Bone marrow failure in dyskeratosis congenita*

Abstract

We report a case of dyskeratosis congenita ( DCG ) with neutropenia, lymphocytopenia and thrombocytopenia. Peripheral blood T lymphocytes (T cells) were proved to have a suppressive effect on the colony forming unit granulocyte-macrophage (CFU-GM). Splenectomy caused a transient increase of neutrophil count with the disappearance of the suppressive T cell activity. However, pancytopenia recurred without re-appearance of suppressive T cell activity.