Hereditary/Acquired Bleeding Disorders

At the ISTH 2021 Congress, held virtually from July 17–21, a team of researchers from Milan reported on an investigation of response to treatment of gastrointestinal (GI) bleeding in 293 patients with von Willebrand disease (VWD) evaluated at the A. Bianchi Bonomi Hemophilia and Thrombosis Center in Milan between 2009 and 2019 (abstract OC 13.2). GI bleeding, they note, is a characteristic feature of VWD that is generally seen in older patients, is often related to arteriovenous malformations, and in most cases requires blood transfusion and hospitalization. The investigators reported that 16% of the patients had at least 1 episode of GI bleeding during their life (occult in 4) and in 13 of them angiodysplastic lesions were documented at least once. Recurrent bleeding was present in 57% of patients, blood transfusions were required in 66%, and local GI lesions were identified in 38%. Long-term prophylaxis or rescue therapy (surgery, atorvastatin, octreotide, lenalidomide) was required in 19% of patients (n=9); 8 of the 9 patients had loss of HMWM (high-molecular-weight multimers), which have been linked to arteriovenous malformations.