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Catastrophic antiphospholipid syndrome mimicking a malignant pancreatic tumour – a case report

Department of Internal Medicine and Vascular Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands

BAJ Bastiaansen

Department of Gastroenterology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands

AK Stroobants

Department of Clinical Chemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands

EJ van den Dool

Department of Clinical Chemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands

MM Idu

Department of Vascular Surgery, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands

M Levi

Department of Internal Medicine and Vascular Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands

ESG Stroes

Department of Internal Medicine and Vascular Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands

Abstract

The catastrophic antiphospholipid syndrome is characterised by rapid onset thromboses, often resistant to conventional anticoagulant treatment, and resulting in life threatening multiple organ dysfunction. The diagnosis of catastrophic antiphospholipid syndrome may be difficult, predominantly due to its frequently atypical presentation. We report a case of a 35-year-old female who presented with a pancreatic tumour and extensive thromboses. Following a storm of ischemic events due to thrombotic occlusions in spite of therapeutic heparin dose, the suspicion of catastrophic antiphospholipid syndrome emerged. The patient was successfully treated with anticoagulants, immunuglobulins, plasmapheresis and rituximab. The present report shows that the use of the diluted Russell’s viper venom time can be helpful in providing additional information on the lupus anticoagulans antibody status, allowing careful monitoring of lupus anticoagulans conversion and hence response to therapy.

Key Words: catastrophic antiphospholipid syndrome (CAPS) • lupus anticoagulans antibodies • treatment

Lupus, Vol. 17, No. 6, 586-590 (2008)
DOI: 10.1177/0961203307087406


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