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Systemic lupus erythematosus complicated by acquired von Willebrand's syndrome

Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea

JH Lee

Division of Hematology, Department of Internal Medicine, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea

HS Chi

Department of Laboratory Medicine, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea

CK Lee

Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea

SS Nah

Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea

YG Kim

Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea

JS Oh

Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea

HB Moon

Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea

B Yoo

Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea byoo{at}amc.seoul.kr

Haematological abnormalities are common in systemic lupus erythematosus (SLE). In some cases of acquired von Willebrand syndrome (AvWS), von Willebrand disease (vWD) is associated with autoimmune or lymphoproliferative disorders. In this study, we describe a 36-year-old woman with SLE and AvWS. The patient was referred to our hospital because of easy bruisability and recurrent vaginal bleeding. She had no history of bleeding tendency and no family history of bleeding diathesis, but she had a history of recurrent arthralgia, photosensitivity and sicca symptoms. Tests for antinuclear, anti–double stranded DNA, anticardiolipin and anti–β2-glycoprotein I antibodies were all positive. Analysis of haemostatic parameters showed complete absence of von Willebrand factor ristocetin cofactor (vWF:Rco), von Willebrand antigen (vWF:Ag) and ristocetin-induced platelet aggregation (RIPA). Electrophoretic analysis of plasma showed a complete absence of high–molecular weight vWF multimer. The presence of antibody to vWF was detected by enzyme linked immunosorbent assay (ELISA). Treatment with corticosteroids improved SLE symptoms and corrected bleeding diasthesis. Also, the multimeric patterns of vWF became normalised and anti–vWF antibody disappeared. These findings indicated that this patient had SLE associated with AvWS, which was ameliorated by corticosteroid treatment.

Key Words: acquired von Willebrand syndrome • anti–phospholipid antibodies • systemic lupus erythematosus • von Willebrand factor

Lupus, Vol. 17, No. 9, 846-848 (2008)
DOI: 10.1177/0961203308089429


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