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

Authors

  • Sunny Jamati Waikato DHB

Keywords:

Acquired von Willebrand syndrome, von Willebrand factor, Biostate, Inhibitor, Monoclonal gammopathy of undetermined significance

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)

References

Franchini M, Mannucci PM. Acquired von Willebrand syndrome: focused for haematologists. Haematologica [Internet]. 2020 [cited 2021 July 1]; 105(8). Available from: https://haematologica.org/issue/view/368

Michiels JJ, Michiels JJ, Budde U. Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and management. Best Pract Res Clin Haematol. [Internet] 2001 [Cited 2021 July 14]; 14(2):401-436. PubMed|https://doi.org/10.1053/beha.2001.0141|Google Scholar

Franchini M, Lippi G. Recent acquisitions in acquired and congenital von Willebrand disorders. Clin Chim Acta. [Internet]. 2007 [Cited 2021 June 1] ; 377(1-2):62 69. PubMed|https://doi.org/10.1016/j.cca.2006.10.017|Google Scholar

Mohri H. Acquired von Willebrand syndrome: features and management. Am J Hematol [Internet]. 2006 [Cited 2021 June 25]; 81 (8): 616-23. Available from: https://pubmed.ncbi.nlm.nih.gov/16823821/

Mehta R, Athar M , Girgis, S , Hassan A, Becker RC. Acquired Von Willebrand Syndrome (AVWS) in cardiovascular disease: a state of the art review for clinicians. J Thromb Thrombolysis [Internet]. 2019 [Cited 2021 June 4]; 48(1): 14-26. Available from: https://pubmed.ncbi.nlm.nih.gov/31004311/

Hanley D , Arkel YS, Lynch J, Kamiyama M. Acquired von Willebrand's syndrome in association with a lupus-like anticoagulant corrected by intravenous immunoglobulin . Am J Hematol [Internet]. 1994 [cited 2021 June 27]; 46(2): 141-6. Available from: https://pubmed.ncbi.nlm.nih.gov/8172182/

Howard C, Lin T, Cunningham M , Lipe B. IgGG Kappa Monoclonal Gammopathy of Undetermined Significance (MGUS) Presenting as Acquired Type III Von Willebrand Syndrome. Blood Coagul Fibrinolysis. [Interent]. 2014 [cited 2021 July 9]; 25(6): 631-633

Tiede A, Rand JH, Budde U, Ganser A, Federic AB. How I treat the acquired von Willebrand syndrome. Blood. [Internet]. 2011 [cited 2021 May 29]; 117 (25): 6777-6785. Available from: https://ashpublications.org/blood/article/117/25/6777/24352/How-I-treat-the-acquired-von-Willebrand-syndrome

Johns Hopkins Medicine. Monoclonal Gammopathies. [Internet]. USA: The Johns Hopkins Hospital; 2021. Available from: https://www.hopkinsmedicine.org/health/conditions-and-diseases/monoclonal-gammopathies

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Published

2022-05-31

How to Cite

Jamati, S. (2022). Case study of treatment responses using Privigen and Biostate with Monoclonal gammopathy of undetermined significance (MGUS) & Acquired von Willebrand syndrome (AvWS). Science and Education, 3(5), 142-147. Retrieved from https://openscience.uz/index.php/sciedu/article/view/3340

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Section

Natural Sciences