Factor V inhibitor in neonatal intracranial hemorrhage secondary to severecongenital factor V deficiency

We report a newborn infant girl, born to consanguine ous parents, with recu rrent intracranial hemorrhage secondary to congenital factor V deficiency w ith factor V inhibitor. Repeated transfusions of fresh-frozen plasma (FFP) and platelet concentrates, administrations of immunosuppressive therapy (pr ednisolone and cyclophosphamide), and intravenous immunoglobulin failed to normalize the coagulation profiles. Exchange transfusion followed-up by adm inistrations of activated prothrombin complex and transfusions of FFP and p latelet concentrates caused a temporary normalization of coagulation profil e, enabling an insertion of ventriculoperitoneal (VP) shunt for progressive hydrocephalus. The treatment was complicated by thrombosis of left brachia l artery and ischemia of left middle finger. The child finally died from an other episode of intracranial hemorrhage 10 days after insertion of the VP shunt.