Antiphospholipid syndrome(APS) is a well-established clinical syndrome characterized by recurrent arterial or venous thromboembolic events, recurrent fetal loss, livedo reticularis, which need not be simultaneously present, and/or
thrombocytopenia,
together with high titers of sustained IgG anticardiolipin antibody(aCL) or lupus anticoagulant(LA). Although systemic lupus erythematosus(SLE) and APS may coexist. a high proportion of patients manifesting the APS do not suffer from classical
lupus or
other connective tissue diseases. These patients has recently been defined as having a "primary" antiphospholipid syndrome.
We experienced seven cases of primary antiphospholipid syndromes. Venous thrombosis was in four including three cases of Budd-Chiari syndrome. Arterial thrombosis was in two, one of which is aortic thrombi and distal arterial embolic events, and
the
other multiple cerebral infarction. Microangiopathy revealed in two cases. Hemolytic anemia was in four. In all cases, LA were positive and five of them showed aPL positivity and two cases were VDRL false positive. And four cases showed
thrombocytopenia.
Four cases showed response to warfarin therapy. In two cases, prednisolone was administered and in one plasmapheresis was done. There has been no evidence of disease. Recurrence until now on.
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