Recurrence of Hereditary Hemorrhagic Telangiectasia After Liver Transplantation: Clinical Implications and Physiopathological Insights.

Liver transplantation (LT) has been proposed as a curative treatment in hereditary hemorrhagic telangiectasia (HHT) with severe hepatic involvement. We provide a long-term evaluation of graft status after LT for HHT, with a focus on the risk of recurrence. The present study included all patients prospectively followed up after LT for HHT in the Lyon Liver Transplant Unit from 1993 to 2010, with a survival of more than 1 year. Protocol clinical, radiological, and histological examinations were performed at regular intervals. Fourteen patients were included (13 women and one man). Median age at LT was 52.5 years (range: 33.1-66.7). In eight patients (seven female), disease recurrence was diagnosed by abnormal radiological features, suggestive of microcirculatory disturbances. Typical vascular lesions, including telangiectasia, were demonstrated by liver biopsy in five of these patients. The median interval between LT and diagnosis of recurrence was 127 months (range: 74-184). The risk of recurrence increased over time; estimated cumulative risk was 47.9% at 15 years. Liver tissue analysis found the coexistence of an angiogenic process combined with endothelial microchimerism, as shown by the presence of vascular lining cells of recipient origin. Conclusion: The present data show that disease recurrence occurs, usually after a long delay, in a significant number of patients treated by LT for liver complications of HHT. This strongly supports the necessity of a lifelong follow-up and suggests that therapeutic strategy needs discussion and evaluation, especially of the role of potential adjuvant treatments to LT, such as antiangiogenic medications, when recurrent disease appears.

Survival and prognostic factors after adjuvant 131iodine-labeled lipiodol for hepatocellular carcinoma: a retrospective analysis of 106 patients over 20 years.

OBJECTIVE: Hepatocellular carcinoma (HCC) has high recurrence rate after curative treatment. The aim of the present study was to report our experience with adjuvant use of 131I-lipiodol after curative treatment of HCC in terms of recurrence and survival in a large cohort of patients with a long follow-up. METHODS: All patients treated with 131I-lipiodol after curative treatment of HCC in two French centers from 1991 to 2009 were included in a retrospective cohort study. RESULTS: One hundred and six patients were included. The median (range) follow-up was 6 years (0.3-22). Forty-three patients (41%) had cirrhosis. Recurrence-free survival rates at 1, 2, 5, 10, and 20 years were 73, 57, 40, 30, and 14%, respectively. Cirrhosis was an independent predictive factor of recurrence [RR = 1.18, 95% CI (1.11-3.02), p = 0.019]. Overall, survival rates at 1, 2, 5, 10, and 20 years were 90, 83, 59, 37, and 23%, respectively. Prognostic factors were recurrence [RR = 2.73, 95% CI (1.35-5.54); p = 0.005], age over 60 years (RR = 1.91, 95% CI [1.02-3.61]; p = 0.044), and tumor number over 3 [RR = 3.31, 95% CI (1.25-8.77); p = 0.016]. CONCLUSION: Our results suggest that the effect of 131I-lipiodol after curative treatment of HCC could be related to a beneficial impact on risk factors of early tumor recurrence. This could be evaluated in further studies using modern radioembolization methods.

Negative impact of de novo malignancies rather than alcohol relapse on survival after liver transplantation for alcoholic cirrhosis: a retrospective analysis of 305 patients in a single center.

OBJECTIVES: Alcoholic liver disease is a leading indication for liver transplantation (LT). The aim of this study was to evaluate long-term results and survival prognostic factors of LT in this indication from a large cohort of patients. METHODS: From October 1990 to October 2005, 305 consecutive patients with alcoholic cirrhosis (from 594 patients presenting with cirrhosis, i.e., 51.3%) underwent LT in our center. There were 229 men and 76 women, with a median age of 50 yr (range 30-68). Clinical and biological variables with possible prognostic value were analyzed. RESULTS: Global survival rate was 92.6% at 1 yr, 88.5% at 3 yr, 84.3% at 5 yr, and 73.4% at 10 yr, and was similar (P=0.78, log-rank test) to that of patients transplanted for other cirrhosis (88.8% at 1 yr, 84.1% at 3 yr, 80.6% at 5 yr, and 74.7% at 10 yr). Recurrence of alcohol consumption was observed in 37 patients (12.1%). De novo cancer occurred in 35 patients after LT (11.5%). Univariate analysis disclosed that male gender, history of smoking, and de novo carcinoma were significant survival prognostic factors (P<0.05, log-rank test). CONCLUSIONS: Our results strongly confirm that alcoholic liver disease is an excellent indication for LT, but long-term survival is reduced because of other target-organ damage of both alcohol and tobacco, especially aero-digestive malignancies, which are greater causes of morbidity and mortality than is recurrent alcohol liver disease.