Mallory-weiss Syndrome as the First Bleeding Manifestation in a 34-year-old Male Patient with Severe Hemophilia B.

The typical symptoms in patients with severe hemophilia are recurrent spontaneous hemarthrosis, and gastrointestinal (Gl) bleeding. A 34-year-old male presented to our hospital with hematemesis. Laboratory findings showed prolongation of the prothrombin time PT (13.7sec) and activated partial thromboplastin time aPTT (71.3sec). Factor IX，XII levels were decreased to 1% and 44% respectively. The bleeding focus was found to be Mallory-Weiss (M-W) syndrome at the esophagogastric junction. A diagnosis of M-W syndrome with severe hemophilia B was made. Endoscopic hemostasis was achieved using band ligation, and transfusion of fresh-frozen plasma was done for the correction of coagulopathy. After treatment, there were no further signs of bleeding. One month later, the patient continued to have no signs of hemarthrosis, hematemesis, or bleeding tendency. Factor IX and XII levels at 1-month follow-up were decreased to 1% and 46% respectively. Here, we present a rare case of severe Type B hemophilia presenting with hematemesis due to Mallory-Weiss syndrome as the first bleeding manifestation in the patient’s life, and successful hemostasis using endoscopic procedure and FFP supplementation.