HELLP syndrome

HELLP syndrome is considered to be a severe form of preeclampsia (sometimes called "atypical preeclampsia") characterized by hemolysis (H), also expressed as microangiopathic hemolytic anemia, elevated liver enzymes (EL), and low platelets (LP).

The condition usually occurs antepartum, between 27 and 37 weeks' gestation; 15% to 30% of cases present initially postpartum. HELLP syndrome poses significant diagnostic and therapeutic challenges because only 80% to 85% of affected people present typically with hypertension and proteinuria.

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

The disease is associated with progressive and sometimes rapid maternal and fetal deterioration.

Early detection and aggressive management with a combination of intravenous magnesium sulfate, intravenous dexamethasone, control of blood pressure to prevent or minimize severe systolic hypertension, replacement of blood products, as needed, and timely delivery of the fetus and placenta seem 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 on 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 hematoma/infarction/rupture, acute pancreatitis) and maternal mortality can be avoided.

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

HELLP syndrome is a severe form of preeclampsia characterized by hemolysis (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, liver transaminases (aspartate aminotransferase [AST], alanine aminotransferase) should be elevated >70 IU/L, or twice the upper limit of normal concentration not accounted for by alternative diagnoses, and the platelet count should be <100,000/mm³.[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 Hemolysis may be indicated by elevated total bilirubin (>1.2 mg/dL), LDH and AST elevations, characteristic findings (schistocytes) on a peripheral blood smear, hematuria, worsening anemia, and a low serum haptoglobin.