Authors: Munther A. Khamashta, M.D., Maria Jose Cuadrado, M.D., Fedza Mujic, M.D., Nick A. Taub, M.Sc., Beverley J. Hunt, M.D., and Graham R.V. Hughes, M.D.
Published April 13, 1995
The risk of recurrent thrombosis in patients with the antiphospholipid-antibody syndrome is high. Long-term anticoagulation therapy in which the international normalized ratio is maintained at or above 3 is advisable in these patients.
The antiphospholipid-antibody syndrome is a thrombophilic disorder in which venous or arterial thrombosis, or both, may occur.1 The serologic markers of the syndrome are antiphospholipid antibodies (anticardiolipin antibodies, the lupus anticoagulant, or both).
The antiphospholipid-antibody syndrome often occurs in systemic lupus erythematosus, but the majority of patients with the syndrome do not meet the criteria for that disease.2 Thus, the combination of recurrent thrombosis and antiphospholipid antibodies in patients without features of lupus is called the primary antiphospholipid syndrome.3–5 Other important features of the syndrome are thrombocytopenia and recurrent spontaneous abortion.
Thrombosis, the main complication of the antiphospholipid-antibody syndrome, can affect vessels of all sizes; the consistent histopathological lesion is a bland thrombus without inflammation.6 The antiphospholipid antibodies persist for years, possibly for a lifetime. Thus, one of the key clinical questions is what causes the sudden development of thrombosis in these patients.7
Preventing thrombosis in the antiphospholipid-antibody syndrome is important, but there is no consensus about the duration and extent of prophylactic antithrombotic treatments.8 Controlled therapeutic trials have been difficult to perform9 because of the limited number of eligible patients available for study at a single center and the need for long-term follow-up. Therefore, the results of only a few small, retrospective studies of antithrombotic treatment of the antiphospholipid-antibody syndrome have been published.10–14 We assessed the efficacy of warfarin, low-dose aspirin, or both in preventing recurrent thrombosis in patients with the antiphospholipid-antibody syndrome seen in our unit since 1983.
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