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 intravenous magnesium sulfate, intravenous 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. 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 hematoma/infarction/rupture, acute pancreatitis) and maternal mortality can be avoided.
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/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 <100,000/mm³. 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.
History and exam
Key diagnostic factors
- brisk tendon reflexes
Other diagnostic factors
- right upper quadrant/epigastric pain and tenderness
- generalized malaise
- visual disturbances
- white ethnicity
- maternal age >35 years
- chronic hypertension
- diabetes mellitus
- autoimmune disorders
- multiple gestation
- abnormal placentation (e.g., molar pregnancy)
- previous pregnancy with preeclampsia with/without HELLP syndrome
1st investigations to order
- CBC with differential including platelets
- peripheral blood smear
- liver transaminases
- bilirubin level
- total serum LDH level
- uric acid level
- urinalysis and protein-to-creatinine ratio
- fibrinogen level
- fetal ultrasound
Investigations to consider
- serum glucose level
- serum creatinine and electrolyte levels
- antithrombin level
- haptoglobin level
- maternal upper abdomen ultrasound, CT, or MRI
- lactate dehydrogenase (LDH)-to-aspartate aminotransferase (AST) ratio
all patients (confirmed HELLP)
- Acute fatty liver of pregnancy (AFLP)
- Thrombotic thrombocytopenic purpura (TTP)
- Atypical hemolytic uremic syndrome (aHUS)
- ACOG practice bulletin no. 222: gestational hypertension and preeclampsia
- Diagnosis, evaluation, and management of the hypertensive disorders of pregnancy
Preeclampsia: what is it?
Preeclampsia: what treatments work?More Patient leaflets
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