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HELLP Syndrome (5-Mark Answer)
Definition
HELLP is an acronym for Hemolysis, Elevated Liver enzymes, and Low Platelets. It is a severe, life-threatening variant/complication of preeclampsia, occurring in 5-10% of women with preeclamptic symptoms, and can arise even in the absence of hypertension or proteinuria (Textbook of Family Medicine, p. 497; Brenner and Rector's The Kidney).
Epidemiology
- Complicates 0.5-0.9% of all pregnancies and 10-20% of cases of severe preeclampsia/eclampsia
- Typically presents in the third trimester but up to 20% of cases develop postpartum
- Not all patients have hypertension or proteinuria at presentation
Pathophysiology
Considered a severe manifestation of preeclampsia driven by widespread endothelial dysfunction and microangiopathy:
- Hemolysis - microangiopathic hemolytic anemia from red cells fragmenting as they pass through damaged small vessels (schistocytes on smear)
- Elevated liver enzymes - hepatic ischemia from fibrin deposition in sinusoids, periportal/focal parenchymal necrosis, sometimes progressing to subcapsular hematoma or hepatic rupture
- Low platelets - consumption/aggregation at sites of endothelial injury
Clinical Features
- Right upper quadrant or epigastric pain (most common presenting symptom)
- Nausea, vomiting, malaise
- Headache, visual disturbance (overlap with preeclampsia)
- Hypertension and proteinuria in most, but not all, cases
- Signs of bleeding/bruising if severe thrombocytopenia
Diagnostic Criteria (Tennessee/Mississippi classification)
- Hemolysis: abnormal peripheral smear (schistocytes), elevated LDH, elevated indirect bilirubin
- Elevated liver enzymes: AST/ALT more than twice the upper limit of normal (typically < 500 IU/L)
- Low platelets: platelet count < 100,000/µL
Key Differentials
Must be distinguished from thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), and acute fatty liver of pregnancy (AFLP) - all can present with microangiopathic hemolysis and thrombocytopenia in pregnancy. Compared to HELLP, TTP/HUS show more severe renal failure and CNS symptoms with less prominent hypertension; AFLP shows higher bilirubin, coagulopathy, and elevated ammonia (Creasy & Resnik's Maternal-Fetal Medicine, Table 48.4).
Complications
Eclampsia (~6%), placental abruption (~10%), acute renal failure (~5%), DIC (~8%), pulmonary edema (~10%), and rarely hepatic hemorrhage/rupture (subcapsular hematoma in ~1% of HELLP cases).
Management
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Definitive treatment is prompt delivery - this is a medical/obstetric emergency
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IV magnesium sulfate for seizure prophylaxis (4 g loading dose over 15-20 min, then 2 g/hr infusion)
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Antihypertensives (e.g., IV hydralazine) if diastolic BP persistently > 110 mmHg
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Corticosteroids may be given to accelerate fetal lung maturity if preterm and maternal condition stable, delaying delivery briefly
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Supportive care: transfusion of blood products (platelets/FFP) if severe bleeding or very low platelet count before delivery/procedures
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Continue monitoring postpartum - lab abnormalities usually improve within 5 days after delivery but may worsen transiently first; watch for postpartum HELLP (up to 20% of cases present after delivery)
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Textbook of Family Medicine 9e, p. 497
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Creasy & Resnik's Maternal-Fetal Medicine, Table 48.4
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Brenner and Rector's The Kidney, 2-Volume Set