Abstract
Pulmonary hemorrhage is a rare, life-threatening condition affecting premature infants. There is no single etiological explanation for it but some common denominators include the presence of extreme prematurity, respiratory distress syndrome, surfactant use, birth asphyxia, etc. Although the incidence of pulmonary hemorrhage in neonates may be small, it is associated with a high risk of mortality. Congenital bleeding disorders such as hemophilia are rare coagulation disorders that have been known to present in the early neonatal period with an increased tendency for bleeding after blood draws, circumcision, surgical interventions, intracranial hemorrhage, oral or mucosal bleeding, and very rarely as gastrointestinal hemorrhage. There are no reports so far in the published literature of hemophilia presenting as pulmonary hemorrhage in early life. We report an unusual primary presentation of hemophilia B in a premature, monochorionic-diamniotic twin with acute life-threatening pulmonary hemorrhage with no family history of bleeding disorders.