Hemolysis, Elevated Liver enzymes, Low Platelets — severe variant of preeclampsia.
Also known as: HELLP, HELLP syndrome, severe preeclampsia variant
Overview
A severe form of preeclampsia characterized by microangiopathic hemolytic anemia, hepatocellular dysfunction, and thrombocytopenia. Diagnosis does not require hypertension or proteinuria, although most patients have one or both.
Epidemiology
Occurs in 0.5-0.9% of all pregnancies and in 10-20% of women with severe preeclampsia. Most cases present between 28 and 36 weeks; up to 30% develop postpartum (usually within 48 hours).
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Abnormal placentation with defective spiral artery remodeling leads to placental ischemia and release of antiangiogenic factors (sFlt-1, soluble endoglin) and inflammatory mediators. Widespread endothelial dysfunction causes microvascular thrombosis, hepatic sinusoidal fibrin deposition (hepatocyte necrosis, elevated transaminases), and consumption of platelets. RBCs are sheared in microthrombi, producing schistocytes and intravascular hemolysis.
Clinical presentation
Symptoms
Right upper quadrant or epigastric pain (90%) — from hepatic capsular distension
Nausea, vomiting (50%)
Headache, visual disturbances, malaise
Up to 15% have neither hypertension nor proteinuria
Petechiae or bleeding with severe thrombocytopenia
Classic findings
Pregnant patient at 28-36 wk with epigastric/RUQ pain, schistocytes on smear, elevated AST/ALT, and platelets <100k.
Differential diagnosis
Acute fatty liver of pregnancy (AFLP) — Hypoglycemia, marked hyperammonemia, hyperbilirubinemia, coagulopathy out of proportion to platelet drop — Swansea criteria
Thrombotic thrombocytopenic purpura (TTP) — Severe thrombocytopenia, MAHA, neurologic symptoms, fever, renal involvement; ADAMTS13 <10%
Hemolytic uremic syndrome (HUS) / aHUS — AKI predominates; often postpartum; complement dysregulation
Viral hepatitis — Marked transaminase elevation, hepatitis serologies positive; no MAHA
Coagulation studies (PT, PTT, fibrinogen) to evaluate for DIC
Comprehensive metabolic panel, uric acid, urinalysis with protein quantification, creatinine
Imaging
Abdominal ultrasound or CT if subcapsular hematoma or hepatic rupture suspected
Fetal: ultrasound for growth, AFI, BPP; continuous monitoring
Diagnostic algorithm
Component
Threshold (Tennessee)
Hemolysis
LDH >=600 IU/L, schistocytes on smear, low haptoglobin, or indirect bilirubin >=1.2
Elevated Liver enzymes
AST >=70 IU/L (>=2x ULN)
Low Platelets
Platelet count <100,000/microL
Partial HELLP
Any 1-2 criteria — still warrants close monitoring
Tennessee diagnostic criteria for HELLP syndrome.
Treatment
First-line
Definitive treatment is delivery — timing depends on gestational age and maternal/fetal status
Maternal stabilization: magnesium sulfate for seizure prophylaxis (4-6 g IV load, then 1-2 g/h)
Blood pressure control if severe range (SBP >=160 or DBP >=110): IV labetalol, hydralazine, or oral nifedipine
Antenatal corticosteroids (betamethasone or dexamethasone) if 24 0/7 to 33 6/7 wk to accelerate lung maturity; dexamethasone has been studied for HELLP itself but does NOT improve maternal outcomes
Platelet transfusion only if active bleeding or platelets <20,000 (or <50,000 prior to cesarean)
Fresh frozen plasma / cryoprecipitate if DIC develops
Immediate delivery (>=34 wk, or any GA with maternal/fetal compromise, DIC, abruption, hepatic infarction or rupture)
Expectant management for 24-48 h ONLY between 24 0/7 and 33 6/7 wk in selected stable patients to complete steroids
Second-line / adjunct
Mode of delivery: cervical favorability and fetal condition guide induction vs cesarean — HELLP itself is not an indication for cesarean
Postpartum continued magnesium 24 h; close monitoring for 48-72 h since hemolysis and platelet nadir typically occur after delivery before improving
Complications
DIC, placental abruption, acute kidney injury
Subcapsular hepatic hematoma and hepatic rupture (rare, catastrophic)
Pulmonary edema, ARDS, cerebral hemorrhage
Eclampsia, maternal death (estimated 1-3%)
Fetal/neonatal: IUGR, preterm birth, perinatal death (7-20%)
PANCE pearls
RUQ or epigastric pain in late pregnancy is HELLP until proven otherwise — do not dismiss as reflux.
Thrombocytopenia in HELLP is usually <100,000; if platelets are <20,000 or hemolysis is profoundly out of proportion, consider TTP and check ADAMTS13.
Hepatic subcapsular hematoma can rupture catastrophically — avoid abdominal palpation under anesthesia and counsel against vigorous activity.
Recurrence risk in subsequent pregnancy is 3-25% for HELLP and up to 50% for any hypertensive disease of pregnancy.
HELLP can present or worsen postpartum; consider it in any postpartum woman with new RUQ pain, malaise, or thrombocytopenia.
References
ACOG PB 222 — ACOG Practice Bulletin 222: Gestational Hypertension and Preeclampsia (Obstet Gynecol 2020)
Sibai BM — Sibai BM. Diagnosis, controversies, and management of HELLP syndrome (Obstet Gynecol 2004)
HYPITAT — Koopmans et al., Lancet 2009 — induction vs expectant in late preterm hypertensive disease
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