TB level started to increase again, when the lesions of chronic GVHD were observed in the eye, the mouth, and the skin. CYA was started on day 142, and FK506 was substituted for CYA on day 161. Despite the improvement of oral and skin lesions, TB level continued to rise, and he died of respiratory failure due to ARDS on day 186. Autopsy revealed both acute and old hepatic VOD lesions, suggesting the occurrence of late-onset VOD which probably contributed to the liver dysfunction observed after clinical resolution of the first episode of VOD.

摘要

一名处于慢性期的40岁慢性粒细胞白血病男性患者接受了来自其 HLA 配型相同的兄弟的异基因骨髓移植。他接受了白消安(16mg/kg)和环磷酰胺(120mg/kg)的预处理。尝试使用环孢素A(CYA)和甲氨蝶呤预防移植物抗宿主病(GVHD)。在第30天,除了总胆红素(TB)升高外,还出现了体重增加、腹水和肝肿大。他被诊断为患有肝静脉闭塞病(VOD)并接受了保守治疗。TB水平在第66天升至20.1mg/dl,然后在第129天降至2.1mg/dl。到那时腹水和肝肿大也已完全消退。然而,在第134天,TB水平再次开始升高,此时在眼睛、口腔和皮肤观察到慢性GVHD病变。CYA于第142天开始使用,FK506在第161天替代CYA。尽管口腔和皮肤病变有所改善,但TB水平持续上升,他在第186天因ARDS导致呼吸衰竭死亡。尸检显示既有急性又有陈旧性肝VOD病变,提示发生了迟发性VOD,这可能是导致首次VOD临床缓解后观察到的肝功能障碍的原因。

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