Acquired von Willebrand syndrome (AvWS)
Authors:
Petr Drenko 1; Petra Přenosilová 1; Alena Lavičková 2
Authors place of work:
I. interní klinika LF UK a FN Plzeň
1; Ústav klinické biochemie a hematologie LF UK a FN Plzeň
2
Published in the journal:
Vnitř Lék 2022; 68(E-4): 16-19
Category:
Case reports
Summary
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
Zdroje
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Štítky
Diabetology Endocrinology Internal medicineČlánok vyšiel v časopise
Internal Medicine
2022 Číslo E-4
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