Hellp Syndrome

Introduction

• Description: Hemolysis, elevated liver enzymes, low platelet count (HELLP) syndrome is considered to be a variant of pregnancy-induced hypertension (PIH) and pre-eclampsia, which are dominated by hepatic and hematologic changes. The course of HELLP syndrome is notable for its progressive and sometimes sudden deterioration in maternal and fetal condition.

• Prevalence: 0.1%–1% of pregnancies; up to 20% of patients with severe pre-eclampsia. Most cases occur during the third trimester, but up to 30% may manifest in the postpartum period.

• Predominant Age: Reproductive age; 80% of cases are diagnosed before 37 weeks gestation.

• Genetics: No genetic pattern.

Etiology and Pathogenesis

• Causes: Unknown. Genetic, endocrine/metabolic (including altered prostaglandin production), uteroplacental ischemia, immunologic. A connection to fetal long-chain 3-hydroxyacyl CoA dehydrogenase (LCHAD) deficiency has been proposed. Complement dysregulation also has been proposed.

• Risk Factors: Older than 40 years, family history of pregnancy-induced hypertension, renal disease, antiphospholipid syndrome, diabetes mellitus, multiple gestation, past history of pre-eclampsia or HELLP syndrome. Chronic hypertension increases the risk for pregnancy-induced hypertension.

Signs and Symptoms

• Pre-eclampsia or eclampsia with hemolysis, thrombocytopenia (the degree of thrombocytopenia is predictive of the severity of the disease and the likelihood of poor outcome), elevated hepatic transaminase levels (any or all; blood pressure may be normal in up to 20% of patients)

• Right upper quadrant or epigastric pain (90%)

• Nausea, vomiting, and malaise (50%)

• Headache or visual changes (20%–60%)

Diagnostic Approach