An uncommon cause of hematemesis in children: factor XI deficiency

Congenital factor XI deficiency, also called hemophilia C, is a rare coagulation disorder that is particularly common in Ashkenazi Jews. Individuals with factor XI deficiency may or may not have a mild bleeding tendency, which is typically provoked by surgery or trauma. We report the case of a 5.5-year-old girl who presented with abdominal pain, vomiting, and hematemesis. Biological tests showed a prolonged activated partial thromboplastin time (aPTT) of 113 s (control=29 s) caused by a constitutional factor Xl deficiency (2.7%). The progression was spontaneously favorable.