DESCRIPTION 
- HELLP syndrome: Hemolysis Elevated Liver enzymes, Low Platelets
- Continuum with severe preeclampsia as most patients will be hypertensive
- Liver involvement is hallmark:
- Other organs may be involved (e.g., brain, kidneys, lungs)
- HELLP syndrome divided into 3 groups, representing severity of the disease; severity is directly related to the platelet count:
- Class 1: Most severe form; platelet nadir < 50,000 platelets/µL
- Class 2: Less severe; platelet nadir between 50,000 and 100,000 platelets/µL
- Class 3: Least severe; platelet nadir between 100,000 and 150,000 platelets/µL
- Most maternal deaths occur with class 1
- Increased mortality rate is associated with hepatic hemorrhage or CNS or vascular insult to the cardiopulmonary or renal systems
- Incidence: 0.2% of all pregnancies
- 12–18% have normal BP
- Occurs in 20% of pregnancies with severe preeclampsia or eclampsia
- At diagnosis:
- 52% preterm
- 18% term
- 32% postpartum
RISK FACTORS
Frequently white, multiparous, older
Pediatric Considerations
Infant mortality is greater in women with HELLP
ETIOLOGY
- Unclear, but vasospasm is the basis:
- Fetal-placental debris is released into maternal circulation, causing systemic inflammatory response
- Vascular constriction causes resistance to blood flow and HTN
- Vasospasm probably damages vessels directly
- Angiotensin II causes endothelial cells to contract
- Endothelial cell is damaged and interendothelial cell leaks are the result
- Small-vessel leaks:
- Platelets and fibrinogen get deposited in the subendothelium
- Fibrin deposition develops in severe cases
- Vascular changes and local tissue hypoxia lead to hemorrhage, necrosis, and end-organ damage
[Outline]
SIGNS AND SYMPTOMS
History
- History and physical exam with attention to symptoms of abdominal pain, nausea, vomiting, and headache
- Obstetric history:
- Parity
- Deliveries
- History of hypertensive disorder during pregnancy
- Estimated gestational age
- Prenatal care
- May present with flulike symptoms, such as fatigue or malaise
- Nausea, usually with vomiting
- Right upper quadrant or epigastic pain:
- Pain increases with severity of disease
- Headache, often with visual changes
- Symptoms which carry higher morbidity:
ALERT
Determination of gestational age and fetal viability is critical in HELLP.
Physical Exam
- Vital signs with attention to BP
- May not have systolic or diastolic HTN
- Many patients will have right upper quadrant pain, concern for liver subcapsular hematoma
- Evidence of fluid overload
- Careful neurologic exam
- Fetal heart tones
ESSENTIAL WORKUP
- Immediate CBC with platelet count and smear, BUN, creatinine, LFTs, coagulation profile, and magnesium level
- Urinalysis for protein; screen for UTI
- Weigh patient to determine recent weight gain
DIAGNOSIS TESTS & INTERPRETATION
Lab
- CBC:
- Platelet count and smear:
- Disseminated intravascular coagulation screen
- Coagulation profile:
- BUN, creatinine, and magnesium levels
- LFTs to assess hemolysis markers and hepatic dysfunction:
- Elevated aspartate aminotransferase level: > 40 IU/L
- Elevated alanine aminotransferase level: > 40 IU/L
- Elevated LDH: > 600 IU/L
- Elevated serum bilirubin: > 1.2 mg/dL
Imaging
- CXR:
- Suspected pulmonary edema
- CT of head:
- Mental status changes or focal neurologic deficit
- US of the pelvis (transabdominal or transvaginal):
DIFFERENTIAL DIAGNOSIS
- GI:
- Hematologic:
- Neurologic:
- Other:
[Outline]
PRE-HOSPITAL
Cautions:
- Transport patient in left lateral decubitus position to prevent inferior vena cava syndrome
- Venous access for anticipated seizure activity
- Routine seizure management (preferably with magnesium sulfate) if the patient seizes
ALERT
Transport to a facility capable of providing high-risk obstetric care.
