Liver Disease in Pregnancy

Rowen K. Zetterman, MD


April 08, 2013

In This Article

HELLP (Hemolysis, Elevated Liver tests, Low Platelets) Syndrome

HELLP syndrome is characterized by hemolysis, abnormal aminotransferases, and a low platelet count. It occurs in 5% of women with preeclampsia, although it can develop as a separate entity in the absence of preeclampsia.[29] HELLP typically occurs during the third trimester of pregnancy, occasionally during the second trimester, or in the immediate postpartum period.[30,31] Risk factors include advanced maternal age, multiparity, and antiphospholipid syndrome.[32,33]

Maternal mortality is uncommon,[34] although it is reported to be as high as 22% with hepatic rupture.[35] Fetal mortality is 6%-22%.[19,36] HELLP syndrome recurs in up to 20% of subsequent pregnancies.[37]

Right upper quadrant pain, nausea, vomiting, malaise, and signs of preeclampsia (edema, hypertension, and proteinuria) are present in up to 80% of patients.[31] Disseminated intravascular coagulation (DIC) and pulmonary edema are rare.

Laboratory findings in HELLP syndrome include:

Evidence of hemolysis, such as lactate dehydrogenase levels > 600 IU/L;

Microangiopathic peripheral blood smear (burr cells and schizocytes);

Elevated aminotransferase levels (> 70 IU/L); and

Thrombocytopenia (platelet count < 100,000/mL).

Platelet counts may continue to decline for a few days following delivery before recovering. Coagulation parameters are normal unless DIC develops, causing elevations of the international normalized ratio (INR), fibrin split products, and D-dimer levels. Elevated uric acid levels are associated with increased maternal and fetal mortality.[36]

Maternal complications of HELLP syndrome include DIC, placental abruption, acute tubular necrosis, adult respiratory distress syndrome, pancreatitis, liver hematoma, hepatic failure, and retinal detachment.[27] Fetal complications include placental insufficiency, premature delivery, low birth weight, and risk for fetal death.

Delivery should be immediately considered if gestation is > 34 weeks or if there is evidence of fetal distress or placental disruption. Coagulation parameters should be corrected, and platelet transfusions should be provided if the platelet count is < 20,000/mL. Hypertension can be controlled with medications (labetalol, nifedipine, or hydralazine) and systemic steroids can be administered to promote fetal lung maturity. Laboratory abnormalities tend to normalize within 48 hours of delivery. HELLP and preeclampsia can recur in subsequent pregnancies.[37]