Single-centre experience of liver transplantation for familial amyloidotic polyneuropathy of non-Val30Met variants in Chinese patients.

OBJECTIVE

To review our experience of liver transplantation (LT) for patients with non-Val30Met familial amyloidotic polyneuropathy (FAP) and patients receiving FAP livers.

METHOD

Data of six FAP patients and five FAP liver recipients, all Chinese, were reviewed.

RESULTS

Among the six FAP patients, five patients were of the V30A variant and one patient was of the G67E variant. One patient had malnutrition. Three patients had peripheral neuropathy. Four patients had orthostatic hypotension. Five patients had cardiac involvement. Two patients underwent living-donor LT and four patients underwent deceased-donor LT. One patient had progressive cardiac and neurological involvement after transplantation. Three patients showed either improvement or a static condition. All five FAP liver recipients had hepatitis-B-related hepatocellular carcinoma before transplantation. With a median follow-up period of 49 months, all of them survived without tumour recurrence. The first recipient developed systemic transthyretin amyloidosis six years after transplantation. Upper endoscopy confirmed the presence of gastric amyloidosis, and nerve conduction test showed evidence of axonal sensorimotor polyneuropathy.

CONCLUSIONS

Most of the FAP patients and FAP liver recipients displayed satisfactory outcomes after transplantation. LT halted disease progression in the FAP patients who had early presentation of the disease.