From the Guidelines
The definitive treatment for HELLP syndrome is prompt delivery, regardless of gestational age, to reduce maternal morbidity and mortality. HELLP syndrome is a life-threatening pregnancy complication characterized by Hemolysis, Elevated Liver enzymes, and Low Platelets, with a maternal mortality rate of 3.4% as reported by Weinstein 1. The condition tends to resolve soon after delivery, but prompt recognition and management are critical to prevent complications.
Diagnosis and Management
Key diagnostic features include evidence of haemolytic anaemia, thrombocytopenia, and increased liver enzyme activity, with the degree of thrombocytopenia correlating well with the degree of liver dysfunction 1. Preoperative haematological investigations should include 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.
Treatment Approach
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. For patients undergoing Caesarean section, platelet transfusion is recommended if the platelet count is less than 50,000 mm-3, and whole blood is recommended if the haemoglobin concentration is less than 10 g% 1. A urinary catheter should be passed for hourly monitoring of urinary output to diagnose acute renal failure early.
Monitoring and Supportive Care
Close monitoring of maternal vital signs, laboratory values, and fetal status is essential. Supportive care includes magnesium sulfate for seizure prophylaxis, antihypertensives for blood pressure management, and blood products as needed. The use of suxamethonium can be useful to ensure early good intubation in patients undergoing Caesarean section 1.
From the Research
Definition and Incidence 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, with about 70% of cases developing before delivery, the majority between the 27th and 37th gestational weeks, and the remainder within 48 hours after delivery 2, 3.
Diagnosis and Classification of HELLP Syndrome
- 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 of HELLP Syndrome
- 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 fetal and/or maternal conditions deteriorate, with vaginal delivery being preferable 2, 3, 4.
- Standard corticosteroid treatment is of uncertain clinical value in the maternal HELLP syndrome, with high-dose treatment and repeated doses to be avoided for fear of long-term adverse effects on the fetal brain 2, 3, 4.
- Close surveillance of the mother should be continued for at least 48 hours after delivery, with monitoring of obstetric complications, controlling hypertension, seizure prevention, and planning for delivery 2, 3, 5.
Complications and Prognosis of HELLP Syndrome
- The syndrome is a progressive condition and serious complications are frequent, with high mortality and morbidity rates, leading to possibly life-threatening complications regarding both the mother and the fetus 2, 3, 4.
- The recognition of HELLP syndrome and an aggressive multidisciplinary approach and prompt transfer of these women to obstetric centers with expertise in this field are required for the improvement of materno-fetal prognosis 3.
- Further large, well-designed, randomized controlled trials are needed to address the role corticosteroids may play in the treatment of women with HELLP and to help improve maternal and fetal outcomes 6.