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Acquired von Willebrand syndrome (AvWS)


Authors: Petr Drenko 1;  Petra Přenosilová 1;  Alena Lavičková 2
Authors‘ workplace: I. interní klinika LF UK a FN Plzeň 1;  Ústav klinické biochemie a hematologie LF UK a FN Plzeň 2
Published in: Vnitř Lék 2022; 68(E-4): 16-19
Category: Case reports

Overview

Acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder caused by von Willebrand factor (vWF) dysfunction of various types and severities. Clinical manifestations and differences in the intensity of bleeding are then given by the diversity of vWF disorders. AvWS usually occurs in adult patients with a negative personal or family history of bleeding symptoms and is associated with underlying disease. In recent years, vWF dysfunction has been most commonly attributed to cardiovascular, autoimmune, lymphoproliferative or myeloproliferative comorbidities. The true prevalence of AvWS is unknown, since many cases may be clinically or laboratory silent and remain undiagnosed. Thus, a higher incidence can be expected in the above-mentioned specific patient population. We present a case report of a 90year-old patient with the very first episode of bleeding manifestation and newly diagnosed vWF dysfunction. By describing the case, we will try to present the diagnostic and treatment options of AvWS, including their pitfalls, and draw attention to situations in which to think about AvWS.

Keywords:

inhibitor – von Willebrand factor – aortic valve stenosis – bleeding disorder – non‑Hodgkin lymphoma


Sources

1.Kasatkar P, Ghosh K, Shetty S. Acquired von Willebrand syndrome: a rare disorder of heterogeneous etiology. J Postgrad Med. 2013 Apr‑Jun; 59(2):98-101. doi: 10.4103/0022- 3859.113816. PMID: 23793308.

2.Kumar S, Pruthi RK, Nichols WL. Acquired von Willebrand’s syndrome: a single institution experience. Am J Hematol. 2003 Apr;72(4):243-7. doi: 10.1002/ajh.10298. PMID: 12666134.

3.Franchini M, Mannucci PM. Acquired von Willebrand syndrome: focused for hematologists. Haematologica. 2020 Aug;105(8):2032-2037. doi: 10.3324/haematol.2020.255117. Epub 2020 Jun 18. PMID: 32554559.

4.Mohri H, Motomura S, Kanamori H, et al. Clinical significance of inhibitors in acquired von Willebrand syndrome. Blood. 1998 May 15;91(10):3623-9. Erratum in: Blood 1999 Jan 1;93(1):413. PMID: 9572997.

5.Handin RI, Martin V, Moloney WC. Antibody‑ induced von Willebrand’s disease: a newly defined inhibitor syndrome. Blood. 1976 Sep;48(3):393-405. PMID: 1085186.

6.Gan TE, Sawers RJ, Koutts J. Pathogenesis of antibody‑ induced acquired von Willebrand Syndrome. Am J Hematol. 1980;9(4):363-71. doi: 10.1002/ajh.2830090403. PMID: 6782864.

7.Dicke C, Schneppenheim S, Holstein K et al. Distinct mechanisms account for acquired von Willebrand syndrome in plasma cell dyscrasias. Ann Hematol. 2016 May;95(6):945- 57. doi: 10.1007/s00277-016-2650-x. Epub 2016 Apr 4. PMID: 27040683.

8.Mohri H, Tanabe J, Ohtsuka M et al. Acquired von Willebrand disease associated with multiple myeloma; characterization of an inhibitor to von Willebrand factor. Blood Coagul Fibrinolysis. 1995 Sep;6(6):561-6. doi: 10.1097/00001721-199509000- 00010. PMID: 7578899.

9.Alattar ML, Ciccone M, Gaballa MR et al. Bleeding diathesis associated with acquired von Willebrand Syndrome in three patients with chronic lymphocytic leukemia. Leuk Lymphoma. 2015;56(12):3452-4. doi: 10.3109/10428194.2015.1037757. Epub 2015 May 26. PMID: 25926060.

10.Otto CM, Prendergast B. Aortic‑valve stenosis--from patients at risk to severe valve obstruction. N Engl J Med. 2014 Aug 21;371(8):744-56. doi: 10.1056/NEJMra1313875. PMID: 25140960.

11.Yoshida K, Tobe S, Kawata M, Yamaguchi M. Acquired and reversible von Willebrand disease with high shear stress aortic valve stenosis. Ann Thorac Surg. 2006 Feb;81(2):490-4. doi: 10.1016/j.athoracsur.2005. 07. 074. PMID: 16427837.

12.Horiuchi H, Doman T, Kokame K et al. Acquired von Willebrand Syndrome Associated with Cardiovascular Diseases. J Atheroscler Thromb. 2019 Apr 1;26(4):303-314. doi: 10.5551/ jat.RV17031. Epub 2019 Mar 12. PMID: 30867356.

13.Loscalzo J. From clinical observation to mechanism--Heyde’s syndrome. N Engl J Med. 2012 Nov 15;367(20):1954-6. doi: 10.1056/NEJMcibr1205363. PMID: 23150964.

14.Richard C, Cuadrado MA, Prieto M et al. Acquired von Willebrand disease in multiple myeloma secondary to absorption of von Willebrand factor by plasma cells. Am J Hematol. 1990 Oct;35(2):114-7. doi: 10.1002/ajh.2830350210. PMID: 2205095.

15.Mital A, Prejzner W, Bieniaszewska M et al. Prevalence of acquired von Willebrand syndrome during essential thrombocythemia: a retrospective analysis of 170 consecutive patients. Pol Arch Med Wewn. 2015;125(12):914-20. doi: 10.20452/pamw.3211. Epub 2015 Dec 10. PMID: 26658493.

16.Lee A, Sinclair G, Valentine K et al. Acquired von Willebrand syndrome: von Willebrand factor propeptide to von Willebrand factor antigen ratio predicts remission status. Blood. 2014 Jul 31;124(5): e1-3. doi: 10.1182/blood-2014-02-557132. Epub 2014 Jun 20. PMID: 24951428.

17.Charlebois J, Rivard GÉ, St‑Louis J. Management of acquired von Willebrand syndrome. Transfus Apher Sci. 2018 Dec;57(6):721-723. doi: 10.1016/j.transci.2018. 10. 012. Epub 2018 Oct 30. PMID: 30401518.

18.Pushin DM, Salikhova TY, Zlobina KE et al. Platelet activation via dynamic conformational changes of von Willebrand factor under shear. PLoS One. 2020 Jun 11;15(6):e0234501. doi: 10.1371/journal.pone.0234501. PMID: 32525962.

19.Nascimbene A, Neelamegham S, Frazier OH et al. Acquired von Willebrand syndrome associated with left ventricular assist device. Blood. 2016 Jun 23;127(25):3133-41. doi: 10.1182/ blood-2015-10-636480. Epub 2016 May 3. PMID: 27143258.

20.Mohri H. Acquired von Willebrand syndrome: features and management. Am J Hematol. 2006 Aug;81(8):616-23. doi: 10.1002/ajh.20455. PMID: 16823821.

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Diabetology Endocrinology Internal medicine
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