Unexpected Bleeding in the Operating Room: The Role of Acquired von Willebrand Disease

摘要: Acquired von Willebrand disease (AvWD) is a rare bleeding disorder that occurs in association with variety of underlying disorders and can lead to unforeseen surgical patients. Cardiovascular as well malignant immunological diseases may be associated AvWD, several pathophysiological mechanisms have been proposed. factor (vWF) plasma glycoprotein mediates platelet adhesion subendothelial collagen causes aggregation under high shear stress. Additionally, vWF acts specific carrier for coagulation VIII (FVIII) the plasma. AvWD results from reduced rate synthesis, an increased removal, or final generation lower-molecular-weight, less active subunits multimers. In contrast inherited patients, who are characterized by lifelong episodes, patients present sudden onset symptoms, which induce acute episodes during critical procedures. Typically, no family history found. The clinical visualization similar hereditary form mucocutaneous perioperative bleeding, ranging mild severe bleeding. Laboratory evaluation mainly based on measurement activity antigens multimeric analysis vWF. A therapeutic approaches used depending mechanisms. Treatment options control hemorrhages prevent complications surgery include desmopressin, FVIII/vWF concentrates, high-dose IV immunoglobulins, exchange. Because half-life doses concentrates administered at frequent intervals necessary episodes. cases unresponsiveness standard therapy, recombinant activated VIIa alternative option. However, most effective therapy resolution disease. review, we focus current understanding outlining disorders, mechanisms, possible treatment options.