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An acute multiorgan thrombotic disorder associated with antiphospholipid antibodies; two ‘catastrophic’ cases
  1. CHRISTIAAN BOERMA,
  2. RONALD DERKSEN
  1. BART VAN DER WIEL
  1. Department of Rheumatology and Clinical Immunology
  2. Department of Pathology, University Hospital Utrecht, the Netherlands
  1. Dr R H W M Derksen, Departments of Rheumatology and Clinical Immunology (F02.126), University Hospital, PO Box 85500, 3508GA, Utrecht, the Netherlands.

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Over the past decade the antiphospholipid syndrome (APS) was defined by the presence of antiphospholipid antibodies (aPL) and clinical manifestations including thrombosis, recurrent fetal loss, thrombocytopenia, chorea, livedo reticularis, heart valve lesions, and renal involvement.1 Asherson et al first drew attention to a catastrophic variant of APS (CAPS) that is characterised by multiple widespread vascular occlusions, leading to multiple organ failure and often death.2 We describe two non-systemic lupus erythematosus (SLE) patients with a strikingly similar clinical presentation of CAPS and emphasise the difficulties in differentiating CAPS from other thrombotic angiopathies.

Case reports

CASE REPORT ONE

A 20 year old woman presented with transient hemichorea in 1993. Computed tomography of the brain was normal. From June 1995, episodes of hemichorea reoccurred together with severe frontal headaches. In November 1995 she was admitted to our hospital with rapid deterioration of vision and behavioural changes.

Physical examination showed subcoma, blood pressure 150/115 mm Hg, livedo reticularis, ischaemic skin ulcerations, and a systolic cardiac murmur. Fundoscopy showed arteriolar occlusions, bleeding, and exudates.

Laboratory findings included: platelet count 47 × 10 …

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