INITIAL STABILIZATION/THERAPY
- ABC management
- Left lateral decubitus position to prevent inferior vena cava syndrome
- High-flow oxygen via face mask
- Maternal monitoring:
- Cardiac
- Pulse oximetry
- Tocography
- Fetal monitoring
ED TREATMENT/PROCEDURES
- Control HTN with antihypertensives (see Medication):
- Avoid ACE inhibitors because of fetal side effects
- Heparin should be avoided because of bleeding complications
- Treat preeclampsia or eclampsia with IV magnesium sulfate:
- Magnesium sulfate is not given to treat HTN
- Order type and screen for possible transfusion
- Call for emergent obstetric consult, consider neonatology consult:
- Consider emergent delivery
- Early plasma exchange therapy has shown promise in postpartum patients with severe disease
- Discuss administration of glucocorticoid with consultant:
- Helps fetal lung maturity
- IV dexamethasone more effective than IM betamethasone
- Depends on gestational age of fetus
- Does not reduce disease severity or duration, but improves platelet counts
- Limit IV fluid administration unless clinical evidence of dehydration:
- Excess fluids promote further capillary leak
- Lactated Ringers or NS at 60 mL/hr (no more than 125 mL/hr)
- Monitor urine output with Foley catheter
- Correct thrombocytopenia by platelet transfusion in women with platelet counts < 20,000 platelets/µL, even without active bleeding, as risk of postpartum bleeding is significantly increased
- Platelet counts > 40,000 platelets/µL are safe for vaginal delivery
- Correct thrombocytopenia to platelet counts > 50,000 platelets/µL if cesarean delivery planned
- If coagulation dysfunction is present, transfusion with fresh frozen plasma and packed RBCs in consultation with obstetrics
- Transfusion with packed RBCs for hemoglobin < 10 g/dL
MEDICATION
First Line
- Hydralazine: 2.5 mg IV, then 5–10 mg q15–20min:
- Up to 40 mg total dose, to keep diastolic BP < 110 mm Hg
- IV drip 5–10 mg/hr titrated
- Labetalol: 10 mg IV, then 20–80 mg IV q10min:
- Up to 300 mg total dose
- IV drip 1–2 mg/min titrated
Second Line
- Nitroprusside: 0.25 µg/kg/min as a drip:
- Increase 0.25 µg/kg/min q5min
- Use only if no response to hydralazine or labetalol
- Magnesium sulfate: 4–6 g in 100 mL IV over 15–20 min as loading dose:
- Maintenance drip starting at 2 g/hr
- Titrate to clinical effect
- Watch for toxicity (antidote is calcium gluconate 10%, 10 mL IV over 3 min).
- Measure magnesium sulfate level at 4–6 hr; adjust drip to achieve levels between 4 and 7 mEq/L.
[Outline]
DISPOSITION
Admission Criteria
- Admit all patients to obstetric service for continuous monitoring of mother and fetus
- ICU admission:
- Pulmonary edema
- Respiratory failure
- Cerebral edema
- GI bleeding with hemodynamic instability
Discharge Criteria
Patients with HELLP syndrome should always be admitted. Discharge should be a decision of the OB Consultant
Issues for Referral
After stabilization in the ED, transfer to facility capable of managing high-risk obstetric conditions unless delivery is imminent.
FOLLOW-UP RECOMMENDATIONS
Patients should be followed closely by OB:
- May develop HELLP after delivery, usually within 48 hr
[Outline]
- Ciantar E, Walker JJ. Pre-eclampsia, severe pre-eclampsia and hemolysis, elevated liver enzymes and low platelets syndrome: What is new? Women's Health. 2011;7(5):555–569.
- Deak TM, Moskovitz JB. Hypertension and pregnancy. Emerg Med Clin North Am. 2012;30:903–917.
- Giannubilo SR, Bezzeccheri V, Cecchi S, et al. Nifedipine versus labetalol in the treatment of hypertensive disorders of pregnancy. Arch Gynecol Obstet. 2012;286:637–642.
- Woudstra DM, Chandra S, Hofmeyr GJ, et al. Corticosteroids for HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome in pregnancy (Review). Cochrane Database Syst Rev. 2010; (9):CD008148.
- Yoder SR, Thornburg LL, Bisognano JD. Hypertension in pregnancy and women of childbearing age. Am J Med. 2009;122:890–895.
See Also (Topic, Algorithm, Electronic Media Element)
Preeclampsia