Acquired Deficiency of Von Willebrand Factor (wWF) Associated with Intestinal Angiodysplasia

Abstract

Acquired von Willebrand's Disease (AVWD) is a rare bleeding disorder that has been associated with angiodysplasia. We present a 79-year-old gentleman that presented with gradually progressive generalized weakness and easy fatigability with history of recurrent self-limiting epistaxis. He is known to have chronic lymphocytic leukemia (CLL), multiple myeloma and acquired Von Willebrand factor deficiency (Ristocetin cofactor of 25%) for which he received multiple chemotherapy cycles, last was two weeks before admission. Examination was evident of pallor with no signs of active epistaxis. Labs showed Hb of 4.8 g/dl, WBC of 8,400 and Platelets of 150,000. Ferritin was 4.64. He received 3 packed RBC's with improvement of his hemoglobin to 9.1 g/dl. Given inactive bleeding he was discharged for outpatient upper and lower endoscopy. Two weeks later he presented with same complain and was found to have Hb of 6.4. He denies any further epistaxis, neither hematemesis, melena or hematochezia. Colonoscopy was done showing diffuse diverticulosis and hyperplastic polyp. Enteroscopy was done showing bleeding angioectasia in the second portion of the duodenum. The bleeding site was coagulated multiple times using a heater probe set at 20J and resulted in cessation of the bleeding. Given the multiple coagulations further advancement of the enteroscope was deferred. Video capsule endoscopy was done with no further small bowel lesions. Hb stayed stable overnight and he was discharged with close clinic follow up Acquired von Willebrand syndrome (AVWS) is a rare and probably underestimated bleeding disorder which mimics most of the clinical symptoms and laboratory features of hereditary von Willebrand disease (VWD) in patients devoid of both personal and family history of bleeding diathesis. The diagnosis and treatment of recurrent GI bleeding in acquired VWD remains challenging and is associated with significant morbidity.1955_A Figure 1. Bleeding angiodysplasia in the second part of the duodenum1955_B Figure 2. Bleeding angiodysplasia in the second part of the duodenum1955_C Figure 3. Successful control of bleeding with coagulation using a heater probe