Maternal morbidity and mortality in pregnant/postpartum women with suspected HELLP syndrome identifiable as probable thrombotic thrombocytopenic purpura or atypical hemolytic uremic syndrome by high LDH to AST ratio

Abstract

Objective: To describe findings in 8 women initially diagnosed as presumptive HELLP Syndrome, eventually confirmed as TTP/aHUS as distinguished by elevated calculated LDH:AST ratio > 22:1.

Methods: All medicolegal files of patients evaluated between 1986 and 2015 with presumptive HELLP syndrome but later determined to have TTP/aHUS had LDH:AST ratios evaluated throughout care.

Results: Fifty-eight pregnant/postpartum women presented with a diagnosis of presumptive HELLP syndrome. In the final analysis, 8 women had TTP/aHUS characterized by severe thrombocytopenia (<20 000/μl) at admission, rare epigastric pain, and the consistent demonstration of a very high calculated total LDH to AST ratio. This calculation greatly exceeded 22:1 with TTP/aHUS (mean = 32:1) versus 2:1 with HELLP and could be consistently demonstrated throughout care. Six of 8 women with TTP/aHUS died.

Conclusion: Correctly distinguishing between HELLP syndrome versus an imitator disorder continues to challenge obstetric specialists. This medicolegal data supplements prior findings supporting the concept of the LDH:AST ratio as a useful screening tool for clinicians to differentiate TTP/aHUS apart from HELLP syndrome in order to facilitate earlier hematology consultation, patient referral to tertiary care and emergent hemotherapy for these mothers.

Keywords: HELLP syndrome; LDH to AST ratio (LDH:AST ratio); TTP/aHUS; imitators of HELLP; maternal morbidity and mortality.