What is HELLP (Hemolysis, Elevated Liver enzymes, and Low Platelet count) syndrome?

From the Guidelines

HELLP syndrome is a serious condition that requires immediate medical attention, and prompt delivery is desirable in the presence of worsening pre-eclampsia, gestational age at or beyond 32-34 weeks, evidence of fetal distress, or evidence of fetal maturity. The diagnosis of HELLP syndrome is suspected on clinical grounds and confirmed by laboratory investigations, including platelet count, white blood cell count, PCV, partial thromboplastin time, fibrinogen concentration, fibrin degradation products, peripheral blood smear, liver function tests, and serum concentrations of creatinine, urea, and uric acid 1.

Key Considerations

• Preoperative haematological investigations are crucial to assess the severity of the condition and guide management decisions 1
• Platelet transfusion is recommended if the platelet count is less than 50,000 mm-3 in patients undergoing Caesarean section 1
• Whole blood transfusion is recommended if the haemoglobin concentration is less than 10 g% 1
• Suxamethonium can be useful to ensure early good intubation due to its short half-life and rapid recovery 1

Management

• Early diagnosis and stabilization are essential to prevent complications and improve outcomes 1
• Prompt delivery is desirable in the presence of worsening pre-eclampsia, gestational age at or beyond 32-34 weeks, evidence of fetal distress, or evidence of fetal maturity 1
• Caesarean section rates are high in patients with HELLP syndrome, and anaesthetists should be prepared for emergency situations with limited time for investigations 1

From the Research

Definition and Characteristics of HELLP Syndrome

• HELLP syndrome is a serious complication in pregnancy characterized by haemolysis, elevated liver enzymes, and low platelet count, occurring in 0.5 to 0.9% of all pregnancies and in 10-20% of cases with severe preeclampsia 2, 3.
• The syndrome may be complete or incomplete, and about 70% of cases develop before delivery, with the majority occurring between the 27th and 37th gestational weeks 2, 3.

Diagnosis and Diagnostic Criteria

• The Tennessee Classification System diagnostic criteria for HELLP include haemolysis with increased LDH (> 600 U/L), AST (>or= 70 U/L), and platelets < 100 x 10(9)/L 2.
• The Mississippi Triple-class HELLP System further classifies the disorder by the nadir platelet counts 2.

Treatment and Management

• Conservative treatment (>or= 48 hours) is controversial, but may be considered in selected cases < 34 weeks' gestation 2, 3.
• Delivery is indicated if the HELLP syndrome occurs after the 34th gestational week or the foetal and/or maternal conditions deteriorate 2, 3.
• Vaginal delivery is preferable, and if the cervix is unfavourable, it is reasonable to induce cervical ripening and then labour 2, 3.
• Corticosteroid treatment, such as dexamethasone, may be used to reduce maternal morbidity and improve outcomes 4, 5.

Complications and Surveillance

• The syndrome is a progressive condition, and serious complications are frequent 2, 3.
• Close surveillance of the mother should be continued for at least 48 hours after delivery 2, 3.
• Blood pressure should be kept below 155/105 mmHg, and signs of intrauterine foetal distress should be monitored closely 2.