Considered to be a severe form of pre-eclampsia (sometimes called 'atypical pre-eclampsia') characterised by haemolysis (H), also expressed as microangiopathic haemolytic anaemia, elevated liver enzymes (EL), and low platelets (LP).
Usually occurs antepartum between 27 and 37 weeks' gestation; 15% to 30% of cases present initially postpartum. Significant diagnostic and therapeutic challenge because only 80% to 85% of affected patients present typically with hypertension and proteinuria.
Should be considered in any pregnant patient presenting in the second half of gestation or immediately postpartum with significant new-onset epigastric/upper abdominal pain until proven otherwise.
Associated with progressive and sometimes rapid maternal and fetal deterioration.
Although definitions and strict cut-off values for diagnosis are often arbitrary, adherence to widely accepted diagnostic criteria facilitates scientific reporting and communication.
Early detection and aggressive management with a combination of intravenous magnesium sulfate, intravenous dexamethasone, control of blood pressure to prevent or minimise severe systolic hypertension, replacement of blood products as needed, and timely delivery of the fetus and placenta, appear to be the best and safest ways to arrest disease progression and reduce adverse outcomes. Maternal outcomes are improved considerably with this management; perinatal outcome depends predominantly upon the gestational age when delivery occurs. A critical step is the early initiation of potent glucocorticoids as soon as the diagnosis of HELLP syndrome is made, so that severe maternal morbidity (stroke, liver haematoma/infarction/rupture, acute pancreatitis) and maternal mortality can be avoided.
Although delivery is the only cure, serious manifestations of the disease continue into the immediate postpartum period.
A severe form of pre-eclampsia characterised by haemolysis (H), elevated liver enzymes (EL), and low platelets (LP) in a pregnant or puerperal patient (usually within 7 days of delivery). An affected patient often displays hypertension and proteinuria (80% to 85%), epigastric/right upper quadrant (RUQ) pain (40% to 100%), nausea, vomiting, headache, and malaise. Some patients may present without these signs/symptoms when first evaluated for unexplained thrombocytopenia. The diagnosis of HELLP syndrome should be considered in any pregnant patient presenting in the second half of gestation or immediately postpartum with significant new-onset epigastric/RUQ pain until proven otherwise. To meet diagnostic criteria, liver transaminases (aspartate aminotransferase [AST], alanine aminotransferase) should be elevated >70 IU/L, or twice the upper limit of normal concentration, and the platelet count should be <100 x 10⁹/L (<100,000/microlitre). Haemolysis may be indicated by elevated total bilirubin (>1.2 mg/dL [>20.5 micromol/L]), LDH and AST elevations, and characteristic findings (schistocytes) on a peripheral blood smear, haematuria, worsening anaemia, and a low serum haptoglobin.
History and exam
James N. Martin, Jr, MD, FACOG, FAGOS, FAHA, F(H)RCOG
Professor Emeritus of Obstetrics, Gynecology, and Maternal-Fetal Medicine
University of Mississippi Medical Center
JNM Jr is an obstetrician-gynaecologist and maternal-fetal medicine consultant to BlueCross & BlueShield of Mississippi and the Mississippi Perinatal Quality Collaborative. He is co-director of the Mississippi Maternal Mortality Review Committee. He serves on the Global Operations Advisory Group of the American College of Obstetricians and Gynecologists (ACOG), and in 2018-9 he chaired the ACOG Presidential Task Force on Pregnancy and Heart Disease. JNM Jr lectures, teaches, researches, and speaks nationally and internationally on the subjects of hypertensive complications of pregnancy, including HELLP syndrome. He is also the lead author and editor of 'The 2015 compendium for HELLP syndrome: from bench to bedside' (Nova Biomedical Publishers). JNM Jr is an author of several references cited in this topic. He has cited his own papers in this topic.
Dr James N. Martin Jr would like to gratefully acknowledge Dr Marium G. Holland and Dr Alex C. Vidaeff, the previous contributors to this topic.
MGH declares that she has no competing interests. ACV is an author of a number of references cited in this topic.
Jerome Yankowitz, MD
Professor and Director
Division of Maternal-Fetal Medicine
Department of Obstetrics and Gynecology
University of Iowa Hospitals and Clinics
JY declares that he has no competing interests.
Deirdre Murphy, MD, MRCOG
Professor of Obstetrics
University of Dublin
Coombe Women and Infants University Hospital
DM declares that she has no competing interests.
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