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Case study of treatment responses using Privigen and Biostate with Monoclonal gammopathy of undetermined significance (MGUS) & Acquired von Willebrand syndrome (AvWS)

Journal: Science and Education (Vol.3, No. 5)

Publication Date:

Authors : ;

Page : 142-147

Keywords : Acquired vonWillebrand syndrome; vonWillebrand factor; Biostate; Inhibitor; Monoclonal gammopathy of undetermined significance;

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Abstract

Initially described in 1968, Acquired von Willebrand syndrome (AvWS) is a rare disorder that mimics hereditary von Willebrand disease (VWD) and can be associated with life-threatening bleeding.(1) Lack of a family history of bleeding, the diagnosis of AvWS is usually based on the laboratory findings. Platelet function assay (PFA), VW antigen, VW activity, VW collagen binding activity and Factor VIII (FVIII) are used to help diagnose both acquired and inherited VWD.(2) Mechanisms of reduced VWF activity in AvWS can be either immune or non-immune. The incidence of AvWS in some populations can be greater. These include; myeloproliferative neoplasms, left ventricular assist device (LVAD), extracorporeal membrane oxygenation, autoimmune disorders, congenital cardiac anomalies or aortic stenosis, lymphoproliferative disorders, and Wilms tumour.(3)

Last modified: 2022-06-13 02:27:18