Hepatocellular Carcinoma in a Patient with Polycystic Liver Disease

Kawada, Manabu; Hayami, Noriko; Suwabe, Tatsuya; Hoshino, Junichi; Sumida, Keiichi; Mise, Kazuyuki; Hamanoue, Satoshi; Yamanouchi, Masayuki; Sawa, Naoki; Takaichi, Kenmei; Fujii, Takeshi; Ubara, Yoshifumi

doi:10.2169/internalmedicine.54.3924

Cited by 3 publications

(1 citation statement)

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“…Liver transplantation remains the only curative option for patients with PLD, being reserved for highly symptomatic patients to relieve symptom burden and improve quality of life [16][17][18][19]. Liver function is preserved in PLD and there is no increased risk of hepatocellular carcinoma [20]. Thus, most patients would not fulfil the common criteria for transplantation listing secondary to chronic liver disease (CLD), which requires a United Kingdom model for End-stage Liver Disease (UKELD) score �49, or the presence of hepatocellular carcinoma.…”

Section: Introductionmentioning

confidence: 99%

Liver transplant recipients with polycystic liver disease have longer waiting times but better long-term clinical outcomes than those with liver disease due to other causes: A retrospective cross-sectional study

Gittus,

Moore,

Ong

2024

PLoS ONE

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Introduction Liver transplantation is the only curative option for patients with polycystic liver disease (PLD). In the United Kingdom, these patients are listed on the variant syndrome list due to their preserved liver function reflected in the United Kingdom End-stage Liver Disease (UKELD) score. The transplantation and survival rates for this patient group in the UK have not been previously reported. Methods A retrospective cross-sectional analysis of patients receiving liver transplantation between 2010 and 2017 was performed using the NHS blood and transplantation database. This database contains the demographic, clinical parameters, indication for transplantation and follow-up of all patients in UK-based transplant centres. Basic statistics was performed using SPSS version 27. Results 5412 recipients received elective liver allografts in the study period. 1.6% (100) of recipients had PLD as their primary indication for transplantation with 60 receiving liver only allografts and 40 receiving combined liver-kidney allografts. PLD patients had a >3-fold longer mean waiting time for transplantation compared to non-PLD patients, 508 days v 154 days respectively. PLD patients receiving combined liver-kidney allografts had a longer waiting time than those receiving a liver only allograft, 610 days v 438 days respectively. There were comparable patient survival rates for people with PLD and non-PLD primary indications at 30 days (94.0% vs 97.6%) and 1 year (92.0% vs 93.2%) but improved survival rates at 5 years (81.3% vs 76.5%). There were also comparable allograft survival rates for people with PLD and non-PLD primary indications at 30 days (93.9% vs 95.3%) and 1 year (91.9% vs 91.2%) but improved survival rates at 5 years (82.5% vs 77.3%). Transplant centre-level analysis identified variation in the proportion of liver transplantations for people with PLD as their primary listed indication. Conclusions Patients with PLD wait significantly longer for liver transplantation compared to other indications. However, transplanted PLD patients demonstrate better longer-term patient and liver allograft survival rates compared to transplanted non-PLD patients. The unexpected variation between individual UK centres transplanting for PLD deserves further study.

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