Spleen Rupture in Congenital Afibrinogenemia

ABSTRACT

Congenital afibrinogenemia is a rare disorder that refers to a congenital lack of production of fibrinogen, a key component of the hemostatic system. Bleeding manifestations of congenital afibrinogenemia vary in severity from mild to catastrophic. This is a case report of splenic rupture occurred in an eight-year-old boy with congenital afibrinogenemia. A conservative treatment was carried out with perfusion of cryoprecipitate and purified virally inactivated fibrinogen concentrates and splenectomy was avoided.