A case of inhibitor-positive acquired von Willebrand syndrome that developed during treatment for cancer of esophagogastric junction

Abstract

We experienced treating a patient diagnosed as having acquired von Willebrand syndrome (AvWS). During hospitalization, he required daily blood transfusion of red blood cells owing to exacerbation of anemia due to sustained massive bleeding from advanced cancer of the esophagogastric junction and deterioration of the general condition. The patient was a male in his forties. His APTT (28.3 s), which was normal at the time of admission, was markedly prolonged on the 28th day of hospitalization (> 180.0 s). The coagulation factor VIII activity was reduced to 9%; hence, acquired hemophilia A was suspected, but the factor VIII inhibitor was not detected (0.00 BU). The cross mixing test since incubation at 37°C for 2 hours showed an upwardly convex inhibitor pattern, inhibitor type AvWS was suspected. As a result of promptly providing the above examination results to the attending physician, steroid pulse therapy was performed without waiting for the diagnosis to be confirmed. Consequently, the bleeding rapidly decreased, and the condition of the whole body improved. A confirmatory diagnosis of AvWS was obtained from the subsequent examination results. It was a case that could be treated early from the examination of clinical symptoms, which can be carried out at any institution.