Evans's Syndrome with Positive Lupus Anticoagulant and Antiphospholipid-Protein Antibodies

Abstract

We report a case of a 34-year-old Caucasian man with Evans's syndrome, first manifested in childhood as autoimmune thrombocytopenia. Besides anti-erythrocyte antibodies and platelet-associated immunoglobulins, this patient's serum showed also the presence of antiphospholipid-protein antibodies detected by coagulometric (lupus anticoagulant) as well as immunoenzymatic methods (anticardiolipin, antiprothrombin, and anti-β2-glycoprotein I antibodies). Over 25 years of clinical observation the patient never experienced any thromboembolic events. We suggest that in the pathogenesis of Evans's syndrome and related disorders a major role is played by an autoimmune reaction with a predilection to various cell phospholipid-containing membrane structures, possibly damaged by a yet unknown primary insult. The significance of these various antiphospholipid-protein antibodies as predictors of thromboembolic complications in such patients remains, however, to be established. Key Words: Evans's syndrome-Lupus anticoagulant-Antiphospholipid-protein antibodies.