Can severe preeclampsia progress to Hemolysis, Elevated Liver enzymes, and Low Platelet count (HELLP) syndrome?

Can Severe Preeclampsia Progress to HELLP Syndrome?

Yes, severe preeclampsia can absolutely progress to HELLP syndrome, which represents the most severe end of the preeclampsia spectrum and requires close monitoring with expeditious delivery once diagnosed. 1

Understanding the Relationship

HELLP syndrome should be considered a manifestation of severe preeclampsia rather than a separate disorder. 1, 2 The American Association for the Study of Liver Diseases explicitly states that "overlapping clinical features of preeclampsia are present in many patients with HELLP syndrome," and the International Society for the Study of Hypertension in Pregnancy recommends that women with features of HELLP syndrome should be considered to have preeclampsia so that all other features will be sought and addressed. 1

Preeclampsia exists on a spectrum, with HELLP syndrome representing the most severe manifestation characterized by hemolysis, elevated liver enzymes, and low platelet count. 1, 2

Clinical Progression and Risk

Epidemiology of Progression

• Approximately 25% of women with severe preeclampsia will develop features consistent with HELLP syndrome 3
• HELLP syndrome affects 0.2%-0.6% of all pregnancies, with most cases occurring between weeks 27-37 1
• Critically, 20% of HELLP cases occur within 48 hours of delivery, and 30% occur or worsen postpartum, emphasizing the need for continued vigilance after birth 1, 4

Warning Signs of Progression

The American College of Obstetricians and Gynecologists identifies specific clinical and laboratory markers that signal progression from severe preeclampsia to HELLP syndrome: 5, 2

• Clinical symptoms: Epigastric or right upper quadrant pain (present in 65% of HELLP cases), nausea/vomiting (35%), headache and malaise (30%), and jaundice (up to 40%) 1, 3
• Laboratory deterioration: Progressive thrombocytopenia (platelets <100,000/mm³), rising liver enzymes (AST/ALT), elevated LDH >600 IU/L, and evidence of hemolysis with schistocytes 1, 5
• The degree of thrombocytopenia correlates directly with the severity of liver dysfunction and predicts adverse maternal outcomes 1, 5

Critical Management Implications

Monitoring Requirements

Women with severe preeclampsia require close monitoring for development of HELLP syndrome, as recommended by the American Association for the Study of Liver Diseases: 1

• Serial laboratory monitoring including complete blood count with platelets, liver function tests (AST, ALT, LDH), evaluation for hemolysis, and coagulation studies 2, 4
• Laboratory values should be checked every 6-12 hours when HELLP is suspected or diagnosed 4
• Continuous monitoring of blood pressure, urinary output, and clinical symptoms 5, 2

Delivery Timing

Prompt delivery is indicated once HELLP syndrome develops, after maternal stabilization and correction of coagulopathy. 1, 2

• For preeclampsia without severe features, delivery is recommended at 37 weeks 1
• For preeclampsia with severe features, delivery at 34 weeks is recommended 1
• When HELLP syndrome is diagnosed, expeditious delivery is recommended after maternal stabilization, regardless of gestational age 1

Maternal Mortality Risk

The progression to HELLP syndrome significantly increases maternal risk, with maternal mortality reported at 3.4% when intervention is delayed. 1, 6 This underscores the critical importance of early recognition and prompt delivery.

Common Pitfalls to Avoid

Delaying delivery while waiting for laboratory values to normalize is dangerous—HELLP syndrome only resolves after delivery. 5 The British Journal of Anaesthesia emphasizes that maternal mortality increases significantly with delayed intervention. 1, 5

Do not use regional anesthesia (epidural/spinal) when platelets are <100,000/mm³ due to risk of epidural hematoma. 5, 2 Platelet transfusion is mandatory before surgical intervention when platelets are <50,000/mm³. 5, 2

Corticosteroids should not be administered to improve maternal HELLP outcomes—they are ineffective for maternal disease, though they remain indicated for fetal lung maturity before 35 weeks. 5, 2

Pathophysiology

The progression involves inadequate placental perfusion leading to endothelial dysfunction and multisystem involvement. 1 The placenta releases nitric oxide, prostaglandins, and endothelin, inducing platelet aggregation and arterial hypertension. 1 Fibrin deposition within hepatic sinusoids results in sinusoidal obstruction and hepatic ischemia, which can progress to subcapsular hematomas, parenchymal hemorrhage, and potentially fatal hepatic rupture. 1