番茄社区

BOSTON -- Patients who developed cirrhosis related to Wilson's disease did not seem to have a higher risk for hepatocellular carcinoma (HCC), researchers said here.

In a series of 130 Wilson's disease patients, about 60% were diagnosed with cirrhosis, but there were only two cases of HCC during a median of 17.1 years of follow-up, reported Suzanne van Meer, MD, from the in Utrecht, the Netherlands, and colleagues.

"The estimated annual risk of hepatocellular carcinoma in all our patients was 0.09%. The estimated annual risk of hepatocellular cancer in our Wilson's disease patients with cirrhosis was 1.4%," van Meer said at her poster presentation during the American Association for the Study of Liver Diseases annual meeting.

As a result, van Meer's group concluded that "our data do not support regular HCC surveillance in [Wilson's disease]."

Patients in the retrospective study came from three Dutch hospitals and had a confirmed diagnosis of Wilson's disease (Leipzig score >4).

The patients were stratified by liver disease severity at baseline with the majority (36%) receiving a diagnosis of compensated cirrhosis/Child Pugh Stage A. The next most common diagnosis was decompensated cirrhosis/Child Pugh Stage B or Stage C (21%). Other diagnoses included mild liver disease exhibiting only disturbed liver biochemistry, moderate liver disease (disturbed biochemistry and imaging or histology abnormalities), unknown liver disease, and normal liver function.

At baseline, cirrhosis was present in 74 patients with 64% compensated and 36% decompensated.

The median period of follow-up for the patients ranged from 1.5 to 51.2 years. At the end of follow-up, 28 patients had undergone liver transplantation with two deaths related to transplantation. Five patients died due to complications of their liver disease.

Treatment in the majority of patients (92%) was with zinc, which blocks the uptake of copper. Other treatments included penicillamine and trientine, and all treatment had been done for 1 year in the analyzed set of patients.

The researchers said that in 34% of cases decoppering efficacy was excellent, while in 42% the decoppering efficacy was considered to be moderate, and in 13% decoppering efforts were considered to have poor outcomes.

The first patient to develop HCC was a 39-year-old male who presented with decompensated cirrhosis in combination with the cancer. The second patient was a 63-year-old female with unequivocal Wilson's disease diagnosed 50 years earlier.

"Despite excellent decoppering at the end of [follow-up], she progressed to decompensated cirrhosis in which an HCC developed," the authors wrote.

But they pointed out that there were no additional risk factors for liver disease in these patients.

"We know that people with cirrhosis do have an increased risk of hepatocellular carcinoma development. We also know that the risk seems low for those with Wilson's disease," van Meer told 番茄社区. "So the risk is not the same for every cirrhosis patient. We determined that in the case of Wilson's disease cirrhosis, the risk of developing hepatocellular carcinoma is too low to perform surveillance."

However, she said that patients with Wilson's disease must undergo regular medical treatment to avoid symptoms related to copper buildup in the organs and particularly in the liver.

"These results are not surprising," said senior lecturer in medicine and honorary consultant in hepatology at Royal Free Hospital/University College London. "In fact, I was surprised the number of patients diagnosed with hepatocellular cancer is so high."

"We have yet to see a case of hepatocellular cancer among our Wilson's disease patients," Tsochatzis told 番茄社区. "We feel that Wilson's disease might be protective against cancer, and certainly having the disease does not warrant special surveillance for development of cancer."

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