Approach Considerations
The chart below illustrates an overview of the management of HELLP syndrome:
Overview of the Management of HELLP Syndrome
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.
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.
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. [18, 9]
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. [18, 9]
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. [22] 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. [30] Intravenous glucocorticoids appear superior to intramuscular steroids [43] and are dose-dependent. [44] 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. [22]
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. [22]
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.
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 [24]
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. [22]
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. [24] The type of skin incision and primary versus secondary skin closure do not affect wound complications. [45] 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. [24, 22]
Postpartum Curettage
Postpartum curettage lowers the mean arterial pressure and improves oliguria and thrombocytopenia. [2]
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 [24, 2]
Activity
Patients should be on bedrest. If hepatic hematoma is present, the risk of trauma should be minimized.
Consultations
Consider consultations with the following:
-
Surgeon
-
Hematologist
-
Renal specialist
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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Overview of the Management of HELLP Syndrome
Tables
|
Class 1 (Severe) |
Class 2 (Moderate) |
Class 3 (Mild) |
Platelets |
≤50,000/µL |
50,000-100,000/µL |
100,000-150,000/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 bleeding |
13% |
8% |
No increased risk |
Laboratory Test |
Possible Result |
Cause |
Recovery to Baseline (in Number of Hours or Days Postpartum) |
Haptoglobin |
↓ |
Hemolysis |
24-30 hours |
LDH |
↑ |
Hemolysis or liver dysfunction |
3-5 days |
AST or ALT |
↑ |
Liver dysfunction |
3-5 days |
Bilirubin |
↑ |
Hemolysis |
- |
Platelets (CBC) |
↓ |
Consumption |
6-11 days |
Hemoglobin/Hematocrit (CBC) |
↓ |
Hemolysis |
- |
PT |
Normal |
|
|
PTT |
↑ |
Liver dysfunction |
- |
D-dimer |
↑ |
Increased coagulation and secondary fibrinolysis |
- |
Fibrinogen |
↓ |
- |
