遗传性血色素沉着症中的原发性肝癌：54例临床、病理及发病机制研究

Primary liver cancer in genetic hemochromatosis: a clinical, pathological, and pathogenetic study of 54 cases.

作者信息

Deugnier Y M, Guyader D, Crantock L, Lopez J M, Turlin B, Yaouanq J, Jouanolle H, Campion J P, Launois B, Halliday J W

机构信息

Clinique des Maladies du Foie and INSERM U 49, CHRU Pontchaillou, Rennes, France.

出版信息

Gastroenterology. 1993 Jan;104(1):228-34. doi: 10.1016/0016-5085(93)90856-8.

DOI:10.1016/0016-5085(93)90856-8

PMID:8419246

Abstract

BACKGROUND

Although liver cancer arises frequently in the course of genetic hemochromatosis (GH), it has not been previously studied in a large series of patients with well-defined GH.

METHODS

The bioclinical and pathological data from 1 cholangiocarcinoma and 53 hepatocellular carcinomas (HCCs) complicating GH in 32 untreated and 22 de-ironed patients are reported.

RESULTS

This study (1) adds three new well-documented cases of HCC in noncirrhotic but only fibrotic hemochromatotic liver, (2) shows the high prevalence (83%) of proliferative and often dysplastic (70%) iron-free foci in the nontumorous liver of untreated patients, and (3) emphasizes the significant increase of cirrhosis (81% vs. 28%) and of associated noniron-related risk factors, mainly chronic alcoholism (48% vs. 25%) and tobacco smoking (50% vs. 18%) in patients with HCC compared with matched hemochromatotic patients without HCC.

CONCLUSIONS

These data (1) suggest that iron-free foci may be markers of an early stage of HCC in GH and (2) supply the basis for defining a cost-effective policy for the screening of HCC in GH patients.

摘要

背景

尽管肝癌在遗传性血色素沉着症（GH）病程中经常出现，但此前尚未在大量明确诊断为GH的患者中进行研究。

方法

报告了32例未经治疗和22例去铁治疗患者中1例胆管癌和53例并发GH的肝细胞癌（HCC）的生物临床和病理数据。

结果

本研究（1）新增了3例记录充分的非肝硬化但仅纤维化血色素沉着性肝病合并HCC的病例，（2）显示未经治疗患者的非肿瘤性肝脏中增殖性且常发育异常（70%）的无铁病灶患病率很高（83%），（3）强调与匹配的无HCC血色素沉着症患者相比，HCC患者的肝硬化（81%对28%）及相关非铁相关危险因素显著增加，主要是慢性酒精中毒（48%对25%）和吸烟（50%对18%）。