原发性肝脏非霍奇金淋巴瘤所致急性肝衰竭
Acute Liver Failure Caused by Primary Non-Hodgkin's Lymphoma of the Liver.
作者信息
Romacho López L, León Díaz F J, Sánchez Pérez B, Pérez Daga J A, Fernández Aguilar J L, Montiel Casado M C, Aranda Narváez J M, Suárez Muñoz M A, Santoyo Santoyo J
机构信息
Liver Transplant Unit, Regional Hospital, Málaga, Spain.
Liver Transplant Unit, Regional Hospital, Málaga, Spain.
出版信息
Transplant Proc. 2016 Nov;48(9):3000-3002. doi: 10.1016/j.transproceed.2016.10.005.
INTRODUCTION
Acute liver failure (ALF) is a rare syndrome involving maximum liver dysfunction. This disease is characterized by a less than 26-week history of coagulopathy (INR ≥1.5) and hepatic encephalopathy and generally occurs in patients without any previously known disease.
METHODS
We report the case of a healthy 25-year-old subject who presented with fulminant liver failure caused by a primary non-Hodgkin's lymphoma of the liver that required emergency liver transplantation. Diagnosis was based on pathologic confirmation of T-cell/histiocyte-rich large B-cell lymphoma and submassive hepatocyte necrosis. One year after surgery, the patient remains in complete remission.
CONCLUSIONS
Fulminant liver failure is a sudden-onset severe disease that can be caused by a primary non-Hodgkin's lymphoma of the liver, which accounts for <1% of extranodal lymphomas. The diagnosis of this rare disease demands high diagnostic suspicion, and progression can be prevented through liver transplantation.
引言
急性肝衰竭（ALF）是一种罕见的综合征，涉及严重的肝功能障碍。该疾病的特征为凝血功能障碍（国际标准化比值[INR]≥1.5）和肝性脑病的病程少于26周，且通常发生于既往无任何已知疾病的患者。
方法
我们报告了一例25岁健康受试者的病例，该患者因原发性肝脏非霍奇金淋巴瘤导致暴发性肝衰竭，需要紧急肝移植。诊断基于富含T细胞/组织细胞的大B细胞淋巴瘤及亚大块肝细胞坏死的病理证实。术后一年，患者仍处于完全缓解状态。
结论
暴发性肝衰竭是一种起病突然的严重疾病，可由原发性肝脏非霍奇金淋巴瘤引起，其在结外淋巴瘤中所占比例不到1%。对这种罕见疾病的诊断需要高度的诊断怀疑，通过肝移植可预防病情进展。
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