Successful perinatal management of a woman with congenital factor XIII deficiency using recombinant factor XIII: A case report and literature review

Abstract

AbstractFactor XIII deficiency is an extremely rare autosomal recessive genetic disorder, occurring in 1 of 3–5 million people, and is associated with perinatal complications, such as habitual abortion and prolonged bleeding. Although plasma‐derived factor XIII (Fibrogamin®) carries a risk of infection and contains very low concentrated forms of factor XIII (FXIII) used for a pregnant woman with congenital coagulation factor XIII deficiency, recombinant factor XIII (rFXIII, Novo Thirteen®; Tretten®, Novo Nordisk, Bagsværd, Denmark), which has no risk of infection and is highly concentrated, has emerged as a novel formulation. Herein, we report the first case of a Japanese pregnant woman with congenital coagulation factor XIII deficiency successfully managed by rFXIII. She had a good perinatal course without pregnancy‐related complications and transfusion through the perinatal period.