Bone marrow failure in dyskeratosis congenita.
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.
D000293:Adolescent; D002452:Cell Count; D006412:Hematopoietic Stem Cells; D006801:Humans; D008231:Lymphopenia / Q000209:etiology; D008297:Male; D009503:Neutropenia / Q000209:etiology; D010198:Pancytopenia / Q000209:etiology*; D012871:Skin Diseases / Q000151:congenital*; D013156:Splenectomy; D013577:Syndrome; D050378:T-Lymphocytes, Regulatory / Q000276:immunology