Describe HELLP syndrome — its aetiology, clinical features and management.

Definition

HELLP syndrome is a severe variant of pre-eclampsia characterised by the triad of Haemolysis, Elevated Liver enzymes and Low Platelets. It may occur with only mildly raised blood pressure and is associated with high maternal and perinatal mortality.

Diagnostic Criteria

• Haemolysis — abnormal peripheral smear (schistocytes, burr cells), raised serum bilirubin (≥ 1.2 mg/dL) and raised LDH (> 600 IU/L), low haptoglobin.
• Elevated liver enzymes — ast/ALT raised (≥ twice normal).
• Low platelets — < 100,000/mm³.

Clinical Features

Malaise, nausea and vomiting, epigastric or right-upper-quadrant pain, and features of pre-eclampsia. It may be mistaken for hepatitis or gastritis.

Complications

DIC, abruptio placentae, acute renal failure, subcapsular liver haematoma and rupture, pulmonary oedema, eclampsia and fetal death.

Classification

The Tennessee system divides HELLP into complete (all three criteria met) and partial (one or two criteria). The Mississippi system grades severity by the platelet count — Class 1 (≤ 50,000/mm³), Class 2 (50,000–100,000) and Class 3 (100,000–150,000).

Differential Diagnosis

Acute fatty liver of pregnancy, thrombotic thrombocytopenic purpura/haemolytic-uraemic syndrome, viral hepatitis, gestational thrombocytopenia and cholecystitis.

Management

• Stabilise as for severe pre-eclampsia — MgSO₄ for seizure prophylaxis and control of blood pressure.
• Correct coagulopathy — transfuse platelets and fresh frozen plasma as required; corticosteroids may transiently improve the platelet count.
• Delivery is the definitive treatment, irrespective of gestation once the mother is stabilised; corticosteroids for lung maturity if preterm.
• Continue intensive monitoring in the puerperium, when the platelet count may fall further before recovering (usually within 48–72 hours).

A Note on WHY Hellp Can Occur Without Severe Hypertension

A dangerous trap worth understanding is why HELLP syndrome is so often missed — because the blood pressure may be only modestly raised and the proteinuria minimal. Clinicians naturally look for severe hypertension as the marker of severe pre-eclampsia; but HELLP is a variant in which the endothelial damage expresses itself chiefly in the liver and the blood rather than in the blood pressure. The woman therefore presents with epigastric or right upper quadrant pain (from stretching of the liver capsule), nausea, vomiting and malaise — and is misdiagnosed as gastritis, hepatitis, cholecystitis or simply 'indigestion of pregnancy', while the underlying process (Haemolysis, Elevated Liver enzymes, Low Platelets) advances. The consequences are severe: DIC, abruption, acute renal failure, pulmonary oedema and — most feared — subcapsular haematoma and rupture of the liver, which is frequently fatal. This is why epigastric pain in any woman in the second half of pregnancy demands a full blood count, platelet count, liver enzymes and a peripheral smear, regardless of her blood pressure. Understanding that HELLP is a laboratory diagnosis which may hide behind an unimpressive blood pressure explains why it must be actively looked for, and why its presence mandates delivery.

May occur without marked hypertension — easily missed.

• HELLP = Haemolysis (schistocytes, ↑LDH, ↑bilirubin), Elevated Liver enzymes (ast/ALT), Low Platelets (<100,000) — a severe variant of pre-eclampsia.
• The blood pressure may be only modestly raised — epigastric/RUQ pain, nausea and malaise are the key symptoms.
• Complications: DIC, abruption, renal failure, pulmonary oedema, and subcapsular liver haematoma/rupture.
• Management: stabilise, give MgSO4 and antihypertensives, correct the coagulopathy, and deliver (the only cure); corticosteroids for fetal lungs if preterm.