Acquired Von Willebrand Syndrome – What You Need to Know, How I treat von Willebrand disease | Blood | American …
von Willebrand Disease Guidelines | CDC, Acquired Von Willebrand Syndrome – What You Need to Know, The acquired von Willebrand syndrome (AVWS) is a bleeding disorder that is frequently unrecognized or is misdiagnosed as von Willebrand disease . AVWS is characterized by structural or functional defects of von Willebrand factor (VWF) that are secondary to autoimmune, lymphoproliferative or myeloproliferative, malignant, cardiovascular, or other …
Because of the heterogeneous mechanisms of AVWS, more than one therapeutic approach is often required to treat acute bleeds and for prophylaxis during invasive procedures; the treatment options include, but are not limited to, desmopressin, VWF-containing concentrates, intravenous immunoglobulin, plasmapheresis or recombinant factor VIIa.
Strategies to prevent and/or treat bleeding episodes should also be in place, including the use of VWF-containing factor VIII concentrates, desmopressin and tranexamic acid. Treatment success will depend largely on the underlying pathogenesis of the disorder. Copyright © 2012 Elsevier Ltd. All rights reserved.
8/6/2009 · The risk assessment and diagnosis of von Willebrand disease (VWD), which is probably the most frequent inherited bleeding disorder, 1-3 remain difficult tasks because of the wide spectrum of clinical severity and genetic penetrance of the disease. Furthermore, because the present classification system does not accurately predict a person’s response to desmopressin (1-deamino-8-D-arginine …
