Hemolysis, Elevated Liver Enzymes, and Low Platelet Count Syndrome With Severe Thrombocytopenia and Severe ADAMTS13 Activity Deficiency.

Abstract

Differentiating preeclampsia with hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome from thrombotic thrombocytopenic purpura (TTP) can present a diagnostic dilemma. We report the case of a 34-year-old woman, G1P0, with monochorionic diamniotic twins who presented with new-onset blurry vision, hypertension, and a platelet count of 4×109/L. After a multidisciplinary discussion, a diagnosis of atypical HELLP syndrome was made, despite overlapping features concerning for TTP. Her platelet count and ADAMTS13 activity testing showed appropriate recovery after delivery, without plasma exchange therapy, supporting the diagnosis of HELLP syndrome. Hemolysis, elevated liver enzymes, and low platelet count syndrome may present with severe thrombocytopenia and severe ADAMTS13 activity deficiency in ranges otherwise known to be more common with TTP.