아밀로이드증과 동반된 후천 폰빌레브란트 증후군 = Acquired von Willebrand Syndrome Associated with Amyloidosis

다국어 초록 (Multilingual 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.

참고문헌 (Reference)

1 Krebs M, "Massive postoperative intramuscular bleeding in acquired von Willebrand’s disease" 81 : 394-396, 2002

2 Scrobohaci ML, "Expression of GpIb on plasma cells in a patient with monoclonal IgG and acquired von Willebrand disease" 84 : 471-475, 1993

3 Collins P, "Epidemiology and general guidelines of the management of acquired haemophilia and von Willebrand syndrome" 14 : 49-55, 2008

4 Fricke WA, "Comparison of inhibitory and binding characteristics of an antibody causing acquired von Willebrand syndrome: an assay for von Willebrand factor binding by antibody" 66 : 562-569, 1985

5 Denis CV, "Clearance of von Willebrand factor" 99 : 271-278, 2008

6 Lenting PJ, "Clearance mechanisms of von Willebrand factor and factor VIII" 5 : 1353-1360, 2007

7 Federici AB, "Carbohydrate moiety of von Willebrand factor is not necessary for maintaining multimeric structure and ristocetin cofactor activity but protects from proteolytic degradation" 74 : 2049-2055, 1984

8 Hoshino Y, "Bleeding Tendency Caused by the Deposit of Amyloid Substance in the Perivascular Region" 32 : 879-881, 1993

9 Tefferi A, "Acquired von Willebrand’s disease due to aberrant expression of platelet glycoprotein Ib by marginal zone lymphoma cells" 96 : 850-853, 1997

10 Franchini M, "Acquired von Willebrand syndrome: an update" 82 : 368-375, 2007

11 Van Genderen PJ, "Acquired von Willebrand disease caused by an autoantibody selectively inhibiting the binding of von Willebrand factor to collagen" 84 : 3378-3384, 1994

12 Federici AB, "Acquired von Willebrand Syndrome: data from an international registry" 84 : 345-349, 2000

13 Van Genderen PJ, "Acquired von Willebrand Disease" 11 : 319-330, 1998

14 Niiya M, "Acquired type 3-like von Willebrand syndrome preceded full-blown systemic lupus erythematosus" 13 : 361-365, 2002

15 Siaka C, "A new ELISA assay for diagnosis of acquired von Willebrand Syndrome" 9 : 303-308, 2003