Dyskeratosis congenita: Hematologic evaluation of a sibship and review of the literature

Abstract
Dyskeratosis conagenita is a rare genodermatosis whose hematologic complications include pancytopenia of variable time of onset, a propensity for opportunistic infections, and neoplasia. A family in which the disorder segregated in 3 generations and involved 9 members is reported, and the hematologic data of the 46 previously reported cases are reviewed.