Abstract: Objective: To study the immunological characteristics of patients with hepatolenticular degeneration(HLD).Methods: The levels of serum immunoglobulins,complement and T-lymphocyte subsets,the indexes of the humoral immunity and the propotion of CD₁₉⁺ in 21 patients with HLD and 19 normal controls were measured with immune turbidimetry and flow cytometry.Results: The levels of IgG and IgM in group HLD were increased compared with the controls,except for C₄,which was depressed(P<0.05~P<0.01),together with the ratio of CD₄⁺/CD₈⁺,the propotion of CD₃^-CD₁₆⁺CD₅₆⁺、CD₄⁺ increased(P<0.05~P<0.01).Conclusions: The study showed that there was disorder of the immunological state in patients with HLD,and suggests the manifestation of which is activation of the humoral immunity and depression of the cellular immunity.