J. Musial et al., EVANSS-SYNDROME WITH POSITIVE LUPUS ANTICOAGULANT AND ANTIPHOSPHOLIPID-PROTEIN ANTIBODIES, Clinical and applied thrombosis/hemostasis, 3(2), 1997, pp. 96-98
We report a case of a 34-year-oId Caucasian man with Evans's syndrome,
first manifested in childhood as autoimmune thrombocytopenia. Besides
antierythrocyte antibodies and platelet-associated immunoglobulins, t
his patient's serum showed also the presence of antiphospholipid-prote
in antibodies detected by coagulometric (lupus anticoagulant) as well
as immunoenzymatic methods (anticardiolipin, antiprothrombin, and anti
-beta(2)-glycoprotein I antibodies). Over 25 years of clinical observa
tion the patient never experienced any thromboembolic events. We sugge
st that in the pathogenesis of Evans's syndrome and related disorders
a major role is played by an autoimmune reaction with a predilection t
o various cell phospholipid-containing membrane structures, possibly d
amaged by a yet unknown primary insult. The significance of these vari
ous antiphospholipid-protein antibodies as predictors of thromboemboli
c complications in such patients remains, however, to be established.