Lymphocytapheresis was carried out as a preliminary clinical trial for four patients with symptomatic primary biliary cirrhosis (PBC). After lymphocytapheresis, severe itching disappeared in two patients and jaundice improved or disappeared in all cases. Serum levels of total bilirubin, alkaline phosphatase, gamma-glutamyl transpeptidase and transaminase were decreased after the treatment. In liver tissue, the number of infiltrated lymphocytes was slightly decreased in both the parenchyma and portal tract. Increased CD3(-)CD56(+) lymphocytes (NK cells) in the liver were dramatically diminished after the treatment. CD3(+)CD56(-) lymphocytes (T cells), especially CD8(+) lymphocytes and CD56(+) T lymphocytes, were increased after the treatment in two cases. Although these results are preliminary and based on only a small number of patients and over short observation periods, lymphocytapheresis might be a useful therapeutic tool for patients with advanced PBC, giving excellent improvement of symptoms and results of liver function tests. (C) 1998 Elsevier Science Ireland Ltd. All rights reserved.