Acquired von Willebrand syndrome: Five cases report and literature review

Abstract

Acquired von Willebrand syndrome is a rare bleeding disorder with laboratory findings similar to those of inherited von Willebrand disease. Principal factors distinguishing acquired von Willebrand syndrome from the latter condition include lack of prior bleeding disorders, diagnosis at older age, negative family history and association with underlying conditions.

Methods: Retrospective, monocentric descriptive case series of acquired von Willebrand syndrome diagnosed between 2010 and 2020. Diagnostic criteria included a recent history of bleeding, a negative family history and a presence of underlying disorders.

Results: Five men were diagnosed with acquired von Willebrand syndrome. All of them presented with recent mucocutaneous bleeding. The biological phenotype was a type 2 von Willebrand disease in all cases, with decreased VWF:RCo/VWF:Ag and VWF:CB/VWF:Ag ratios (<0.7). Lymphoproliferative, autoimmune and cardiovascular diseases were the main underlying conditions identified. Screening for an anti-von Willebrand factor inhibitor was positive in two patients. Four patients received treatment for the underlying disorder. High-dose intravenous immunoglobulins were the most frequent treatment administrated. Improvement of plasma von Willebrand factor levels was observed in four cases.

Conclusion: Acquired von Willebrand syndrome is a rare but potentially serious disease. The diagnostic should be suspected in adults with unusual mucocutaneous bleeding associated with lymphoproliferative, myeloproliferative, autoimmune and cardiovascular disorders.

Keywords: Acquired von Willebrand syndrome; Bleeding disorders; Hemostasis.