HELLP! A cry for laboratory assistance: a comprehensive review of the HELLP syndrome highlighting the role of the laboratory.

Abstract

The HELLP syndrome is a dangerously severe form of preeclampsia associated with multiorgan system damage and occurs in 0.2-0.6% of all pregnancies. It usually presents with abdominal pain, often in the setting of preeclampsia. In most cases, HELLP is initiated by inadequate placental vessel development with subsequent placental ischemia, leading to the release of circulating vasoconstrictors. These powerful vasoconstrictors include thromboxane A2, angiotensin, prostaglandin F2, and endothelin-1. The ischemic placenta also produces fewer vasodilators, such as prostacyclin, prostaglandin, E2, and nitric oxide. The ensuing imbalance in vasoactive substances causes intense systemic vasospasm and multiorgan endothelial damage. Multiple genetic, coagulation, and immunologic disorders also appear to contribute to the endothelial damage. Fibrin and platelets are then deposited on the endothelial surfaces leading to the hemolytic anemia, elevated liver enzymes, and low platelets of the HELLP syndrome. The most reliable laboratory tests for the diagnosis of HELLP are a complete blood count with peripheral smear, lactate dehydrogenase, serum transaminases, and urinalysis. Supportive tests include serum haptoglobin, D-dimer fragment levels, lactate dehydrogenase isoenzymes, total bilirubin, prothrombin times, and activated partial thromboplastin times. Lactate dehydrogenase and the platelet count are the two best tests to monitor the course of the disease. Prompt delivery is the treatment of choice. The intensity of the HELLP syndrome peaks 24 hours after delivery. Extended atypical HELLP has been successfully treated with plasma exchange. The clinical laboratory professional plays an important role in the diagnosis, follow-up, and treatment of patients with the HELLP syndrome.