How I treat gastrointestinal bleeding in congenital and acquired von Willebrand disease

Gastro-intestinal (GI) bleeding is distinctive of severe von Willebrand disease (VWD). It arises generally in older patients, requiring in most cases blood transfusion and hospitalization. The presence of arteriovenous malformations is often described when endoscopic examinations are performed. Patients with congenital type 3, 2A and 2B are those most frequently affected by this symptom, possibly due to the loss of high-molecular-weight multimers (HMWM) of von Willebrand factor (VWF). GI bleeding can also occur in patients affected by the acquired von Willebrand syndrome (AVWS). Endoscopic examination of the GI tract is necessary to exclude ulcers and polyps or cancer as possible causes of GI bleeding. In case of congenital VWD, prophylaxis with VWF/(factor VIII, FVIII) concentrates is generally started after the GI bleeding events, but this therapy is not always successful. Iron supplementation must be prescribed to avoid chronic iron-deficiency. Possible rescue therapies (high dose statins, octreotide, thalidomide, lenalinomide and tamoxifen) were described in few case-reports and series, while surgery may be necessary in emergency or in case of failure of the medical treatment to stop bleeding. In this article we present a few clinical cases that highlight the clinical challenges of these patients and possible strategies for their long-term management.