Updated: 3/5/2022

HELLP Syndrome

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  • Snapshot
    • A 35-year-old G4P3 at 35 weeks gestation presents to the emergency room with nausea, vomiting, and right upper quadrant pain. She has a history of pre-eclampsia in her previous pregnancy. On physical exam, she is noted to be dry mucous membranes and mild periorbital edema. Her blood pressure is 180/90 mmHg. Laboratory testing shows elevated liver enzymes, increased lactate dehydrogenase, and decreased platelets. She is started on steroids, intravenous magnesium sulfate, and prepped for immediate delivery.
  • Introduction
    • Overview
      • HELLP syndrome is named for its characteristic findings: Hemolysis, Elevated Liver enzymes, and Low Platelets
      • it is a life-threatening complication of pregnancy
        • some consider this a severe form or preeclampsia/eclampsia, though this is controversial
    • Associated conditions
      • preeclampsia and eclampsia
  • Epidemiology
    • Incidence
      • 0.1-0.6% of all pregnancies
    • Demographics
      • usually occurs after 27 weeks of pregnancy
      • can also occur postpartum, commonly 2 days after delivery
    • Risk factors
      • maternal age > 34 years
      • multiparity
      • Caucasian/European descent
  • ETIOLOGY
    • Pathogenesis
      • mechanism
        • thought to be due to endothelial activation, consumption of platelets, microangiopathic hemolysis, and microvascular injury
        • complement dysfunction may also play a role
  • Presentation
    • Symptoms
      • common symptoms
        • nausea and vomiting
        • right upper quadrant pain
        • headache
        • visual changes
        • jaundice
    • Physical exam
      • inspection
        • jaundice
        • dry mucous membranes
        • weakness
        • hypertension
        • nondependent edema
          • periorbital
          • upper or lower extremities
  • Studies
    • Serum labs
      • elevated AST/ALT
      • hemolysis
        • elevated lactate dehydrogenase
        • elevated bilirubin
        • hemolysis on peripheral smear
          • schistocytes, helmet cells, and burr cells
      • decreased platelets
      • typically normal coagulation studies
  • Differential
    • Acute fatty liver of pregnancy
      • key distinguishing factor
        • characterized primarily with fulminant liver failure
    • Preeclampsia
      • key distinguishing factor
        • signs and symptoms of preeclampsia have significant overlap with HELLP syndrome
        • however, preeclampsia classically occurs in younger patients (mean age of 19) and is characterized by hypertension and proteinuria
  • Treatment 
    • Medical
      • resuscitation
        • indications
          • all patients
        • modalities
          • intravenous fluids
          • transfusion of blood products
      • intravenous steroids
        • indications
          • platelets < 100,000/mm3
      • intravenous magnesium sulfate
        • indications
          • seizure prophylaxis
      • antihypertensive medications
        • indications
          • hypertension
        • drugs
          • labetalol
          • hydralazine
          • nifedipine
    • Surgical
      • delivery
        • indications
          • if stable, consider steroids and immediate delivery
          • if not stable, immediate delivery
  • Complications
    • Maternal complications
      • cerebral hemorrhage
      • disseminated intravascular coagulopathy
      • acute renal failure
      • hepatic rupture
      • risk of recurrence in subsequent pregnancies
      • increased risk of preeclampsia, preterm delivery, and placental abruption
    • Neonatal complications
      • prematurity
      • intrauterine growth retardation
      • fetal demise
    • Other complications
      • increased risk of cardiovascular disease
  • Prognosis
    • Most patients stabilize within 48 hours
    • Maternal mortality 1-3%

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