Reproductive · Hypertensive Disorders of Pregnancy
The facts most likely to be tested
HELLP syndrome is a severe variant of preeclampsia characterized by hemolysis, elevated liver enzymes, and low platelets.
Patients classically present with right upper quadrant (RUQ) pain or epigastric pain due to Glisson's capsule distension from hepatic subcapsular hematoma.
Laboratory findings include microangiopathic hemolytic anemia evidenced by schistocytes on peripheral blood smear and elevated LDH.
Thrombocytopenia is defined by a platelet count < 100,000/µL, which is a critical diagnostic criterion.
Definitive management for HELLP syndrome is immediate delivery of the fetus regardless of gestational age if the patient is unstable or beyond 34 weeks.
Magnesium sulfate is indicated for seizure prophylaxis in all patients with HELLP syndrome.
Maternal complications include disseminated intravascular coagulation (DIC), placental abruption, and hepatic rupture.
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A 32-year-old G2P1 woman at 33 weeks gestation presents to the emergency department with a 2-day history of severe epigastric pain and nausea. Physical examination reveals blood pressure of 165/105 mmHg and RUQ tenderness on palpation. Laboratory studies demonstrate a platelet count of 85,000/µL, AST of 180 U/L, ALT of 160 U/L, and the presence of schistocytes on peripheral smear. The patient is currently stable, and fetal heart rate monitoring shows a reassuring category I tracing.
What is the most appropriate next step in management?
Immediate delivery after stabilization with magnesium sulfate and antihypertensives.
The patient meets the diagnostic criteria for HELLP syndrome (hemolysis, elevated liver enzymes, low platelets). Because the patient is at 33 weeks gestation with severe features, immediate delivery is the definitive treatment to prevent maternal and fetal morbidity.
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Etiology / Epidemiology
Severe variant of preeclampsia occurring in the 3rd trimester or postpartum. Risk factors include nulliparity and advanced maternal age.
Clinical Manifestations
Classic RUQ pain, nausea, and vomiting. Hemolysis, elevated liver enzymes, and low platelets are the diagnostic triad.
Diagnosis
Diagnosis requires platelets <100,000/µL, AST/ALT >70 IU/L, and LDH >600 IU/L.
Treatment
Immediate delivery is the definitive treatment. Administer magnesium sulfate for seizure prophylaxis.
Prognosis
High risk of abruptio placentae and hepatic rupture. Maternal mortality is <1% with prompt management.
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Epidemiology & Etiology
HELLP is a life-threatening obstetric complication typically presenting in the 3rd trimester. It is strongly associated with preeclampsia with severe features, though it can occur without hypertension. Patients often present with systemic symptoms mimicking gastroenteritis or cholecystitis.
Pertinent Anatomy
The liver is the primary organ of concern due to periportal necrosis and fibrin deposition. This leads to Glisson's capsule distension, causing the characteristic severe epigastric or RUQ pain.
Pathophysiology
The process begins with abnormal placentation leading to systemic endothelial dysfunction. This triggers a microangiopathic hemolytic process, platelet consumption, and hepatic microvascular injury. The resulting vasospasm and ischemia drive the rapid clinical deterioration.
Clinical Manifestations
Patients present with RUQ pain, malaise, and nausea. Red flags include visual disturbances, severe headache, and epigastric tenderness. If left untreated, patients may progress to seizures or disseminated intravascular coagulation.
Diagnosis
The Tennessee Classification System is the standard for diagnosis. Key thresholds include platelets <100,000/µL, AST/ALT >70 IU/L, and LDH >600 IU/L. A peripheral blood smear will show schistocytes due to microangiopathic hemolysis.
Treatment
The only definitive treatment is delivery regardless of gestational age. Magnesium sulfate is the first-line agent for seizure prophylaxis. Corticosteroids (e.g., dexamethasone) may be used to stabilize platelet counts temporarily, but do not replace delivery. Avoid delayed delivery in the presence of fetal distress or maternal instability.
Prognosis
Major complications include abruptio placentae, pulmonary edema, and hepatic rupture. Patients require intensive monitoring for acute renal failure and postpartum hemorrhage. Recurrence risk in future pregnancies is approximately 20%.
Differential Diagnosis
Acute fatty liver of pregnancy: hypoglycemia and coagulopathy are more prominent
Thrombotic thrombocytopenic purpura: fever and neurological symptoms dominate
Hemolytic uremic syndrome: renal failure is the primary feature
Cholecystitis: lacks the systemic hemolysis and thrombocytopenia
Viral hepatitis: AST/ALT levels are typically much higher (>500 IU/L)