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HELLP Syndrome
Aka: HELLP Syndrome, Hemolysis Elevated Liver Enzymes Low Platelets
- See Also
- Preeclampsia
- Severe Preeclampsia
- Blood Pressure Management in Pregnancy
- Definitions
- HELLP Syndrome
- Characterized by Hemolysis, elevated liver enzymes, and Low Platelet Count
- Variant of Severe Preeclampsia
- 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
- Sibai (1993) Am J Obstet Gynecol 169(4): 1000-6 [PubMed]
- Pathophysiology
- Microangiopathic Hemolysis
- RBCs fragmented in vessels with damaged endothelium
- Fibrin deposition
- Vascular deposition contributes to Hemolysis
- Hepatic sinusoid deposition with periportal necrosis
- Results in liver enzyme abnormalities
- Platelet aggregation
- 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
- Precautions
- HELLP may present in normotensive patients without Proteinuria
- Symptoms
- Viral-type prodrome
- Malaise
- Gastrointestinal symptoms (most common)
- Right Upper Quadrant Abdominal Pain
- Epigastric Pain
- Nausea or Vomiting
- Other symptoms that are variably present
- Jaundice
- Severe Preeclampsia symptoms may be present
- Severe Headache
- Vision changes (scotomata)
- Leg Edema
- Bleeding may occur if Coagulopathy is present
- Hematuria
- Gastrointestinal Bleeding
- Signs
- Hypertension (85% of cases)
- Proteinuria (87% of cases)
- Weight gain
- Edema
- Differential Diagnosis
- See Right Upper Quadrant Abdominal Pain
- See Hemolysis Causes
- See Thrombocytopenia
- See Aminotransferase
- 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
- 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)
- 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
- Labs
- Consider Preeclampsia labs
- Significant Proteinuria may be present on Urinalysis, but is not required for diagnosis
- Complete Blood Count with platelets
- Peripheral Blood Smear
- Comprehensive Panel
- Aspartate transaminase (AST)
- Alanine transaminase (ALT)
- Serum Bilirubin
- Lactate Dehydrogenase
- Coagulation Factors if platelets <50,000 or bleeding (evaluate for DIC)
- Fibrinogen
- Fibrin split products
- Prothrombin (PT)
- Partial Thromboplastin Time (PTT)
- 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)
- See Blood Pressure Management in Pregnancy
- Consult maternal fetal medicine
- Consider Dexamethasone 10 mg IV q12 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
- Complications
- Hepatic rupture
- Acute Renal Failure
- Pulmonary Edema
- Ascites
- Pleural Effusion
- Abruptio Placenta
- Postpartum Hemorrhage
- Disseminated Intravascular Coagulation (DIC)
- Prognosis
- Maternal mortality: 1-3 (up to 30% in some studies)%
- Infant and fetal mortality: 6-36%
- 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]
- 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]