HELLP Syndrome at Term
A 34-year-old woman at 38 weeks gestation reports severe headache. Blood pressure is 168/108 mm Hg with 2+ lower limb edema; no petechiae are seen and neurologic examination is normal. Platelets are 68 × 10⁹/L, AST 210 U/L, LDH 820 U/L, creatinine 88 µmol/L, urine protein 2+. Smear shows fragmented erythrocytes. Which of the following is the most appropriate management?
- A.Intravenous dexamethasone
- B.Therapeutic plasma exchange
- C.Eculizumab infusion
- D.Immediate deliveryCorrect
Explanation
This is HELLP syndrome, a severe variant of preeclampsia defined by hemolysis, elevated liver enzymes, and a low platelet count, and the only definitive treatment is delivery. The placenta is the source of the antiangiogenic factors, principally soluble fms-like tyrosine kinase 1, that produce widespread endothelial injury, so removing it is curative. All the diagnostic elements are present: schistocytes and an LDH of 820 U/L indicate microangiopathic hemolysis, AST of 210 U/L reflects periportal hepatocellular necrosis, and platelets of 68 × 10⁹/L reflect consumption on damaged endothelium. At 38 weeks there is no fetal reason to delay, and expectant management risks abruption, hepatic hematoma or rupture, disseminated intravascular coagulation, eclampsia, and stroke. Alongside delivery, intravenous magnesium sulfate is given for seizure prophylaxis and antihypertensives such as labetalol, hydralazine, or nifedipine are used to bring severe-range pressures down. Corticosteroids are given before 34 weeks for fetal lung maturity, but they do not reverse maternal HELLP and are irrelevant at 38 weeks. Plasma exchange is the treatment for thrombotic thrombocytopenic purpura, which would show far more profound thrombocytopenia, neurologic signs, and severely deficient ADAMTS13 activity. Eculizumab treats complement-mediated atypical hemolytic uremic syndrome, typically dominated by acute kidney injury that persists after delivery. The distinction matters, because the pregnancy-associated microangiopathies are separated by whether delivery resolves them.
Why each option
- A.
- Corticosteroids are given before 34 weeks for fetal lung maturity and do not reverse maternal HELLP syndrome; at 38 weeks they add nothing.
- B.
- Plasma exchange treats thrombotic thrombocytopenic purpura, which shows severe thrombocytopenia, neurologic features, and ADAMTS13 activity below 10 percent.
- C.
- Eculizumab is used for complement-mediated atypical hemolytic uremic syndrome, usually with prominent acute kidney injury persisting after delivery.
- D.
- Correct. Delivery removes the placenta, the source of the antiangiogenic injury, and is the definitive treatment for HELLP syndrome at term.
Reference: ACOG Practice Bulletin No. 222, Gestational Hypertension and Preeclampsia, 2020
This is one of 3,009 questions in the MedBoardSA SMLE bank, with timed test mode, tutor mode, and progress tracking by topic.
Practise the full bank