SUPERStudy: HELLP Syndrome 

By usmlevideoss

Introduction: HELLP syndrome is a severe and life-threatening complication of pregnancy, typically considered a variant of preeclampsia. It is characterized by Hemolysis, Elevated Liver enzymes, and Low Platelets. This syndrome usually occurs in the third trimester but can also develop postpartum.

Components of HELLP Syndrome

1.Hemolysis: Breakdown of red blood cells (RBCs), evident by abnormal peripheral smear findings (e.g., schistocytes), elevated lactate dehydrogenase (LDH), and low haptoglobin levels.

2.Elevated Liver Enzymes: Liver dysfunction or ischemia caused by microvascular endothelial damage, leading to elevated aspartate aminotransferase (AST) and alanine aminotransferase (ALT).

3.Low Platelets (Thrombocytopenia): Platelet consumption and destruction, with platelet counts typically <100,000/µL.

Epidemiology

  • HELLP syndrome is considered a variant of severe preeclampsia, occurring in 0.2–0.6% of all pregnancies and in 10–20% of patients with severe preeclampsia.
  • Typically presents between 27-37 weeks of gestation but can occur postpartum. Seventy percent of patients with HELLP syndrome develop this problem between weeks 27 and 37 of pregnancy and 20% develop within 48 hours of delivery. 
  • A pregnant woman at >20 weeks of gestation or up to 7 days postpartum with abdominal pain should be evaluated for HELLP syndrome.

Pathophysiology

  • HELLP syndrome is caused by systemic endothelial dysfunction, platelet activation, and microvascular injury, which are hallmarks of preeclampsia.
  • The resulting microangiopathy leads to hemolysis, hepatic injury, and platelet consumption.
  • Liver damage is due to fibrin deposition in hepatic sinusoids, causing ischemia and hepatocellular necrosis.

Risk Factors

  • History of preeclampsia or eclampsia.
  • Previous HELLP syndrome in prior pregnancies.
  • Multiparity.
  • Advanced maternal age (>35 years).
  • Obesity or preexisting hypertension.
  • Clinical Features

General Symptoms:

  • Malaise and fatigue (common initial signs).
  • Nausea and vomiting (may mimic gastroenteritis).
  • Abdominal Pain: Severe epigastric or right upper quadrant (RUQ) pain due to liver swelling or infarction.
  • Hypertension and Proteinuria: Present in most cases but may not be as pronounced as in classic preeclampsia.
  • Other Signs:
  • Headache and visual changes (indicating severe disease).
  • Edema or signs of fluid overload.
  • Jaundice, oliguria, ascites 
  • Complications

1.Maternal Complications:

  • Disseminated intravascular coagulation (DIC).
  • Liver hematoma or rupture (rare but life-threatening).
  • Acute kidney injury.
  • Placental abruption.
  • Pulmonary edema.

2.Fetal Complications:

  • Preterm birth due to the need for early delivery.
  • Intrauterine growth restriction (IUGR).
  • Stillbirth in severe cases.

Diagnosis

Laboratory Findings:

  • Hemolysis: Hemolysis is the hallmark of the HELLP syndrome triad. 
  • Peripheral smear: Schistocytes or fragmented RBCs.
  • Elevated LDH (>600 U/L).
  • Reduced haptoglobin.
  • Elevated Liver Enzymes: AST/ALT >70 U/L.
  • Low Platelets: Platelet count <100,000/µL.
  • Imaging: Liver ultrasound or CT may be used to evaluate for hepatic hematoma or rupture in cases of severe RUQ pain. The classic hepatic lesion associated with HELLP syndrome is periportal or focal parenchymal necrosis.

Management: The initial management of HELLP syndrome is similar to that of severe preeclampsia or eclampsia: IV magnesium, blood pressure control, and hospital admission for stabilization.

1.Stabilization:

  • Blood Pressure Control: Administer antihypertensives such as labetalol or hydralazine to maintain systolic BP <160 mmHg and diastolic BP <110 mmHg.
  • Seizure Prophylaxis: Magnesium sulfate is used to prevent seizures.

2.Delivery:

  • Definitive Treatment: Delivery of the fetus and placenta is the only cure.
  • Timing of delivery depends on gestational age and maternal/fetal stability:
  • Immediate delivery if >34 weeks or if maternal/fetal condition deteriorates.
  • Conservative management may be attempted in a tertiary care setting if <34 weeks and stable.Corticosteroid administration can help delay delivery and improve fetal outcome in pregnancies <34 weeks of gestation.

3.Supportive Care:

  • Blood products (e.g., platelets, fresh frozen plasma) for coagulopathy or thrombocytopenia.
  • Intensive monitoring in a high-risk obstetric or ICU setting.
  • Plasma exchange therapy has been used in severe cases of HELLP syndrome and organ failure refractory to conservative management, and is generally started on the day of delivery until improvement in laboratory parameters.

4.Postpartum Care:

  • Monitor for resolution of symptoms and complications, as HELLP can worsen postpartum.

Prognosis

  • Maternal:
  • Good prognosis with early recognition and appropriate management, but complications can be life-threatening if untreated.
  • Recovery usually occurs within days to weeks after delivery.
  • Fetal:
  • Depends on gestational age and severity of the syndrome. Preterm infants are at higher risk of complications.

Summary: HELLP syndrome is a severe complication of pregnancy characterized by hemolysis, elevated liver enzymes, and low platelets, often associated with preeclampsia. Presents with nonspecific symptoms like malaise, nausea, and RUQ pain. Early diagnosis and stabilization followed by timely delivery are critical for maternal and fetal outcomes.

SUPERPoint: HELLP syndrome, a severe pregnancy complication characterized by hemolysis, elevated liver enzymes, and low platelets, requires prompt recognition, stabilization, and delivery to prevent life-threatening complications for both mother and fetus.

SUPERFormula: Pregnant woman presents with nonspecific symptoms such as fatigue, nausea, RUQ pain, and hypertension + laboratory results reveal Hemolysis (schistocytes, elevated LDH), Elevated Liver enzymes (AST/ALT >70 U/L), and Low Platelets (<100,000/µL) + Diagnosed via lab findings (e.g., hemolysis, liver dysfunction, thrombocytopenia) and imaging if liver rupture is suspected + Managed by stabilizing blood pressure, seizure prophylaxis with magnesium sulfate, and delivering the fetus and placenta = HELLP syndrome 

References: 

Tintinalli’s Emergency Medicine: A Comprehensive Study Guide, 9e

Judith E. Tintinalli, O. John Ma, Donald M. Yealy, Garth D. Meckler, J. Stephan Stapczynski, David M. Cline, Stephen H. Thomas

-CURRENT Diagnosis & Treatment: Gastroenterology, Hepatology, & Endoscopy, 3e

Norton J. Greenberger, Richard S. Blumberg, Robert Burakoff

-CURRENT Diagnosis & Treatment: Obstetrics & Gynecology, 12e

Alan H. DeCherney, Lauren Nathan, Neri Laufer, Ashley S. Roman