HELLP syndrome

Considered to be a severe form of pre-eclampsia (sometimes called 'atypical pre-eclampsia') characterised by haemolysis (H), also expressed as microangiopathic haemolytic anaemia, elevated liver enzymes (EL), and low platelets (LP).

Usually occurs antepartum between 27 and 37 weeks' gestation; 15% to 30% of cases present initially postpartum. Significant diagnostic and therapeutic challenge because only 80% to 85% of affected patients present typically with hypertension and proteinuria.

Should be considered in any pregnant patient presenting in the second half of gestation or immediately postpartum with significant new-onset epigastric/upper abdominal pain until proven otherwise.

Associated with progressive and sometimes rapid maternal and fetal deterioration.

Although definitions and strict cut-off values for diagnosis are often arbitrary, adherence to widely accepted diagnostic criteria facilitates scientific reporting and communication.

Early detection and aggressive management with a combination of intravenous magnesium sulfate, intravenous dexamethasone, control of blood pressure to prevent or minimise severe systolic hypertension, replacement of blood products as needed, and timely delivery of the fetus and placenta, appear to be the best and safest ways to arrest disease progression and reduce adverse outcomes. Maternal outcomes are improved considerably with this management; perinatal outcome depends predominantly upon the gestational age when delivery occurs. A critical step is the early initiation of potent glucocorticoids as soon as the diagnosis of HELLP syndrome is made, so that severe maternal morbidity (stroke, liver haematoma/infarction/rupture, acute pancreatitis) and maternal mortality can be avoided.

Although delivery is the only cure, serious manifestations of the disease continue into the immediate postpartum period.

A severe form of pre-eclampsia characterised by haemolysis (H), elevated liver enzymes (EL), and low platelets (LP) in a pregnant or puerperal patient (usually within 7 days of delivery). An affected patient often displays hypertension and proteinuria (80% to 85%), epigastric/right upper quadrant (RUQ) pain (40% to 100%), nausea, vomiting, headache, and malaise. Some patients may present without these signs/symptoms when first evaluated for unexplained thrombocytopenia. The diagnosis of HELLP syndrome should be considered in any pregnant patient presenting in the second half of gestation or immediately postpartum with significant new-onset epigastric/RUQ pain until proven otherwise. To meet diagnostic criteria,[1]Sibai BM, Ramadan MK, Usta I, et al. Maternal morbidity and mortality in 442 pregnancies with hemolysis, elevated liver enzymes and low platelets (HELLP syndrome). Am J Obstet Gynecol. 1993 Oct;169(4):1000-6. http://www.ncbi.nlm.nih.gov/pubmed/8238109?tool=bestpractice.com liver transaminases (aspartate aminotransferase [AST], alanine aminotransferase) should be elevated >70 IU/L, or twice the upper limit of normal concentration, and the platelet count should be <100 x 10⁹/L (<100,000/microlitre). Haemolysis may be indicated by elevated total bilirubin (>1.2 mg/dL [>20.5 micromol/L]), LDH and AST elevations, and characteristic findings (schistocytes) on a peripheral blood smear, haematuria, worsening anaemia, and a low serum haptoglobin.