HELLP SYNDROME

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HELLP Syndrome

Definition & Acronym

HELLP is an acronym for:

H - Hemolysis (microangiopathic hemolytic anemia)
EL - Elevated Liver enzymes
LP - Low Platelets

It represents a severe form of preeclampsia in which hepatic and hematologic abnormalities dominate, driven by thrombotic microangiopathy (TMA).

Harrison's Principles of Internal Medicine 22E, p. 2490
Comprehensive Clinical Nephrology 7e, p. 630
Brenner and Rector's The Kidney, p. 2028

Epidemiology

Statistic	Figure
Incidence in all pregnancies	0.2-0.9%
In severe preeclampsia	10-20%
Maternal mortality	1-7.4% (up to 34% in severe cases)
Perinatal mortality	7-34% (depends on gestational age)
Cases before week 28	~20%
Postpartum onset	~30% of cases

Risk factors: Advanced maternal age, Caucasian ethnicity, multiparity, abnormal placentation, family history, elevated fetal mRNA for FLT1/endoglin. Patients with antiphospholipid syndrome have elevated risk.

Importantly, ~20% of cases have NO preceding hypertension or proteinuria, making it a diagnostic trap.

Pathophysiology

The exact cause is unknown, but the central mechanism involves:

Abnormal placentation → systemic endothelial dysfunction
Activation of the coagulation cascade → fibrin deposition in hepatic sinusoids and microvasculature
Microangiopathic hemolytic anemia → platelet consumption and red cell fragmentation (schistocytes)
Hepatic ischemia → from sinusoidal fibrin deposition and intravascular hypovolemia → elevated transaminases
Complement dysregulation - complement gene mutations or dysfunction found in 30-40% of HELLP patients
Elevated inflammatory markers (CRP, IL-1Ra, IL-6) and soluble HLA-DR compared to preeclampsia alone

Clinical Features

Symptom/Sign	Notes
Epigastric / RUQ pain	Most common presenting symptom - can mimic gastroenteritis, cholecystitis, pancreatitis
Nausea & vomiting	Frequent accompanying feature
Malaise	May mimic viral syndrome
Headache, blurred vision	Signs of CNS involvement
Hypertension	May be absent initially or at all
Proteinuria	Present in 86-100% overall, but may be absent

Key pitfall: Hypertension may be absent, leading to misdiagnosis as gastroenteritis, cholecystitis, hepatitis, or pyelonephritis. Any pregnant woman at >20 weeks gestation (or up to 7 days postpartum) with abdominal pain must be evaluated for HELLP.

Tintinalli's Emergency Medicine, p. 674

Diagnostic Criteria

ACOG Task Force Criteria (current standard)

Hemolysis PLUS at least 2 of the following:
- Thrombocytopenia (platelets <100,000/μL)
- Renal insufficiency (creatinine >1.1 mg/dL or doubling)
- Impaired liver function (transaminases twice upper limit of normal)
- Pulmonary edema
- New-onset headache unresponsive to medication, or visual symptoms

Key Lab Findings

Test	Finding in HELLP
Peripheral smear	Schistocytes (fragmented RBCs)
Platelet count	<100,000/μL (<150,000 suspicious)
LDH	>600 U/L
AST/ALT	Elevated (usually <500 U/L, rarely up to >1000 U/L)
Total bilirubin	>1.2 mg/dL
Serum haptoglobin	Low (hemolysis marker)
Urine protein	Proteinuria (86-100%)

Classification Systems

Mississippi Triple-Class System (platelet-based severity)

Class	Platelet Nadir
Class I (Severe)	≤50,000/mm³
Class II (Moderate)	50,001-100,000/mm³
Class III (Mild)	100,001-150,000/mm³

Tennessee Classification

Complete HELLP: All 3 criteria present (hemolysis + elevated LDH/AST + platelets <100,000)
Incomplete (Partial) HELLP: Only 1 or 2 criteria present (e.g., EL only, LP only, HEL without thrombocytopenia)
Sleisenger & Fordtran's GI and Liver Disease, p. 4067
Yamada's Textbook of Gastroenterology 7e

