HELLP Syndrome

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Chapter 210 HELLP Syndrome

INTRODUCTION

Description: Hemolysis, elevated liver enzymes, low platelet count (HELLP) syndrome is a variant of pregnancy-induced hypertension (PIH) and preeclampsia, which are dominated by hepatic and hematologic changes.

Prevalence: Six percent to eight percent of pregnancies, up to 20% of patients with severe pre-eclampsia.

Predominant Age: Reproductive.

Genetics: No genetic pattern.

ETIOLOGY AND PATHOGENESIS

Causes: Unknown. Genetic, endocrine/metabolic (including altered prostaglandin production), uteroplacental ischemia, immunologic.

Risk Factors: Nulliparity, age older than 40 years, African American race, family history of pregnancy-induced hypertension, renal disease, antiphospholipid syndrome, diabetes mellitus, multiple gestation. Chronic hypertension increases the risk of pregnancy-induced hypertension.

CLINICAL CHARACTERISTICS

Signs and Symptoms

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

• Right upper quadrant or epigastric pain

DIAGNOSTIC APPROACH

Workup and Evaluation

Laboratory: Liver and renal function studies (e.g., enzymes, renal clearance, 24-hour urinary protein), platelet counts, clotting studies. (Platelet counts of >50,000/mm³ generally are not associated with spontaneous bleeding.)

Imaging: Ultrasonography to monitor fetal growth (frequently restricted).

Special Tests: Assessment of fetal lung maturation may be performed, but if maternal disease is severe, management is based on maternal factors and not fetal maturation.

Diagnostic Procedures: Measurement of blood pressure, laboratory confirmation.

Pathologic Findings

HELLP syndrome is a multiorgan process, including the renal, hepatic, hematologic, and nervous systems.

MANAGEMENT AND THERAPY

Nonpharmacologic

General Measures: Evaluation, support, and preparation for delivery.

Specific Measures: Patients with HELLP syndrome often represent the sickest patients with pre-eclampsia or eclampsia. The only true treatment is delivery. The presence of HELLP syndrome generally militates against conservative treatment for any but the briefest stabilization period.

Diet: No specific dietary changes indicated.

Activity: No restriction.

Patient Education: American College of Obstetricians and Gynecologists Patient Education Pamphlet AP034 (High Blood Pressure During Pregnancy).

Drug(s) of Choice

For mild to moderate chronic hypertension, α-methyldopa is considered first-line therapy.

During labor or labor induction, magnesium sulfate is often used to reduce the chance of seizures (4 g IV for 20 minutes, then 2 to 3 g/hour IV continuous infusion; therapeutic range 4 to 8 mg/dL).

If blood pressure >180 torr systolic or 110 torr diastolic: hydralazine HCl 5 to 10 mg IV bolus every 20 minutes as needed or labetalol 20 mg IV bolus every 10 minutes as needed to a maximum of 300 mg in 24 hours. (Sodium nitroprusside may be used for extreme disease.)

Steroids have been advocated, but there is insufficient evidence to conclude that they are beneficial.

Contraindications: Angiotensin-converting enzyme (ACE) inhibitors are teratogenic and are contraindicated in pregnancy. Diuretics should be avoided in pregnancy because of the possibility of adverse fetal effects caused by reduced plasma volume. (Despite the common occurrence of edema, these patients have constricted circulatory volume.)

Precautions: Central hemodynamic monitoring should be considered if blood pressure is high or potent agents are used.

Alternative Drugs

Verapamil or nifedipine may also be used to acutely reduce blood pressure.

FOLLOW-UP

Patient Monitoring: Increased maternal and fetal surveillance, antenatal testing.

Prevention/Avoidance: The value of low-dose aspirin therapy or calcium supplementation remains unproved.

Possible Complications: Maternal—cardiac decompensation, stroke, pulmonary edema (10%) and respiratory failure, renal failure (5%), seizures and seizure-related injuries (6%), intracranial hemorrhage, coma, and death (0.5% to 5% mortality). Fetal risk (growth restriction and death) is directly proportional to both the degree of proteinuria and the level of maternal diastolic blood pressure. Placental abruption may occur in up to 10% of cases.

Expected Outcome: HELLP syndrome generally resolves after delivery, but the risk of recurrence with future pregnancies or elevated blood pressure in later life is increased.

MISCELLANEOUS

ICD-9-CM Code: 642.5.

Barrilleaux PS, Martin JNJr, Klauser CK, et al. Postpartum intravenous dexamethasone for severely preeclamptic patients without hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome: a randomized trial. Obstet Gynecol. 2005;105:843.

Fonseca JE, Mendez F, Catano C, Arias F. Dexamethasone treatment does not improve the outcome of women with HELLP syndrome: a double-blind, placebo-controlled, randomized clinical trial. Am J Obstet Gynecol. 2005;193:1591.

Ganzevoort W, Rep A, Bonsel GJ, et al. PETRA Investigators: A randomised controlled trial comparing two temporising management strategies, one with and one without plasma volume expansion, for severe and early onset pre-eclampsia. BJOG. 2005;112:1358.

Isler CM, Barrilleaux PS, Magann EF, et al. A prospective, randomized trial comparing the efficacy of dexamethasone and betamethasone for the treatment of antepartum HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome. Am J Obstet Gynecol. 2001;184:1332.

Matchaba P, Moodley J. Corticosteroids for HELLP syndrome in pregnancy. Cochrane Database Syst Rev. 2004. CD002076.

Thangaratinam S, Ismail KM, Sharp S, et alTests in Prediction of Pre-eclampsia Severity Review Group. Accuracy of serum uric acid in predicting complications of pre-eclampsia: a systematic review. BJOG. 2006;113:369.

American College of Obstetricians and Gynecologists. Thrombocytopenia in pregnancy. ACOG Practice Bulletin 6. Washington, DC: ACOG, 1999.

American College of Obstetricians and Gynecologists. Chronic hypertension in pregnancy. ACOG Practice Bulletin 29. Obstet Gynecol. 2001;98:177.

American College of Obstetricians and Gynecologists. Diagnosis and management of preeclampsia and eclampsia. ACOG Practice Bulletin 33. Obstet Gynecol. 2002;99:159.

Clenney TL, Viera AJ. Corticosteroids for HELLP (haemolysis, elevated liver enzymes, low platelets) syndrome. BMJ. 2004;329:270.

Geary M. The HELLP syndrome. Br J Obstet Gynaecol. 1997;104:8871.

Martin JNJr, Rose CH, Briery CM. Understanding and managing HELLP syndrome: the integral role of aggressive glucocorticoids for mother and child. Am J Obstet Gynecol. 2006;195:914. Epub 2006 May 2

O’Brien JM, Milligan DA, Barton JR. Impact of high-dose corticosteroid therapy for patients with HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome. Am J Obstet Gynecol. 2000;183:921.

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