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HELLP Syndrome Treatment & Management

  • Author: Huma Khan, MD; Chief Editor: Ronald M Ramus, MD  more...
 
Updated: Dec 30, 2015
 

Approach Considerations

The chart below illustrates an overview of the management of HELLP syndrome:

Overview of the Management of HELLP Syndrome Overview of the Management of HELLP Syndrome
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Stabilization

Management of HELLP syndrome begins with early and immediate recognition of the diagnosis. Stabilization of the patient begins in the prehospital setting. If seizures and hypertension are present, both should be controlled en route to the hospital.

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Emergency Department Care

Early recognition of HELLP syndrome begins with a close look at the history, vital signs, and physical examination findings. Emergency department management includes seizure prophylaxis, hypertension control, repletion of blood products, as indicated, and general stabilization of patient condition. See section Medications for a more complete pharmacologic guide.

Intravenous fluids should be given cautiously. Patients with HELLP syndrome may be volume overloaded and present with edema but are in fact intravascularly depleted.

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Seizure Prophylaxis

Intravenous magnesium sulfate is given until 24 hours after delivery, at which point it can be stopped if the maternal condition is improved. The dosage for magnesium sulfate IV is a 6-g loading dose over 20 minutes with a 2-g per hour maintenance dose.[16, 9]

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Treatment of Hypertension

Hypertension is managed similarly to hypertension in preeclampsia. The blood pressure (BP) goal is to keep the systolic at 160 or less and the diastolic at 105 or less. Labetalol and hydralazine are the recommended drugs to treat a hypertensive crisis.[16, 9]

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Corticosteroid Therapy

Although controversial, corticosteroids can be given as a treatment regimen for antepartum and postpartum management in patients with HELLP. Steroids are theorized to alter the degree of intravascular endothelial injury and prevent further hepatocyte death and platelet activation.[20] While evidence of maternal improvement is limited, studies have demonstrated improved laboratory findings, including improved platelet counts, liver function, blood pressure, and urine output with the use of high-dose dexamethasone.[27] Intravenous glucocorticoids appear superior to intramuscular steroids[40] and are dose-dependent.[41] Therefore, aggressive therapy with high-dose dexamethasone has been recommended over the standard regimens used for enhancing fetal lung maturity. Steroids are also believed to improve fetal morbidity by reducing the incidence of respiratory distress syndrome and intraventricular hemorrhage, as well as maternal morbidity.[20]

Dosing for high-risk patients with severe disease (platelet count < 20,000 or CNS dysfunction): 20 mg IV dexamethasone every 6 hours for up to 4 doses

Dosing for all other patients with HELLP syndrome: 10 mg IV dexamethasone every 6 hours for 2 doses then 6 mg IV dexamethasone every 6 hours for 2 doses

If no clinical or laboratory improvement, expedite delivery.[20]

Steroids can be initiated in the emergency department and continued on an inpatient basis.

Once stabilized, the patient should be transferred to a labor and delivery unit under an obstetrician’s care, where she can be closely monitored.

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Delivery

Delivery: Delivery (either vaginal delivery or cesarean section) is indicated if HELLP syndrome occurs close to 34 weeks’ gestation, in the setting of fetal lung maturity, or upon evidence of significant maternal or fetal distress before 34 weeks’ gestation. Steroids administered antenatally may increase the platelet count so that regional anesthesia can be given. General guidelines are as follows:

  • If at 34 weeks’ gestation or later and unstable, deliver immediately
  • If at 34 weeks’ gestation or later and stable, consider administration of steroids; evaluate over 24-48 hours and deliver
  • If at 24-34 weeks’ gestation and stable, consider administration of steroids; wait 24-48 hours and evaluate for delivery based on the maternal-fetal condition
  • If 34 weeks’ gestation or earlier with evidence of maternal or fetal distress, deliver immediately[22]

Cesarean versus vaginal delivery should be determined based on the following:

  • Cervical ripening
  • Fetal nonstress test or biophysical profile results
  • Umbilical artery Doppler study

If there is a preterm gestation with intrauterine growth restriction or significantly abnormal Doppler test results, cesarean section should be performed.[20]

Cesarean section

Prior to performing a cesarean section, severe thrombocytopenia should be corrected. While platelets should be transfused, caution is advised owing to increased consumption of these platelets. A recommended dose is 6-10 U of platelets with thrombocytopenia of less than 40,000/µL. General anesthesia should be performed for thrombocytopenia of less than 75,000/µL.[22] The type of skin incision and primary versus secondary skin closure do not affect wound complications.[42] Hematoma formation at the operative site occurs in 20% of cases. To reduce this risk, an open bladder flap is recommended, and a subfascial drain can be used for 24-48 hours.[22, 20]

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Postpartum Curettage

Postpartum curettage lowers the mean arterial pressure and improves oliguria and thrombocytopenia.[2]

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Treatment of Hepatic Hematoma

Hepatic hematoma usually manifests as a subcapsular hematoma.