Complications

Maternal

Complication	Approximate Rate
Eclampsia	6%
Placental abruption	10%
DIC	8-21%
Acute renal failure	5-50%
Pulmonary edema	10%
Hepatic subcapsular hematoma/rupture	~1% of HELLP cases
Cerebral infarction/hemorrhage	Rare but life-threatening
Purtscher-like retinopathy	Rare (hemorrhagic/vaso-occlusive)

Liver rupture occurs predominantly in the right lobe, following subcapsular hemorrhage stretching the hepatic capsule. More than 95% of liver ruptures in pregnancy involve severe preeclampsia/HELLP. The triad of RUQ pain + hypertension + shock should raise immediate suspicion.

Fetal

Preterm birth
IUGR (fetal growth restriction)
Fetal demise

Differential Diagnosis

The table below summarizes the key distinctions from overlapping conditions:

Feature	HUS/TTP	HELLP	AFLP
Hemolytic anemia	+++	++	±
Thrombocytopenia	+++	++	±
Coagulopathy	-	±	+
CNS symptoms	++	±	±
Renal failure	+++	+	++
Hypertension	±	+++	±
Elevated AST	±	++	+++
Elevated bilirubin	++	+	+++
Serum ammonia	Normal	Normal	High
Effect of delivery	None	Recovery	Recovery
ADAMTS13	<10% (TTP)	30-60% reduced	Normal
Management	Plasma exchange	Supportive + delivery	Supportive + delivery

LDH:AST ratio can help: ratio ~13:1 in HELLP with preeclampsia vs. ~29:1 in non-preeclamptic patients.

Brenner and Rector's The Kidney, p. 2034-2036
Harrison's 22E

Management

1. Immediate Stabilization

IV access, blood products on standby
Continuous fetal monitoring
Seizure prophylaxis: Magnesium sulfate (4 g IV loading dose, then 1-2 g/hr infusion)
Antihypertensives for BP ≥160/110 mmHg:

Drug	Mechanism	Onset	Dosage
Labetalol	α + β blocker	5 min	20 mg IV, then 40-80 mg q10 min (max 300 mg)
Hydralazine	Arterial vasodilator	20 min	5 mg IV or 10 mg IM, repeat q20 min
Nifedipine	Calcium channel blocker	10-20 min	10 mg PO, repeat in 30 min

2. Delivery - The Definitive Treatment

The key treatment is delivery; HELLP syndrome resolves spontaneously after delivery
Gestational age ≥34 weeks: Prompt delivery recommended
<34 weeks, no serious complications: Consider antenatal corticosteroids (betamethasone) for fetal lung maturity FIRST, then deliver
Serious maternal/fetal complications: Prompt delivery regardless of gestational age
Cesarean section: Consider platelet transfusion to 40,000-50,000 cells/μL before procedure

3. Platelet Transfusion

Indicated for platelets <40,000-50,000/μL, especially pre-operatively

4. Corticosteroids (controversial)

Dexamethasone IV has been used in some protocols to accelerate recovery of platelets and transaminases, especially postpartum - but evidence is mixed and this is NOT routinely recommended per some guidelines

5. Plasma Exchange

No proven benefit in HELLP
May be indicated if TTP has not been ruled out
Eculizumab (anti-C5 complement inhibitor) has been reported in small case series if complement-mediated aHUS is suspected

6. Postpartum Monitoring

Labs may worsen before improving after delivery
Resolution typically within 48-72 hours, complete by 5 days
Monitor up to 7 days postpartum since 20-30% develop HELLP after delivery

Prognosis & Recurrence

Maternal mortality: ~1% (with modern care), perinatal mortality 7-34%
HELLP usually resolves fully after delivery
Recurrence risk in future pregnancies: reported at ~3-27%
Patients with complement mutations (30-40% of HELLP) may be at elevated risk of aHUS in future pregnancies

Summary Mnemonic

HELLP = Think whenever a pregnant woman >20 weeks presents with:

Headache + Epigastric pain + Low platelets + Liver enzymes up + Proteinuria

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