Delayed Diagnosis of Hemophilia B Presenting with Hematemesis

A 36-year-old nonalcoholic gentleman presented with history of moderate-amount hematemesis. He received multiple blood transfusions. upper gastrointestinal endoscopy was done which showed no evidence of varices, but a tiny bleeding ulcer was seen in lower end of esophagus; sclerosing agent was injected at the base of ulcer. All the blood investigations were normal. A diagnosis of Dieulafoy’s lesion was made. After two months, he developed hemarthrosis after sustaining injury in right knee. Again investigations were done and coagulation profile revealed raised activated partial thromboplastin time and normal prothrombin time. Factor VIII and IX assay were sent in view of raised aPTT. Factor VIII and IX was 120% and 4% of normal respectively. Von Willebrand factor levels were within normal limits. Revised diagnosis of moderate hemophilia B was made.

[1]  R. Kanth,et al.  Outcomes in Dieulafoy’s Lesion: A 10-Year Clinical Review , 2015, Digestive Diseases and Sciences.

[2]  C. Kempton,et al.  Toward optimal therapy for inhibitors in hemophilia. , 2014, Hematology. American Society of Hematology. Education Program.

[3]  L. Azamfirei,et al.  Lethal gastric hemorrhage from a caliber-persistent artery of the antrum - a branch of the right gastric artery. , 2014, Hippokratia.

[4]  A. A. Abd Elrazek,et al.  Endoscopic management of Dieulafoy's lesion using Isoamyl-2-cyanoacrylate. , 2013, World journal of gastrointestinal endoscopy.

[5]  J. Mahlangu,et al.  Guidelines for the management of hemophilia , 2013, Haemophilia : the official journal of the World Federation of Hemophilia.

[6]  A. Rodríguez-Pérez,et al.  Prognostic factors for recurrence of gastrointestinal bleeding due to Dieulafoy's lesion. , 2012, World journal of gastroenterology.

[7]  N. Shah,et al.  Gastrointestinal Bleeding Due to a Dieulafoy Lesion in the Afferent Limb of a Billroth II Reconstruction. , 2011, Gastroenterology & hepatology.

[8]  C. Hyun Periampullary Dieulafoy's Lesion , 2005 .

[9]  K. Palmer,et al.  Non-variceal upper gastrointestinal haemorrhage: guidelines , 2002, Gut.

[10]  D. Monroe,et al.  A Cell-based Model of Hemostasis , 2001, Thrombosis and Haemostasis.

[11]  R. Enns,et al.  Endoscopic management of Dieulafoy lesions of the stomach: a case study of 26 patients. , 1998, Gastrointestinal endoscopy.

[12]  C. Gostout,et al.  Clinical features and endoscopic management of Dieulafoy's disease. , 1992, Gastrointestinal endoscopy.

[13]  V. Blanchette,et al.  Inherited bleeding disorders. , 1991, Bailliere's clinical haematology.