RESUMO
Identification of acquired von Willebrand syndrome (AVWS) was key to treating a patient with chronic gastrointestinal (GI) bleeding due to angiodysplasia. After exhausting endoscopic and pharmacological options, the patient was successfully treated with rituximab. A 78-year-old man developed chronic GI bleeding from caecal and jejunal angiodysplasia. Red cell transfusion was required weekly despite argon plasma coagulation. A diagnosis of AVWS was made from analysis of clotting factors. Therapies including von Willebrand factor concentrate, thalidomide and tranexamic acid were unsuccessful. With failed endoscopic therapy and no viable surgical option, the patient was given intravenous immunoglobulins (IVIGs). Haemoglobin remained stable from this point. The impact on the patient and hospital of attending for IVIG every 3 weeks necessitated consideration to longer-term therapy. After a single course of rituximab, no further blood products, IVIG or rituximab were required. This case is the first to describe the use of rituximab in AVWS-associated angiodysplasia.
