HELLP Syndrome: Pathophysiology and Current Therapies

Abstract

HELLP syndrome is a disorder associated with serious maternal morbidity and mortality. Distinguishing HELLP from other pregnancy related disorders is often challenging and may result in delay of treatment. Differential diagnoses include acute fatty liver of pregnancy, thrombotic thrombocytopenic purpura, antiphospholipid syndrome, and hemolytic uremic syndrome, and are reviewed in this chapter. While there is not any current treatment for HELLP, the mainstay of treatment involves maternal stabilization and timely delivery. Various treatment strategies have been attempted to help decrease the morbidity and mortality of HELLP, including the maternal use of corticosteroids. The authors review the studies and controversies surrounding the maternal use of corticosteroids, plasma exchange, and low molecular weight heparin for the treatment of HELLP, as well as the role of the complement system in HELLP. Further large, well-designed, randomized controlled trials are needed to address the role corticosteroids may play in the treatment of women with HELLP and to help improve maternal and fetal outcomes.

Acute Kidney Injury in Pregnancies Complicated With Preeclampsia or HELLP Syndrome

“…Diagnosis is typically made using the following criteria: lactate dehydrogenase elevated to 600 IU/L or more, aspartate aminotransferase and alanine aminotransferase elevated more than twice the upper limit of normal, and platelet count <100,000 × 10 9 /L (20). HELLP syndrome has a similar pathophysiology to preeclampsia as women with HELLP syndrome have abnormal placentation, immune cell activation and endothelial dysfunction (50). HELLP syndrome is also associated with increased rates of maternal morbidity and mortality (50,54).…”

“…among postpartum hospitalizations occurred among women with ARF of any etiology (9). Evidence suggests that hypertensive disorders of pregnancy are an important contributor to the burden of AKI in pregnancy, in particular preeclampsia and HELLP syndrome which are already associated with increased incidences of maternal and perinatal morbidity and mortality (35,(49)(50)(51). PR-AKI also has a significant impact on both maternal and fetal morbidity and mortality (26,47).…”

“…HELLP syndrome has a similar pathophysiology to preeclampsia as women with HELLP syndrome have abnormal placentation, immune cell activation and endothelial dysfunction (50). HELLP syndrome is also associated with increased rates of maternal morbidity and mortality (50,54). While the mechanisms leading to preeclampsia and HELLP syndrome have not been fully elucidated, the clinical findings of edema, hypertension, proteinuria, and renal insufficiency can be explained by the changes in renal physiology that are characteristic of the disease.…”

“…However, there are other, less common, disorders that are also characterized by end-organ injury, thrombocytopenia and microangiopathic hemolytic anemia that defines TMA. Other TMA disorders in pregnancy include atypical hemolytic uremic syndrome (aHUS) and thrombotic thrombocytopenic purpura (TTP) (50,55). Unlike HELLP syndrome and preeclampsia the presence of the placenta is not needed for the immediate causation of these disorders which has likely contributed to the advancement in therapies for treating aHUS and TTP.…”

Acute kidney injury during pregnancy leads to increased sFlt-1 and sEng and decreased renal T regulatory cells in pregnant rats with HELLP syndrome

“…HELLP syndrome is considered to be a rare but potentially deadly complication of pregnancy, affecting 10–20% of women with preeclampsia [ 7 , 8 ]. HELLP syndrome when complicated with AKI increases maternal mortality and morbidity compared to patients affected by HELLP alone [ 9 , 10 ].…”