This report describes a new familial syndrome characterized by a combi
nation of bone marrow failure and chronic liver disease. This disorder
appears to be genetic in origin with an autosomal dominant inheritanc
e and was characterized by hyperactivity of the immune system with inc
reased activated cytotoxic T lymphocytes in peripheral blood and bone
marrow and the presence of gamma-interferon messenger RNA in bone marr
ow of several cases.