Korean J Hematol.  2009 Dec;44(4):310-314. 10.5045/kjh.2009.44.4.310.

Acquired von Willebrand Syndrome Associated with Amyloidosis

• Pakk H
• Kim IH
• Park SH
• Lee DS
• Park SY
• Cho HI
• Kim HK

• KMID: 2252118
• DOI: http://doi.org/10.5045/kjh.2009.44.4.310

Abstract

Acquired von Willebrand syndrome (AvWS) is a relatively rare acquired bleeding disorder similar to inherited von Willebrand disease in terms of laboratory findings, and occurs without a personal or family history of bleeding. A 23-year-old man with no previous disease history and no family history of hemorrhagic diathesis was referred to our hospital because of recurrent epistaxis and intramuscular hematoma. He was diagnosed as having AvWS because of an almost complete absence of ristocetin cofactor activity (vWF : RCo) despite normal vWF antigen level. Furthermore, anti-vWF antibody was detected in his serum using home-brewed ELISA. Finally, the amyloid deposit was found in muscle biopsy. He was diagnosed with AvWS which is associated with amyloidosis. AvWS should be considered in patients with current bleeding diatheses and no past history of bleeding.

Keyword

von Willebrand disease; Autoantibodies; Amyloidosis

MeSH Terms

Amyloidosis
Autoantibodies
Biopsy
Disease Susceptibility
Enzyme-Linked Immunosorbent Assay
Epistaxis
Hematoma
Hemorrhage
Hemorrhagic Disorders
Humans
Muscles
Plaque, Amyloid
von Willebrand Diseases
von Willebrand Factor
Young Adult
Autoantibodies
von Willebrand Factor

Figure

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  Fig. 1. Microscopic findings of Extensor muscle. (A) Amyloid deposition showing Congo red staining (Congo red stain, ×200). (B) Apple-green birefringence in polarized light (Congo red stain, ×200).

Reference

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