Recurrent Spontaneous Splenic Rupture an Unusual Complication of Congenital Factor XIII Deficiency

Abstract

Factor XIII deficiency is a rare bleeding disorder with autosomal recessive inheritance. We report a 23 year old ladywith recurrent spontaneoussplenic rupture with factor XIII deficiency. She was diagnosed to have congenital factor XIII deficiency in neonatal period when she presented with prolonged bleeding from the umbilical stump. She had an episode of intracranial hemorrhage at three years of age after which she received regular cryoprecipitate transfusion till 20 years of age. She had a history of spontaneous splenic laceration with perisplenic hematoma about one year back which was managed conservatively. Presently she was admitted with abdominal pain and imaging of abdomen revealed splenic laceration with perisplenic hematoma. She was managed conservatively with Cryoprecipitate transfusion and was discharged in stable condition on monthly cryoprecipitate transfusion as prophylaxis.