Ricerc@Sapienza

Hemolysis, Elevated Liver Enzymes, and Low Platelet (HELLP) syndrome is a disease specific to pregnancy and is thought to be secondary to abnormal placentation in the first trimester. Recently, HELLP syndrome has shown to be a disease associated with the alternative pathway of complement (APC). Diseases of the APC especially atypical hemolytic uremic syndrome (aHUS) are highly morbid and phenotypically similar to HELLP syndrome as they both have hemolysis, renal injury, altered mental status, liver enzyme elevation, and thrombocytopenia. Instead of an origin of placental pathology, HELLP syndrome could be augmented by complement augmentation and thus a variant of aHUS. In fact, 20% of women diagnosed with aHUS have a pregnancy trigger, and similar germline mutations of the APC in HELLP are prevalent in both diseases. HELLP syndrome's current treatment involves blood pressure control, magnesium sulfate for seizure prophylaxis, fetal monitoring, and ultimately delivery after completion of betamethasone for fetal lung maturity. This often leads to iatrogenic preterm birth. With new data that HELLP syndrome is a disease of the APC, exploring complement inhibition with eculizumab (ECU) is warranted as it is the standard treatment in aHUS. If HELLP syndrome is truly a disease of the APC, ECU should be effective in halting, slowing, and/or reversing disease process. Treatment could hopefully lead to a decrease in maternal morbidity and safe latency of pregnancy beyond 48 hours after betamethasone completion.