II. Definitions
- HELLP Syndrome
- Characterized by Hemolysis, elevated liver enzymes, and Low Platelet Count
- Variant of Severe Preeclampsia
III. Epidemiology
- More common in Multiparous caucasians
- More common after age 25 years
- Complicates 15-20% of Severe Preeclampsia cases (but <1% of all pregnancies)
- Prevalence: 6 per 1000 deliveries
- Onset after 20 weeks gestation
- Preterm <27 weeks: 7% of cases
- Preterm <37 weeks: 46-52% of cases
- Term (37-41 weeks): 14-18% of cases
- Postpartum: 30-33% of cases
- Typically within 48 hours of delivery
- References
IV. Pathophysiology
V. Risk factors
- Advanced maternal age
- Caucasian
- Multiparity
-
Preeclampsia
- HELLP complicates 12% of Preeclampsia cases overall (20% of Severe Preeclampsia)
- However, normal Blood Pressure in up to 18% of cases and no Proteinuria in 13% of cases
VI. Precautions
- HELLP may present in normotensive patients without Proteinuria
VII. Symptoms
- Viral-type prodrome
- Malaise
- Gastrointestinal symptoms (most common)
- Other symptoms that are variably present
- Severe Preeclampsia symptoms may be present
- Bleeding may occur if Coagulopathy is present
VIII. Signs
- Hypertension (85% of cases)
- Proteinuria (87% of cases)
- Weight gain
- Edema
IX. Differential Diagnosis
- See Right Upper Quadrant Abdominal Pain
- See Hemolysis Causes
- See Thrombocytopenia
- See Aminotransferase
- Disseminated Intravascular Coagulation
- Thrombocytopenic Purpura
-
Acute Fatty Liver of Pregnancy
- Fatty Liver is associated with more severe liver failure and Renal Insufficiency
- HELLP is associated with Severe Hypertension, which is less common in Fatty Liver
X. Diagnosis
-
Hemolysis (diagnosis requires 2 of the following)
- Serum Bilirubin >1.2 mg/dl
- Serum Haptoglobin decreased
- Significant Hemoglobin drop not due to Hemorrhage
- Peripheral Blood Smear with signs of Hemolysis
- Schistocytes
- Burr Cells
- Helmet Cells
- Elevated liver enzymes
- Liver transaminases (AST, ALT) increased more than twice normal
- Lactate Dehydrogenase >600 IU/L (or more than twice normal, increases with Hemolysis)
-
Low Platelet Count (Thrombocytopenia)
- Platelet Count < 100,000 per mm3
- Low Platelet Count by Alternative Classification
- Class 3: Platelet Count 100,000 to 150,000 per mm3
- Class 2: Platelet Count 50,000 to 100,000 per mm3
- Class 1: Platelet Count <50,000 per mm3
XI. Labs
-
General
- Obtain labs at baseline and repeat every 12 hours until stable
-
Urine Protein to Creatinine Ratio
- Significant Proteinuria may be present on Urinalysis, but is not required for diagnosis
- Complete Blood Count with Platelets
- Peripheral Blood Smear
- Lactate Dehydrogenase (marker of Hemolysis)
- Comprehensive Panel
- Serum Creatinine
- Aspartate transaminase (AST)
- Alanine transaminase (ALT)
- Serum Bilirubin
-
Coagulation Factors if Platelet Count<50,000 or bleeding (evaluate for DIC)
- Fibrinogen
- Fibrin split products
- Prothrombin (PT)
- Partial Thromboplastin Time (PTT)
XII. Management
- See Severe Preeclampsia Management
-
Magnesium Sulfate
- Start at admission and continue for 24-48 hours after delivery
- Emergent Management of Hypertension >160/110 mmHg (2 values 15 min apart)
- Consult maternal fetal medicine
- Transfer to tertiary center
- Consider Dexamethasone 10 mg IV every 12 hours
- Consider when Platelet Count <100,000/mm3
- May improve lab abnormalities and delay delivery
- Magann (1994) Am J Obstet Gynecol 171:1148-53 [PubMed]
- Consider Blood Products
- Platelet Transfusion
- Platelet Count <20,000/mm3 (some use cutoff <10,000/mm3)
- Platelet Count <50,000/mm3 prior to ceserean
- Avoid Regional Anesthesia (spinal Anesthesia) if Platelet Count <50,000/mm3
- Other Blood Products to consider in active bleeding
- Packed Red Blood Cells for severe Anemia with Hemoglobin <7 g/dl
- Fresh Frozen Plasma for coagulation abnormalities
- Platelet Transfusion
XIII. Complications
XIV. Prognosis
- Maternal mortality: 1-3 (up to 30% in some studies)%
- Infant and fetal mortality: 6-36%
XV. Prevention
XVI. References
- Fontaine (2000) in ALSO, B:1-36
- Marlow (2021) Crit Dec Emerg Med 35(2): 19-23
- Barton (2004) Clin Perinatol 31(4): 807-33 [PubMed]
- Farahi (2024) Am Fam Physician 109(3): 251-60 [PubMed]
- Leeman (2008) Am Fam Physician 78:93-100 [PubMed]
- Leeman (2016) Am Fam Physician 93(2):121-7 [PubMed]
- Padden (1999) Am Fam Physician 60:829-39 [PubMed]
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Related Studies
| 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ｼｮｳｺｳｸﾞﾝ |
| 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 |
