A Rare Cause of Acquired Von Willebrand Syndrome Resulting in Gastrointestinal Bleeding

Abstract

Von willebrand disease (vWD) is the most common congenital bleeding disorder but acquired von willebrand syndrome (AVWS) is rare. Pathogenesis of AWVS is complex and not fully understood. We report the case of a patient with chronic gastrointestinal bleeding and AVWS from a myeloproliferative neoplasm. A 59-year-old female with a two year history of gastrointestinal bleeding, angioectasia and recurrent hospitalizations for transfusion-dependent anemia presented with a 3 day history of melena. Two weeks prior to presentation, she had an extended upper endoscopy which revealed angioectasias in the jejunum that were cauterized with gold probe. Medications included omeprazole daily and ferrous sulfate three times daily. On arrival, vital signs were stable. Physical exam was remarkable for heart murmur and melena. Abnormal labs included hemoglobin 6.4g/dL and partial prothrombin time 38 seconds. Deep enteroscopy showed a single actively bleeding angioectasia in the proximal jejunum treated successfully with argon plasma coagulation (Fig 1). Heart murmur prompted a von willebrand factor (vWF) panel and echocardiogram. Echocardiogram was normal but ristocetin cofactor, vWF activity, vWF antigen, and factor 8 levels were low. vWF multimers were barely detectable. Further workup included a bone marrow aspiration that confirmed prefibrotic myelofibrosis myeloproliferative neoplasm, AVWS was diagnosed. She was discharged home in stable condition and has been closely monitored by hematology for her myeloproliferative neoplasm. She has not required any further blood transfusions. AVWS is due to a deficiency in the quality or quantity of vWF. The true incidence is unknown since many patients are asymptomatic and therefore undiagnosed. AVWS is characterized by a negative personal and family history of previous bleeding. It is typically seen in the elderly. Angioectasias have been reported in 2-4% of patients with vWD. Management relies on therapy of the underlying process. This case highlights an interesting presentation of a patient with recurrent gastrointestinal bleeding from AVWS secondary to a myeloproliferative neoplasm. Gastroenterologists should be cognizant of this phenomenon as early diagnosis is crucial for appropriate management.Figure: Actively bleeding angiodysplastic lesion in the proximal jejunum.