We describe the case of a young woman with a known history of thrombocytopenia, who developed respiratory and circulatory failure thought to be caused by sepsis. She subsequently was shown to have high titres of anticardiolipin antibodies as well as lupus anticoagulant. Serological tests for various connective tissue diseases were negative. Multiple cerebral ischaemic lesions led to a poor outcome. This case highlights the potential difficulties in differentiating catastrophic anti-phospholipid syndrome from disseminated intravascular coagulation.
IngramS.B., GoodnightS.H.Jr., BennettR.M.An unusual syndrome of a devastating noninflammatory vasculopathy associated with anticardiolipin antibodies: a report of two cases.Arthritis Rheum1987; 30: 1167–1172.
2.
GreismanS.G., ThayaparanR-S, GodwinT.A., LockshinM.D.Occlusive vasculopathy in systemic lupus erythematosus. Association with anticardiolipin antibody.Arch Intern Med1991; 151: 389–392.
3.
AshersonR.A., PietteJ.C.The catastrophic antiphosopholipid syndrome in 1996: Acute multi-organ failure associated with antiphospholipid antbodies. A review of 31 patients.Lupus1996; 5: 414–417.
BelmontH.M., AbramsonS.B., LieJ.T.Pathology and patho-genesis of vascular injury in systemic lupus erythematosus.Arthritis Rheum1996; 39: 9–22.
6.
OostingJ.D., DerksenR.H., BobbinkI.W.Anti-phospholipid antibodies directed against a combination of phospholipids with prothrombin, protein C, or protein S: an explanation for their pathogenic mechanism.Blood1993; 81: 2618–2625.
7.
ArnoutJ.The pathogenesis of the antiphospholipid syndrome: a hypothesis based on parallelisms with heparin-induced thrombocytopenia.Thromb Haemost1996; 75: 536–541.
8.
KitchensC.S.Thrombotic storm: when thrombosis begets thrombosis.Am J Med1998; 104: 381–385.
9.
DosekunA.K., PollakV.E., Glas-GreenwaltP.Ancrod in systemic lupus erythematosus with thrombosis. Clinical and fibrinolytic effects.Arch Intern Med1984; 144: 37–42.