HELLP syndrome

Diagnostic Criteria — Tennessee Classification (Sibai) and Mississippi

• Hemolysis (H): Blood smear with schistocytes (erythrocyte fragments due to microvascular shearing) + LDH ≥ 600 IU/L + total bilirubin ≥ 20 µmol/L + undetectable haptoglobin< 0.25 g/L) + Direct Coombs negative (non-immune mechanical hemolysis)
• Elevated liver enzymes (EL): AST ≥ 70 U/L (2× ULN) and/or elevated ALT and/or LDH ≥ 600 U/L (reflects both hemolysis and liver cell damage)
• Thrombocytopenia (LP) plates < 100 000/µL (classe 1 de Mississippi) + plaquettes 100 000–150 000/µL (classe 2) + plaquettes > 150,000/µL (Class 3 - partial ELPP syndrome without frank thrombocytopenia)
• Mississippi Classification (Prognosis): class 1 (platelets) < 50,000/µL (most severe) + class 2 (50,000–100,000/µL) + class 3 (100,000–150,000/µL)
• Partial HELP presence of only 1 or 2 components (EL + LP without frank hemolysis = ELLP syndrome + or H only) → risk of evolution to complete HELLP → close monitoring

Clinical presentation

• Symptoms: epigastric pain or right upper quadrant pain (70–90% % — cardinal sign — compression of the hepatic capsule by edema + sinusoidal thrombi) + nausea + vomiting + malaise + headaches + visual disturbances + hypertension may be absent in 15–20 % of cases (normotensive HELLP) → do not rule out HELLP based on the absence of hypertension
• Pain location: epigastrium + right hypochondrium (epigastric bar) → radiation to the right shoulder or back → Spiegel's sign (pain on palpation of the right hypochondrium) + alarm sign if intense right scapular pain → subcapsular liver hematoma (imminent rupture)
• Concomitant pre-eclampsia present in 85 % of cases (hypertension + proteinuria) + but 15 % of HELLP are normotensive without proteinuria → never exclude HELLP based on the absence of classic pre-eclampsia
• HELP post-partum 25-30% of cases occur within 48 hours of delivery (rarely up to 7 days postpartum) → epigastric pain + vomiting + malaise + liver function tests + platelet count systematically checked postpartum if suspected

Dreaded maternal complications

• Subcapsular hematoma of the liver + hepatic rupture (< 2 %) : the most severe complication + severe abdominal pain + right shoulder radiation + hemorrhagic shock + maternal mortality 50–86% % in case of rupture + diagnosis: hepatic ultrasound + abdominal CT scan + treatment: hepatic arterial embolization + or surgery (hepatic packing)
• DIC (Disseminated Intravascular Coagulation — 20 %): Prolonged PT + low fibrinogen + elevated D-dimers + major postpartum hemorrhage + bleeding at puncture site + massive transfusion
• Acute kidney injury (7–15 %): Oliguria + elevated creatinine + acute tubular necrosis due to renal ischemia + dialysis if severe
• Pulmonary edema (6–8 %): water overload + increased vascular permeability + hypoalbuminemia → SpO2 + chest X-ray + O2 + diuretics (with caution)
• Placental abruption (15-20 %) : Abdominal pain + vaginal bleeding + fetal distress → urgent delivery
• Hemorrhagic stroke (< 1 %): Severe thrombocytopenia + uncontrolled hypertension → severe headaches + neurological deficit → neurosurgical emergency

Support

• Fundamental principle — childbirth = only definitive treatment: HELLP syndrome healing requires delivery and placental evacuation → any decision to delay (for lung maturation) must be weighed against maternal risk → gestational age ≥ 34 weeks of gestation → immediate delivery recommended + gestational age 27–34 weeks of gestation → corticosteroids (betamethasone 12 mg IM × 2 doses / 24 h) + if clinical stabilization + delivery within 24–48 h + gestational age < 27 SA → case-by-case discussion at a reference center
• Magnesium Sulfate IV — Prevention and Treatment of Seizures (Eclampsia): loading dose 4–6 g IV over 15–20 min + maintenance infusion 1–2 g/h → prevention of eclampsia + fetal neuroprotection if < 32 SA + surveiller : réflexes ostéo-tendineux (disparition si toxicité) + fréquence respiratoire (> 12/min) + diuresis (> 25 mL/h) + serum magnesium (target 2–3.5 mmol/L) + antidote for overdose: calcium gluconate 1 g IV slowly
• Blood pressure control: Objective: PAS Less than 160 mmHg plus PAD < 110 mmHg → labetalol IV 20–80 mg bolus (or infusion) + or hydralazine 5–10 mg IV + or immediate-release nifedipine 10–20 mg PO + avoid ACE inhibitors and ARBs (contraindicated in pregnancy)
• Transfusions and blood products: platelet concentrates if platelets < 20,000/µL (or < 50,000/µL before cesarean) + fresh frozen plasma if DIC + fibrinogen if fibrinogen Less than 1.5 g/L
• High-dose corticosteroids (dexamethasone): used to accelerate fetal lung maturation if premature (betamethasone or dexamethasone 12 mg IM x 2 / 24 h) + some studies suggest a benefit for maternal thrombocytopenia (dexamethasone 10 mg IV x 2/12 h) but the data are contradictory → non-standard use for maternal indication alone
• Mode of delivery Vaginal delivery preferred if cervical conditions are favorable and there are no obstetric contraindications. Case-by-case decision with the obstetrics team. Cesarean section if fetal emergency, unfavorable cervix, or severe prematurity.

Consult at Clinique Omicron

Clinique Omicron's physicians and nurse practitioners (NPs) provide prenatal care, including monitoring blood pressure, proteinuria, and edema at each appointment. They also prescribe urgent testing (CBC + platelets + LDH + transaminases + bilirubin + creatinine) if HELLP syndrome is suspected, immediately refer patients to tertiary-level obstetrical emergencies upon suspicion of diagnosis, provide early postpartum follow-up (7–10 days) to screen for postnatal HELLP, and educate at-risk women (history of pre-eclampsia or HELLP) on warning signs during future pregnancies. Consultations are available at several service locations in Quebec and via telemedicine. To book an appointment, visit cliniqueomicron.ca.

The content of this page is provided for informational purposes only and is not a substitute for the advice of a physician, obstetrician, or maternal-fetal medicine specialist. HELLP syndrome is an obstetric emergency requiring immediate hospitalization in a tertiary care center with neonatal intensive care. The only curative treatment is delivery.