Chronic graft-versus-host disease of the liver: Presentation as an acute hepatitis
Chronic graft-versus-host disease of the liver: Presentation as an acute hepatitis
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DOI：
10.1053/jhep.2000.20067
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发表时间：
2000-12-01
期刊：
影响因子：
13.5
通讯作者：
McDonald, GB
中科院分区：
文献类型：
--
作者：
Strasser, SI;Shulman, HM;McDonald, GB
肝脏的慢性移植物抗宿主病（GVHD）通常表现为一种无痛性胆汁淤积性疾病，累及皮肤、口腔和眼睛。我们观察了14例慢性GVHD的肝脏表现为血清转氨酶明显升高，临床上类似于急性病毒性肝炎。肝功能不全的发生是在异基因造血细胞移植后294天（范围，74-747天），与最近停止或逐渐减少免疫抑制药物相一致。血清丙氨酸转氨酶（ALT）峰值中位数为1，640 U/L（698- 2，565 U/L），胆红素中位数为12.3 mg/dL（0.9-55.9 mg/dL）。所有活检均显示GVHD的特征性特征，伴有小胆管受损和变性。其他特征包括明显的小叶肝炎、中度至显著量的肝细胞不安、窦状隙炎症伴小静脉周围坏死性炎症灶和许多嗜酸性小体分散在整个小叶中。当高剂量的免疫抑制剂治疗后不久介绍，逐步改善和最终正常化的肝酶和胆红素水平进行了观察。然而，在未作出诊断和治疗延迟的情况下，出现了进行性胆汁淤积的图片与小胆管和门静脉纤维化的损失的组织学证据，我们得出结论，慢性肝脏GVHD表现为急性肝炎的独特综合征，可以在没有接受或接受最低限度的免疫抑制药物治疗的风险患者中识别。肝活检是必要的，以排除肝功能不全的病毒原因，并确认小胆管的特征性异常。早期应用大剂量免疫抑制剂可预防胆管的进行性破坏，并可有效缓解黄疸。
Chronic graft-versus-host disease (GVHD) of the liver usually presents as an indolent cholestatic disease in patients with skin, mouth, and eye involvement. We observed 14 patients in whom chronic GVHD of the liver presented with marked elevations of serum aminotransferases, clinically resembling acute viral hepatitis. Onset of liver dysfunction was at 294 days (range, 74-747 days) after allogeneic hematopoietic cell transplantation and coincided with a recent cessation or taper of immunosuppressive drugs. Median peak serum alanine transaminase (ALT) was 1,640 U/L (698-2,565 U/L), and median bilirubin was 12.3 mg/dL (0.9-55.9 mg/dL). All biopsies showed characteristic features of GVHD with damaged and degenerative small bile ducts. Other features included a marked lobular hepatitis, moderate to marked amounts of hepatocyte unrest, sinusoidal inflammation with perivenular necroinflammatory foci, and many acidophilic bodies scattered throughout the lobule. When high-dose immunosuppressive therapy was instituted soon after presentation, progressive improvement and eventual normalization of liver enzymes and bilirubin levels were observed. However, in cases in which the diagnosis was not made and therapy was delayed, a progressive cholestatic picture emerged with histologic evidence of loss of small bile ducts and portal fibrosis, We conclude that a distinct syndrome of chronic liver GVHD presenting as an acute hepatitis can be recognized in a patient at risk who is receiving no, or minimal, immunosuppressive medications. Liver biopsy is necessary to exclude viral causes of liver dysfunction and to confirm characteristic abnormalities of small bile ducts. Institution of high-dose immunosuppression can prevent progressive bile duct destruction and effect resolution of jaundice if given early.