• HELLP is a syndrome of pregnancy characterized by Hemolysis, Elevated Liver enzymes, and Low platelets.
• Diagnostic criteria for HELLP include thrombocytopenia (platelet count <100,000/L), AST and/or ALT >70 IU/L, and hemolysis with LDH >600 IU/L.
• Twenty percent of patients with severe preeclampsia develop HELLP but the syndrome can occur without hypertension (HTN) and/or proteinuria.
• Signs of HELLP usually manifest between 28 and 36 weeks gestation; however, ~20% of patients will show initial manifestations at >48 hours after delivery. Resolution of symptoms usually occurs within 48 hours postpartum but can take up to 4 weeks.

• Etiology unclear; HELLP is considered a severe form of preeclampsia.
• Maternal risk factors for preeclampsia
  • Demographics: Age >40 years old, African-American ethnicity
  • Pregnancy associated: Nulliparity, multiple gestations, molar pregnancy
  • Comorbidities: HTN, renal disease, diabetes mellitus, factor V Leiden deficiency, antiphospholipid antibody
• Paternal risk factors for preeclampsia: History of fathering a preeclamptic pregnancy with another woman; history of having a mother with preeclampsia during his gestation
• HELLP recurrence is rare (~3%).

• Theories include abnormal implantation of trophoblastic tissue and imbalanced production of mediators. An atypical maternal immune response to embryonic trophoblasts can result in impaired invasion of trophoblastic cells into the uterine wall. This results in maladaptation of myometrial spiral arteries (high-resistance, and hence reduced uteroplacental flow). The hypoperfusion can cause placental hypoxia and the release of inflammatory cytokines into the maternal circulation with consequent endothelial dysfunction.
• An imbalance of placental production of prostacyclin and thromboxane can result in systemic vasoconstriction and placental ischemia.

• Control HTN (target SBP <160 mm Hg; DBP <110 mm Hg).
• Seizure prophylaxis
• Accelerate fetal lung maturity for gestational age <36 weeks with betamethasone if preterm delivery anticipated.
• Evaluate for hemorrhage risk factors including thrombocytopenia, coagulopathy, and hepatic dysfunction.
• Identify increased risk of hepatic rupture including thrombocytopenia, elevated liver enzymes, and right upper quadrant pain.
• Prepare for urgent/emergent delivery. Indications for emergent delivery include non-reassuring fetal heart tones lasting >10 minutes, placental abruption, hepatic rupture, seizure, and/or end-organ damage.

• Pulmonary: Dyspnea.
• Hepatic: Abdominal pain, ascites, jaundice.
• Hematologic: History of petechiae, mucosal bleeding, bruising.
• Neurologic: Hyperexcitability, headaches, visual changes, seizure.

Preeclampsia therapy

• Antihypertensives
• Seizure prophylaxis
• Betamethasone

• Cardiovascular: Decreased CVP; increased peripheral vascular resistance, sensitivity to catecholamines; vasospasm, LV hypertrophy.
• Pulmonary: Airway edema, pulmonary edema, increased risk of gastric aspiration.
• Renal: Acute renal failure; decreased GFR, clearance of uric acid.
• Hematologic: Hemoconcentration (increased blood viscosity), thrombocytopenia, coagulopathy.
• Hepatic: Liver hematoma, hepatocellular damage, periportal hepatic necrosis.
• CNS: Risk of cerebral edema, cerebral hemorrhage, alteration in cerebral autoregulation.
• Placental: Uteroplacental insufficiency may lead to fetal hypoxia, IUGR. Increased risk of placental abruption, premature labor.

• Delivery of the fetus and placenta is the only curative therapy.
• Emergent delivery may be delayed in the absence of the following: Deterioration of clotting function (progression of thrombocytopenia and/or DIC); evidence of hepatic dysfunction, hematoma, and/or rupture; severe, refractory HTN >24 hours; renal failure; pulmonary edema, eclampsia or onset of neurologic deficits; placental abruption; nonreassuring fetal status.

• HELLP syndrome
• Preeclampsia
• Eclampsia

Consider ICU admission in patients with evidence of end-organ damage and/or need for mechanical ventilation.

• Continue antihypertensive medication until BP normalizes.
• Continue seizure prophylaxis for 24 hours following last seizure.
• Labs. Follow platelets, INR/PT, hepatic enzymes, BUN/Cr until values normalize.

Hepatic rupture or dysfunction; pulmonary edema, renal failure, thrombocytopenia, DIC, seizure, placental abruption, IUGR, preterm delivery, maternal or fetal death.

• Pregnancy Induced Hypertension
• Preeclampsia
• Eclampsia

• 642.50 Severe pre-eclampsia, unspecified as to episode of care or not applicable
• 642.51 Severe pre-eclampsia, delivered, with or without mention of antepartum condition
• 642.52 Severe pre-eclampsia, delivered, with mention of postpartum complication
• 642.53 Severe pre-eclampsia, antepartum condition or complication
• 642.54 Severe pre-eclampsia, postpartum condition or complication

• O14.20 HELLP syndrome (HELLP), unspecified trimester
• O14.22 HELLP syndrome (HELLP), second trimester
• O14.23 HELLP syndrome (HELLP), third trimester

Judith A. Turner , MD, PhD