HELLP Syndrome

Aka: HELLP Syndrome, Hemolysis Elevated Liver Enzymes Low Platelets

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II. Definitions

  1. HELLP Syndrome
    1. Characterized by Hemolysis, elevated liver enzymes, and Low Platelet Count
    2. Variant of Severe Preeclampsia

III. Epidemiology

  1. More common in Multiparous caucasians
  2. More common after age 25 years
  3. Complicates 15-20% of Severe Preeclampsia cases (but <1% of all pregnancies)
  4. Prevalence: 6 per 1000 deliveries
  5. Onset after 20 weeks gestation
    1. Preterm <27 weeks: 7% of cases
    2. Preterm <37 weeks: 46-52% of cases
    3. Term (37-41 weeks): 14-18% of cases
    4. Postpartum: 30-33% of cases
      1. Typically within 48 hours of delivery
  6. References
    1. Sibai (1993) Am J Obstet Gynecol 169(4): 1000-6 [PubMed]

IV. Pathophysiology

  1. Microangiopathic Hemolysis
    1. RBCs fragmented in vessels with damaged endothelium
  2. Fibrin deposition
    1. Vascular deposition contributes to Hemolysis
    2. Hepatic sinusoid deposition with periportal necrosis
      1. Results in liver enzyme abnormalities
  3. Platelet aggregation

V. Risk factors

  1. Advanced maternal age
  2. Caucasian
  3. Multiparity
  4. Preeclampsia
    1. HELLP complicates 12% of Preeclampsia cases overall (20% of Severe Preeclampsia)
    2. However, normal Blood Pressure in up to 18% of cases and no Proteinuria in 13% of cases

VI. Precautions

  1. HELLP may present in normotensive patients without Proteinuria

VII. Symptoms

  1. Viral-type prodrome
    1. Malaise
  2. Gastrointestinal symptoms (most common)
    1. Right Upper Quadrant Abdominal Pain
    2. Epigastric Pain
    3. Nausea or Vomiting
  3. Other symptoms that are variably present
    1. Jaundice
  4. Severe Preeclampsia symptoms may be present
    1. Severe Headache
    2. Vision changes (scotomata)
    3. Leg Edema
  5. Bleeding may occur if Coagulopathy is present
    1. Hematuria
    2. Gastrointestinal Bleeding

VIII. Signs

  1. Hypertension (85% of cases)
  2. Proteinuria (87% of cases)
  3. Weight gain
  4. Edema

IX. Differential Diagnosis

  1. See Right Upper Quadrant Abdominal Pain
  2. See Hemolysis Causes
  3. See Thrombocytopenia
  4. See Aminotransferase
  5. Disseminated Intravascular Coagulation
  6. Thrombocytopenic Purpura
  7. Acute Fatty Liver of Pregnancy
    1. Fatty Liver is associated with more severe liver failure and Renal Insufficiency
    2. HELLP is associated with Severe Hypertension, which is less common in Fatty Liver

X. Diagnosis

  1. Hemolysis (diagnosis requires 2 of the following)
    1. Serum Bilirubin >1.2 mg/dl
    2. Serum Haptoglobin decreased
    3. Significant Hemoglobin drop not due to Hemorrhage
    4. Peripheral Blood Smear with signs of Hemolysis
      1. Schistocytes
      2. Burr Cells
      3. Helmet Cells
  2. Elevated liver enzymes
    1. Liver transaminases (AST, ALT) increased more than twice normal
    2. Lactate Dehydrogenase >600 IU/L (or more than twice normal, increases with Hemolysis)
  3. Low Platelet Count (Thrombocytopenia)
    1. Platelet Count < 100,000 per mm3
    2. Low Platelet Count by Alternative Classification
      1. Class 3: Platelet Count 100,000 to 150,000 per mm3
      2. Class 2: Platelet Count 50,000 to 100,000 per mm3
      3. Class 1: Platelet Count <50,000 per mm3

XI. Labs

  1. General
    1. Obtain labs at baseline and repeat every 12 hours until stable
  2. Urine Protein to Creatinine Ratio
    1. Significant Proteinuria may be present on Urinalysis, but is not required for diagnosis
  3. Complete Blood Count with Platelets
  4. Peripheral Blood Smear
  5. Lactate Dehydrogenase (marker of Hemolysis)
  6. Comprehensive Panel
    1. Serum Creatinine
    2. Aspartate transaminase (AST)
    3. Alanine transaminase (ALT)
    4. Serum Bilirubin
  7. Coagulation Factors if Platelet Count<50,000 or bleeding (evaluate for DIC)
    1. Fibrinogen
    2. Fibrin split products
    3. Prothrombin (PT)
    4. Partial Thromboplastin Time (PTT)

XII. Management

  1. See Severe Preeclampsia Management
  2. Magnesium Sulfate
    1. Start at admission and continue for 24-48 hours after delivery
  3. Emergent Management of Hypertension >160/110 mmHg (2 values 15 min apart)
    1. See Blood Pressure Management in Pregnancy
  4. Consult maternal fetal medicine
    1. Transfer to tertiary center
  5. Consider Dexamethasone 10 mg IV every 12 hours
    1. Consider when Platelet Count <100,000/mm3
    2. May improve lab abnormalities and delay delivery
    3. Magann (1994) Am J Obstet Gynecol 171:1148-53 [PubMed]
  6. Consider Blood Products
    1. Platelet Transfusion
      1. Platelet Count <20,000/mm3 (some use cutoff <10,000/mm3)
      2. Platelet Count <50,000/mm3 prior to ceserean
      3. Avoid Regional Anesthesia (spinal Anesthesia) if Platelet Count <50,000/mm3
    2. Other Blood Products to consider in active bleeding
      1. Packed Red Blood Cells for severe Anemia with Hemoglobin <7 g/dl
      2. Fresh Frozen Plasma for coagulation abnormalities

XIII. Complications

  1. Hepatic rupture
  2. Acute Renal Failure
  3. Pulmonary Edema
  4. Ascites
  5. Pleural Effusion
  6. Abruptio Placenta
  7. Postpartum Hemorrhage
  8. Disseminated Intravascular Coagulation (DIC)

XIV. Prognosis

  1. Maternal mortality: 1-3 (up to 30% in some studies)%
  2. Infant and fetal mortality: 6-36%

XV. Prevention

  1. See Pregnancy Induced Hypertension Prophylaxis

XVI. References

  1. Fontaine (2000) in ALSO, B:1-36
  2. Marlow (2021) Crit Dec Emerg Med 35(2): 19-23
  3. Barton (2004) Clin Perinatol 31(4): 807-33 [PubMed]
  4. Farahi (2024) Am Fam Physician 109(3): 251-60 [PubMed]
  5. Leeman (2008) Am Fam Physician 78:93-100 [PubMed]
  6. Leeman (2016) Am Fam Physician 93(2):121-7 [PubMed]
  7. Padden (1999) Am Fam Physician 60:829-39 [PubMed]

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