HELLP syndrome
HELLP syndrome is a thrombotic microangiopathy of pregnancy, a severe-feature variant of preeclampsia defined by hemolysis, transaminases at least twice the upper limit of normal, and a platelet count under 100,000/uL. Delivery is the only curative treatment; management pairs blood pressure control and magnesium sulfate seizure prophylaxis with prompt, gestational-age-guided delivery.
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What this article covers
- Overview
A thrombotic microangiopathy of pregnancy: HELLP syndrome (Hemolysis, Elevated Liver enzymes, Low Platelets) is a severe-feature variant of preeclampsia driven by vasospasm and endothelial injury…
- Epidemiology
Complicates 10% to 20% of severe preeclampsia: most cases escalate from established preeclampsia with severe features, though a minority present de novo.
- Risk factors
Previous HELLP syndrome: the strongest and most specific risk factor.
- Pathophysiology
Placental and endothelial origin: abnormal placental vascular development and generalized maternal endothelial dysfunction, the same process underlying preeclampsia, trigger diffuse vasospasm.
- Clinical features
Right upper quadrant or epigastric pain: the most common and specific presenting symptom, reflecting hepatic capsule distention; a pregnant or postpartum patient with new RUQ pain should have HELLP considered.
- Differential diagnosis
Acute fatty liver of pregnancy: similar nausea, vomiting, and abdominal pain, but distinguished by more severe hepatic failure: hypoglycemia, acute kidney injury, marked hyperbilirubinemia, ascites…
- Diagnosis
First, establish the diagnosis with the laboratory triad: hemolysis (schistocytes, elevated LDH, decreased haptoglobin, indirect hyperbilirubinemia), liver transaminases (AST/ALT) at least twice the…
- Management
First, admit to a high-risk obstetric or labor and delivery unit for continuous maternal and fetal monitoring: all patients with confirmed HELLP require inpatient management.
- Complications
Hepatic subcapsular hematoma with rupture: the most serious complication, causing severe hypotension from intraperitoneal hemorrhage.
- Prognosis
Delivery is curative, but recovery is not immediate: hepatic dysfunction and thrombocytopenia can worsen for up to 48 hours postpartum, and proteinuria can take months to resolve, before resolution typically occurs within days.
- Prevention
Low-dose aspirin starting at 12 weeks' gestation for patients at increased risk (prior preeclampsia or HELLP, chronic hypertension, multiple gestation, renal disease), continued through delivery to…
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- Preeclampsia
New-onset hypertension after 20 weeks of gestation with proteinuria or end-organ dysfunction, driven by abnormal placental spiral artery remodeling and widespread endothelial dysfunction. Eclampsia is preeclampsia complicated by seizures; magnesium sulfate treats and prevents seizures, and prompt delivery is the only definitive cure for both.
- Acute fatty liver of pregnancy
Acute fatty liver of pregnancy is a rare, life-threatening third-trimester disorder of microvesicular hepatic steatosis driven by a fetal-maternal fatty-acid oxidation mismatch (classically LCHAD deficiency); it is treated by immediate delivery, the only intervention that reverses the maternal disease.
- Thrombotic thrombocytopenic purpura (TTP)
Thrombotic thrombocytopenic purpura is a thrombotic microangiopathy caused by severe ADAMTS13 deficiency that lets ultralarge von Willebrand factor multimers drive platelet microthrombi; the key dyad of microangiopathic hemolytic anemia plus thrombocytopenia without another cause mandates the PLASMIC score, an ADAMTS13 activity and inhibitor level, and emergent plasma exchange started before results return.
- Hemolytic uremic syndrome
Hemolytic uremic syndrome is a thrombotic microangiopathy defined by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, most often triggered in children by Shiga toxin-producing Escherichia coli O157:H7 after bloody diarrhea. Diagnosis rests on schistocytes with a negative Coombs test plus a normal ADAMTS13 level to exclude TTP, and typical STEC-HUS is managed supportively while antibiotics and antimotility agents are avoided, whereas complement-mediated atypical HUS requires eculizumab.
- Disseminated intravascular coagulation
Disseminated intravascular coagulation is a secondary, systemic activation of coagulation and fibrinolysis, most often from sepsis, that simultaneously consumes platelets and clotting factors, causing microvascular thrombosis and bleeding. Diagnosis rests on a laboratory constellation, thrombocytopenia, prolonged PT and aPTT, low fibrinogen, and elevated D-dimer, in a predisposing clinical setting, and management centers on treating the underlying trigger while replacing blood components only for active or high-risk bleeding.
- Antiphospholipid syndrome
Antiphospholipid syndrome is an acquired autoimmune thrombophilia in which antiphospholipid antibodies cause recurrent venous and arterial thrombosis and pregnancy morbidity, paradoxically prolonging the aPTT in vitro while promoting clotting in vivo. Diagnosis requires a qualifying clinical event plus a laboratory antibody confirmed positive on two occasions at least 12 weeks apart, and thrombotic disease is treated with indefinite warfarin rather than a DOAC.