Mh. Ornstein et Jh. Rand, AN ASSOCIATION BETWEEN REFRACTORY HELLP-SYNDROME AND ANTIPHOSPHOLIPIDANTIBODIES DURING PREGNANCY - A REPORT OF 2 CASES, Journal of rheumatology, 21(7), 1994, pp. 1360-1364
The HELLP syndrome (hemolysis, elevated liver enzymes and low platelet
s) is a thrombotic microangiopathic vasculopathy that presents in preg
nancy. HELLP syndrome usually resolves with delivery of the fetus with
out sequelae and is often managed successfully with conservative care.
To our knowledge, there has been no prior report of an association be
tween HELLP syndrome and elevated titers of antiphospholipid antibodie
s (aPL). We describe 2 patients who each presented with HELLP syndrome
that was refractory despite delivery of the fetus, corticosteroids, a
nd anticoagulation. Both patients had elevated levels of anticardiolip
in antibodies. Examination of skin and placental pathology revealed di
ffuse deposition of fibrin with small vessel thrombi, without evidence
of vasculitis. Coincident with plasmapheresis, HELLP resolved in both
patients. We conclude that there may be an association between the pr
esence of aPL and a protracted HELLP syndrome in pregnancy; this may b
e a new manifestation of the antiphospholipid syndrome. Plasmapheresis
appears to benefit these patients. Further investigation into this as
sociation is warranted.