If it is not ruptured, it may be managed conservatively with close hemodynamic and coagulation status monitoring. Avoid any increase in intraabdominal pressure, including emesis, palpation, or trauma in transport. Serial CT scans or careful ultrasonography should guide management.

Rupture most often involves the anterior superior portion of the right upper lobe. Patients may present with shoulder pain, ascites, respiratory distress, shock, or fetal demise. Immediate intervention is mandated for a ruptured hematoma. Treatment considerations are as follows:

  • Resuscitate: Transfuse and correct any coagulopathy with fresh frozen plasma or platelets
  • Emergent laparotomy by general or vascular surgeon: Based on extent of bleeding, may pack and drain or surgically ligate hepatic segments that are bleeding, embolize the hepatic artery to the affected hepatic segment, and loosely suture the omentum or surgical mesh to the liver; hepatic lobectomy or liver transplantation in patients with total hepatic necrosis may also be indicated in some cases[22, 2]
  • Owing to exsanguination and coagulopathy, maternal and fetal mortality rates are close to 50%, even with treatment[22, 20]
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Activity

Patients should be on bedrest. If hepatic hematoma is present, the risk of trauma should be minimized.

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Consultations

Consider consultations with the following:

  • Surgeon
  • Hematologist
  • Renal specialist
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Transfer

Maternal and fetal conditions can deteriorate rapidly, necessitating a closely monitored unit under an experienced obstetrician’s care. Patients, once stabilized and if remote from term, should be transferred to a tertiary care facility, where such monitoring can be provided.

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Long-Term Monitoring

Repeat laboratory tests every 6-12 hours until improvement noted. Monitor for remission.[20]

Consider steroids for up to 2 days postpartum to prevent rebound of liver dysfunction, thrombocytopenia, and oliguria.[2]

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Contributor Information and Disclosures
Author

Huma Khan, MD Resident Physician, Department of Emergency Medicine, Northshore-Long Island Jewish Medical Center

Disclosure: Nothing to disclose.

Coauthor(s)

Natalie B Meirowitz, MD Assistant Professor of Obstetrics and Gynecology, Albert Einstein College of Medicine; Chief of Maternal-Fetal Medicine, Director, Center for Maternal-Fetal Health, Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Northshore-Long Island Jewish Medical Center

Natalie B Meirowitz, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, Society for Maternal-Fetal Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Ronald M Ramus, MD Professor of Obstetrics and Gynecology, Director, Division of Maternal-Fetal Medicine, Virginia Commonwealth University School of Medicine

Ronald M Ramus, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American Institute of Ultrasound in Medicine, Medical Society of Virginia, Society for Maternal-Fetal Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Jordan G Pritzker, MD, MBA, FACOG Adjunct Professor of Obstetrics/Gynecology, Hofstra North Shore-LIJ School of Medicine at Hofstra University; Attending Physician, Department of Obstetrics and Gynecology, Long Island Jewish Medical Center; Medical Director, Aetna, Inc; Private Practice in Gynecology

Disclosure: Nothing to disclose.

References
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Overview of the Management of HELLP Syndrome
Table 2: Mississippi Classification of HELLP Syndrome
 Class 1 (Severe)Class 2 (Moderate)Class 3 (Mild)
Platelets≤50,000/µL50,000-100,000/µL100,000-150,000/µL
AST or ALT≥70 IU/L≥70 IU/L≥40 IU/L
LDH≥600 IU/L≥600 IU/L≥600 IU/L
Incidence of bleeding13%8%No increased risk
Table 1: Laboratory Findings in HELLP Syndrome
Laboratory TestPossible ResultCauseRecovery to Baseline (in Number of Hours or Days Postpartum)
HaptoglobinHemolysis24-30 hours
LDHHemolysis or liver dysfunction3-5 days
AST or ALTLiver dysfunction3-5 days
BilirubinHemolysis-
Platelets (CBC)Consumption6-11 days
Hemoglobin/Hematocrit (CBC)Hemolysis-
PTNormal  
PTTLiver dysfunction-
D-dimerIncreased coagulation and secondary fibrinolysis-
Fibrinogen-
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