HELLP Syndrome

1. See Also
   1. Preeclampsia
   2. Severe Preeclampsia
   3. Blood Pressure Management in Pregnancy
2. Definitions
   1. HELLP Syndrome
      1. Characterized by Hemolysis, elevated liver enzymes, and Low Platelet Count
      2. Variant of Severe Preeclampsia
3. 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]
4. 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
5. 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
6. Precautions
   1. HELLP may present in normotensive patients without Proteinuria
7. 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
8. Signs
   1. Hypertension (85% of cases)
   2. Proteinuria (87% of cases)
   3. Weight gain
   4. Edema
9. Differential Diagnosis
   1. See Right Upper Quadrant Abdominal Pain
   2. See Hemolysis Causes
   3. See Thrombocytopenia
   4. See Aminotransferase
   5. 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
10. 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)
    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
11. Labs
    1. Consider Preeclampsia labs
       1. Significant Proteinuria may be present on Urinalysis, but is not required for diagnosis
    2. Complete Blood Count with Platelets
    3. Peripheral Blood Smear
    4. Comprehensive Panel
       1. Aspartate transaminase (AST)
       2. Alanine transaminase (ALT)
       3. Serum Bilirubin
       4. Lactate Dehydrogenase
    5. Coagulation Factors if Platelets <50,000 or bleeding (evaluate for DIC)
       1. Fibrinogen
       2. Fibrin split products
       3. Prothrombin (PT)
       4. Partial Thromboplastin Time (PTT)
12. 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
    5. Consider Dexamethasone 10 mg IV q12 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
13. 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)
14. Prognosis
    1. Maternal mortality: 1-3 (up to 30% in some studies)%
    2. Infant and fetal mortality: 6-36%
15. 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. Leeman (2008) Am Fam Physician 78:93-100 [PubMed]
    5. Leeman (2016) Am Fam Physician 93(2):121-7 [PubMed]
    6. Padden (1999) Am Fam Physician 60:829-39 [PubMed]