Gane E, Portmann B, Saxena R, Wong P, Ramage J, Williams R
Institute of Liver Studies, King's College Hospital, London, United Kingdom.
Hepatology. 1994 Jul;20(1 Pt 1):88-94. doi: 10.1016/0270-9139(94)90138-4.
Nodular regenerative hyperplasia has not previously been reported in patients who have undergone orthotopic liver transplantation. In this report we describe the clinical, biochemical and histologic findings in nine liver transplant recipients in whom nodular regenerative hyperplasia developed between 6 and 144 mo (median, 64 mo) after transplantation. Six of the patients manifested features of portal hypertension (variceal bleeding, ascites, severe intractable peripheral edema), whereas three patients were asymptomatic at the time of diagnosis. All patients had elevated serum alkaline phosphatase (median, 269 IU; range, 159 to 1182 IU) and gamma-glutamyl transferase levels (median, 247 IU; range 104 to 1513 IU). Other liver function tests remained normal. In five patients, earlier biopsies had identified histological features of venous outflow block, consistent with azathioprine hepatotoxicity. All nine patients had been taking azathioprine up to the time of presentation. After withdrawal of azathioprine, liver function tests improved appreciably in five patients and histologic improvement was documented in four of these who underwent follow-up liver biopsies. In the remaining four patients progressive graft failure ensued and all subsequently underwent repeat liver retransplantation. At a certain stage, nodular regenerative hyperplasia, in the context of a liver graft, becomes an irreversible lesion, emphasizing the need for early identification and withdrawal of the agent.
此前尚未有原位肝移植患者发生结节性再生性增生的报道。在本报告中，我们描述了9例肝移植受者的临床、生化和组织学表现，这些患者在移植后6至144个月（中位数为64个月）出现了结节性再生性增生。其中6例患者表现出门静脉高压的特征（静脉曲张出血、腹水、严重顽固性外周水肿），而3例患者在诊断时无症状。所有患者血清碱性磷酸酶水平升高（中位数为269 IU；范围为159至1182 IU），γ-谷氨酰转移酶水平也升高（中位数为247 IU；范围为104至1513 IU）。其他肝功能检查仍正常。5例患者早期活检发现有静脉流出道阻塞的组织学特征，符合硫唑嘌呤肝毒性表现。所有9例患者在出现症状时均一直在服用硫唑嘌呤。停用硫唑嘌呤后，5例患者的肝功能检查明显改善，其中4例接受随访肝活检的患者有组织学改善记录。其余4例患者发生了进行性移植物功能衰竭，并随后均接受了再次肝移植。在某个阶段，肝移植背景下的结节性再生性增生会成为不可逆病变，这强调了早期识别并停用该药物的必要性。
