Von Willebrand disease with intestinal angiodysplasia

Von Willebrand disease with intestinal angiodysplasia
#00063815
Author: Dr. Tareq A. Saleh, consultant haematologist; Zahraa A. Thabit, MD; Laith Alrubaie, MD; Aqeel S. Mahmood, MD
Category: Hemostasis and coagulation > Bleeding disorders > Inherited Coagulation disorders > von Willebrand disease
Published Date: 10/25/2021

 A 65-year-old female who is a known case of Von Willebrand disease Type 1 with VWF Ag ˂ 10, her PT:16 sec. , APTT: 31 sec. with a platelet count of 225x109/l, presented with recurrent attacks of bleeding per rectum causing severe anemia, to which more than 16 units of packed RBC were transfused to her with other supportive measures including antifibrinolytic tranexamic acid and VWF.

Consultant gastroenterologist advised to do upper and lower endoscopy which revealed no abnormality, and the next step was Capsule endoscopy which revealed a segment of terminal jejunum and proximal ilium with angiodysplasia and massive fresh blood in the ilium.

A gastroenterologist surgeon advise to resect the involved segment of the small bowel with safe margin resection and the histopathologic examination revealed proliferation of variously sized irregular shaped vascular spaces, congested by red blood cells RBCs, overlined by benign intestinal epithelium with focal erosion and ulceration, these features were consistent (Intestinal Angiodysplasia / Vascular ectasia). Currently, after surgical resection, the patient with no more bleeding.