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. 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)
  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. 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)

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
  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

XIV. Prognosis

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

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Ontology: HELLP Syndrome (C0162739)

Definition (NCI) Severe preeclampsia associated with any of the following findings: thrombocytopenia (platelets less than 100,000 per microliter), impaired liver function (twice normal elevation of hepatic transaminases; severe, persistent right upper quadrant or epigastric pain), progressive renal insufficiency (serum creatinine greater than 1.1 mg/dL or doubling of baseline in the absence of other renal disease), pulmonary edema, or new-onset cerebral or visual disturbances.(NICHD)
Definition (MSH) A syndrome of HEMOLYSIS, elevated liver ENZYMES, and low blood platelets count (THROMBOCYTOPENIA). HELLP syndrome is observed in pregnant women with PRE-ECLAMPSIA or ECLAMPSIA who also exhibit LIVER damage and abnormalities in BLOOD COAGULATION.
Concepts Disease or Syndrome (T047)
MSH D017359
ICD10 O14.2
SnomedCT 199010001, 95605009
LNC LA16928-6
English Syndrome, HELLP, HELLP Syndrome, HELLP - Syndrome of haemolysis/elev liver enz/low platelet, HELLP - Syndrome of hemolysis/elev liver enz/low platelet, HELLP - haemo/el liv en/low pl, Haemo/el liv en/low pl, Syndrome of haemolysis, elevated liver enzymes and low platelet, Syndrome of haemolysis/elev liver enz/low platelet, Syndrome of hemolysis, elevated liver enzymes and low platelet, Syndrome of hemolysis/elev liver enz/low platelet, HELLP syndrome (diagnosis), HELLP syndrome (hemolysis, elevated liver enzymes, low platelets), HELLP Syndrome [Disease/Finding], Hemolysis, Elevated Liver Enzymes, Lowered Platelets, syndrome hellp, hellp syndrome, HELLP, HEMOLYSIS, ELEVATED LIVER ENZYMES, AND LOW PLATELET COUNT, Syndrome of haemolysis, elevated liver enzymes and low platelet (disorder), Hemolysis-Elevated Liver Enzymes-Low Platelet Count Syndrome, HELLP syndrome, HELLP - Syndrome of haemolysis, elevated liver enzymes and low platelet, HELLP - Syndrome of hemolysis, elevated liver enzymes and low platelet, Haemolysis-elevated liver enzymes-low platelet count syndrome, Hemolysis-elevated liver enzymes-low platelet count syndrome (disorder), Hemolysis-elevated liver enzymes-low platelet count syndrome, HELLP syndrome (disorder)
Spanish síndrome de hemólisis, enzimas hepáticas elevadas y bajo recuento de plaquetas, síndrome HELLP (trastorno), síndrome HELLP, síndrome de hemólisis, enzimas hepáticas elevadas y bajo recuento de plaquetas (trastorno), síndrome de hemólisis-enzimas hepáticas elevadas-bajo recuento de plaquetas, Síndrome de HELLP, Síndrome HELLP
Japanese HELLP症候群, HELLPショウコウグン
Swedish HELLP-syndrom
Finnish HELLP-oireyhtymä
Polish Zespół HELLP
Hungarian HELLP syndroma
Czech HELLP syndrom, syndrom HELLP
Norwegian HELLP-syndrom
Portuguese Síndrome de HELLP, Síndrome HELLP
Dutch HELLP syndrome, HELLP-syndroom, Syndroom, HELLP-
French HELLP syndrome, Hellp syndrome
German HELLP - Syndrom, HELLP-Syndrom
Italian Sindrome HELLP