Considered to be a severe form of preeclampsia (sometimes called “atypical preeclampsia”) characterized by hemolysis (H), also expressed as microangiopathic hemolytic anemia, 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.
Early detection and aggressive management with a combination of IV magnesium sulfate, IV dexamethasone, control of blood pressure to prevent or minimize 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.
Although delivery is the only cure, serious manifestations continue into the immediate postpartum period.
A severe form of preeclampsia characterized by hemolysis (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/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 (AST, ALT) should be elevated >70 IU/L, total serum lactate dehydrogenase (LDH) should be 600 IU/L or more, and the platelet count should be <100,000/mm^3. Hemolysis may be indicated by elevated total bilirubin (>1.2 mg/dL), LDH and AST elevations, characteristic findings (schistocytes) on a peripheral blood smear, hematuria, worsening anemia, and a low serum haptoglobin.
Professor of Obstetrics and Gynecology
Vice Chair for Research and Academic Development
Director of the Divisions of Maternal-Fetal Medicine and Research
University of Mississippi Medical Center
JNM Jr is a maternal-fetal medicine (MFM) consultant to Mississippi Blue Cross Blue Shield and the Mississippi Perinatal Quality Collaborative. He is a member of several American Congress of Obstetricians and Gynecologists (ACOG) committees including the Global Operations Advisory Group, Compensation, and the Telehealth WorkGroup, and a member of the International Federation of Gynecology and Obstetrics (FIGO) Preterm Birth Working Group. He is a teacher, lecturer, and speaker about HELLP syndrome for the District VII ACOG, ACOG ACM Postgraduate Course on HELLP and Hypertensive Disorders of Pregnancy, and similar topics in Peru/Mexico/Philippines. JNM Jr is also an author of a number of references cited in this monograph
Dr James N. Martin Jr would like to gratefully acknowledge Dr Marium G. Holland and Dr Alex C. Vidaeff, the previous contributors to this monograph. MGH declares that she has no competing interests. ACV is an author of a number of references cited in this monograph.
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.
Professor of Obstetrics
University of Dublin
Coombe Women and Infants University Hospital
DM declares that she has no competing interests.
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