Partial HELLP Syndrome

Partial HELLP syndrome is the presence of one or two of the three features of HELLP syndrome (haemolysis, elevated liver enzymes, low platelets) in a woman with preeclampsia. It lies between severe preeclampsia and full HELLP syndrome. It matters because some women progress to full HELLP syndrome within days.

• Often presents with epigastric pain and raised liver enzymes only.
• Repeat blood tests are essential.
• Managed in a facility able to deliver quickly.

Women have epigastric or right upper quadrant pain, nausea, malaise, headache or visual change, with hypertension in most. Others have no symptoms.

• Mild liver tenderness.
• Blood pressure may be normal in some women.
• Differential diagnosis: preeclampsia with severe features, early HELLP syndrome, acute fatty liver of pregnancy, viral hepatitis, intrahepatic cholestasis, thrombotic thrombocytopenic purpura, cholecystitis, gastritis, malaria.
• Complications: progression to full HELLP syndrome, abruption, eclampsia, disseminated intravascular coagulation, liver haematoma, preterm birth, stillbirth, maternal death.

The diagnosis needs preeclampsia with one or two of the three HELLP criteria.

• Haemolysis: fragmented red cells, bilirubin 20 micromol/L or more, lactate dehydrogenase 600 IU/L or more.
• Elevated liver enzymes: aspartate aminotransferase 70 IU/L or more.
• Low platelets: below 100 x 10^9/L.
• Repeat full blood count, liver enzymes, lactate dehydrogenase, creatinine and coagulation screen every 6 to 12 hours; liver ultrasound; cardiotocography and growth scan.

The principle is intensive monitoring, magnesium sulphate and blood pressure control, with delivery if the syndrome progresses or at 34 weeks.

• Supportive: admit; left lateral rest; blood pressure 4-hourly; total fluids 80 mL per hour if severe; urinary catheter with hourly output when on magnesium; avoid NSAIDs and IM injections when platelets are falling.
• Pharmacological: magnesium sulphate 4 g IV over 20 minutes then 1 g per hour for 24 hours after birth; stop for absent reflexes, respiratory rate below 12 per minute or urine below 30 mL per hour and give calcium gluconate 1 g IV over 10 minutes for toxicity; labetalol 200 mg oral every 8 to 12 hours (maximum 2.4 g daily) or nifedipine modified release 30 to 60 mg oral once daily (maximum 120 mg) or methyldopa 250 to 500 mg oral 8-hourly (maximum 3 g daily); labetalol 20 mg IV over 2 minutes then 40 mg, 80 mg and 80 mg at 10 minute intervals (maximum 220 mg) or nifedipine immediate release 10 mg oral repeated once after 30 minutes or hydralazine 5 mg IV then 5 to 10 mg every 20 to 30 minutes (maximum 20 mg) for 160/110 mmHg or more; betamethasone 12 mg IM twice, 24 hours apart, before 34 weeks; oxytocin 10 IU IM after birth and no ergometrine.
• Procedures: delivery at 34 weeks, earlier for progression to full HELLP syndrome, abruption, eclampsia, pulmonary oedema, uncontrolled hypertension or fetal compromise; induction with misoprostol 25 micrograms vaginal 6-hourly (maximum 4 doses) or Foley catheter, or caesarean section; platelet transfusion for below 50 x 10^9/L before caesarean section.
• Special groups: kidney disease reduces magnesium sulphate to 0.5 g per hour with level monitoring; asthma uses nifedipine instead of labetalol.
• Red flags: platelets falling below 100 x 10^9/L, rising enzymes or lactate dehydrogenase, severe epigastric pain, seizure, bleeding, blood pressure 160/110 mmHg or more.
• Follow-up: repeat blood tests every 6 to 12 hours until delivery and daily after; blood pressure at 6 hours, daily for 5 days, then at 2 and 6 weeks; review at 6 weeks and aspirin 150 mg oral at night from 12 weeks in the next pregnancy.