II. Definition

  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 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. Headache
    2. Jaundice
  4. Severe Preeclampsia symptoms may be present
  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
    1. Serum Bilirubin >1.2 mg/dl
    2. Lactate Dehydrogenase >600 IU/L
    3. Peripheral Smear with signs of Hemolysis
      1. Schistocytes
      2. Burr Cells
      3. Helmet Cells
  2. Elevated liver enzymes
    1. Liver transaminases increased (twice normal is a common threshold used)
  3. Low Platelet Count (Thrombocytopenia)
    1. Platelet Count < 100,000 mm3
    2. Low Platelet Count by Alternative Classification
      1. Class 3: Platelet Count 100,000 to 150,000 mm3
      2. Class 2: Platelet Count 50,000 to 100,000 mm3
      3. Class 1: Platelet Count <50,000 mm3

XI. Labs

  1. Consider Preeclampsia labs
  2. Complete Blood Count with platelets
  3. Peripheral Blood Smear
  4. Liver 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. Consult maternal fetal medicine
  4. 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]
  5. Consider Blood Products
    1. Platelet Transfusion
      1. Platelet Count <20,000/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 blood loss Anemia
      2. Fresh Frozen Plasma for coagulation abnormalities

XIV. Prognosis

  1. Maternal mortality: 1-3%
  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
Russian GEMOLITICHESKII SINDROM S TROMBOTSITOPENIEI, PREEKLAMPSICHESKII GEMOLITICHESKII SINDROM, HELLP SINDROM, HELLP СИНДРОМ, ГЕМОЛИТИЧЕСКИЙ СИНДРОМ С ТРОМБОЦИТОПЕНИЕЙ, ПРЕЭКЛАМПСИЧЕСКИЙ ГЕМОЛИТИЧЕСКИЙ СИНДРОМ
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