What is Hemolysis, Elevated Liver enzymes, and Low Platelet count (HELLP) syndrome?

From the Guidelines

HELLP syndrome is a serious pregnancy complication characterized by Hemolysis, Elevated Liver enzymes, and Low Platelet count, and its definitive treatment is delivery of the baby, regardless of gestational age, to prevent maternal and fetal morbidity and mortality. This condition typically occurs in the third trimester or shortly after delivery and is considered a variant of preeclampsia 1. The diagnosis of HELLP syndrome is suspected on clinical grounds, including symptoms such as epigastric pain, upper abdominal tenderness, proteinuria, hypertension, jaundice, and nausea and vomiting, and is confirmed by laboratory investigations, including evidence of hemolytic anemia, thrombocytopenia, and increased liver enzyme activity 1.

Key Characteristics and Complications

• Hemolysis, characterized by a low hemoglobin level and evidence of microangiopathic hemolytic anemia on peripheral blood smear
• Elevated Liver enzymes, including alanine aminotransferase and aspartate aminotransferase
• Low Platelet count, often below 20,000/μL
• Complications may include disseminated intravascular coagulation, placental abruption, acute kidney injury, pulmonary edema, and even maternal death 1

Management and Treatment

• Delivery of the baby is the definitive treatment for HELLP syndrome, and should be done as soon as possible, regardless of gestational age, to prevent maternal and fetal morbidity and mortality
• Prior to delivery, management includes blood pressure control with medications like labetalol or hydralazine, seizure prevention with magnesium sulfate, and corticosteroids to accelerate fetal lung maturity if the pregnancy is less than 34 weeks
• Close monitoring of maternal laboratory values, blood pressure, and fetal well-being is essential, and patients may require blood products, including platelets, if counts fall below 20,000/μL or if bleeding occurs 1

Prognosis and Outcome

• The condition typically resolves within days after delivery, though some women may experience worsening symptoms before improvement
• Maternal mortality rates for HELLP syndrome can be as high as 3.4%, emphasizing the importance of prompt recognition and intervention 1

From the Research

Definition and Characteristics of HELLP Syndrome

• HELLP syndrome is a serious complication in pregnancy characterized by hemolysis, elevated liver enzymes, and low platelet count, occurring in 0.5 to 0.9% of all pregnancies and in 10-20% of cases with severe preeclampsia 2, 3.
• The syndrome may be complete or incomplete, and about 70% of cases develop before delivery, with the majority occurring between the 27th and 37th gestational weeks 2, 3.
• The Tennessee Classification System diagnostic criteria for HELLP include hemolysis with increased LDH (> 600 U/L), AST (>or= 70 U/L), and platelets < 100 x 10(9)/L 2.

Diagnosis and Complications

• Clinical diagnosis can be difficult due to the lack of specific symptoms, but abdominal pain or vomiting during the third trimester should raise suspicion for HELLP syndrome 4.
• Biological criteria for diagnosis include hemolysis, hepatic dysfunction, and thrombocytopenia, with the evolution of these parameters being a major prognostic factor 4.
• Maternal morbidity is increased with HELLP syndrome, with complications such as eclampsia, placental abruptio, disseminated intravascular coagulation, pulmonary edema, acute renal insufficiency, and subcapsular liver hematoma 4.

Management and Treatment

• The management of HELLP syndrome requires termination of pregnancy as soon as possible, either by cesarean section or vaginal delivery if cervical conditions are optimal 4, 5.
• Delivery is indicated if HELLP syndrome occurs after 34 gestational weeks or if fetal and/or maternal conditions deteriorate 2, 3, 5.
• Standard corticosteroid treatment is of uncertain clinical value in maternal HELLP syndrome, but may be considered for fetal lung maturation in gestational ages between 24 and 34 weeks 2, 3, 5.
• Close surveillance of the mother should be continued for at least 48 hours after delivery, with a focus on postpartum care and physiologic and emotional wellbeing 6.