What are the symptoms and treatment of HELLP (Hemolysis, Elevated Liver enzymes, and Low Platelet count) syndrome?

HELLP Syndrome: Symptoms and Treatment

The definitive treatment for HELLP syndrome is immediate delivery regardless of gestational age, with management including antihypertensive therapy, magnesium sulfate for seizure prophylaxis, and blood product transfusions as needed. 1

Clinical Presentation and Symptoms

HELLP syndrome presents with a constellation of symptoms that may include:

• Common symptoms:

  • Right upper quadrant or epigastric pain (characteristic) 1, 2
  • Nausea and vomiting 2
  • Headache 1
  • Malaise 1
  • Jaundice 1
  • Hypertension (may be absent in 20% of cases) 2
  • Proteinuria (may be absent in 20% of cases) 2

• Laboratory findings (diagnostic criteria):

  • Hemolysis (microangiopathic hemolytic anemia) 3
  • Elevated liver enzymes 1
  • Low platelet count (thrombocytopenia) 1

It's important to note that HELLP syndrome can occur without the classic signs of preeclampsia (hypertension and proteinuria) in approximately 20% of cases, making diagnosis challenging 2.

Diagnosis

Early detection is crucial for immediate therapeutic management 2. Any pregnant woman presenting with upper abdominal pain should be evaluated for HELLP syndrome regardless of whether preeclampsia symptoms are present 2.

Laboratory evaluation should include:

• Complete blood count with platelets
• Liver function tests
• Renal function tests
• Peripheral blood smear (to detect schistocytes indicating hemolysis)
• Coagulation studies

Treatment Approach

Immediate Management

1. Delivery: The definitive treatment for HELLP syndrome is delivery regardless of gestational age 1, 4. Conservative management has been associated with higher stillbirth rates 4.

2. Blood Pressure Control: For severe hypertension (SBP ≥160 mmHg and/or DBP ≥110 mmHg):

   • First-line IV medications: labetalol, hydralazine, or oral nifedipine 1
   • Target BP: diastolic 85 mmHg, systolic 110-140 mmHg 1

3. Seizure Prophylaxis:

   • Magnesium sulfate should be administered and continued for at least 24 hours postpartum 1

4. Blood Product Support:

   • Platelet transfusion if count is less than 50,000/mm³, especially prior to cesarean section 1, 5
   • Whole blood or packed red cells if hemoglobin is less than 10 g/dL 1

Delivery Considerations

• Mode of delivery depends on maternal condition, fetal status, and gestational age 1
• Vaginal delivery can be accomplished in most cases 5
• If cesarean section is required:
  • Consider general anesthesia for severe thrombocytopenia 3, 5
  • Use subfascial drains 5
  • Preoperative platelet transfusion for counts less than 50,000/mm³ 5

Anesthetic Considerations

• Neuroaxial block (particularly spinal anesthesia) is first choice for cesarean if there is only moderate, non-progressive thrombocytopenia 3
• General anesthesia is indicated when:
  • Severe thrombocytopenia exists
  • Neurological signs are present
  • Severe hypertension is difficult to control 3
• Be prepared for difficult airway management 3

Postpartum Care

• Continue blood pressure monitoring every 4-6 hours for at least 3 days 1
• Taper antihypertensive medications slowly after days 3-6 1
• Follow-up within 1 week if still on antihypertensives at discharge 1
• Laboratory abnormalities typically resolve over several days after delivery 4

Special Considerations

• Patients with complications such as pulmonary edema, acute renal failure, liver rupture, or extreme prematurity should be referred to a tertiary care center with maternal and neonatal intensive care facilities 5
• A multidisciplinary approach involving obstetrics, anesthesiology, and critical care specialists is essential for optimal management 3

Common Pitfalls

1. Delayed diagnosis: HELLP syndrome can mimic other conditions such as gastroenterologic diseases, viral hepatitis, or autoimmune disorders 2

2. Failure to recognize HELLP without classic preeclampsia signs: Remember that 20% of cases may not present with hypertension and proteinuria 2

3. Conservative management: Attempting to prolong pregnancy can lead to increased maternal and fetal mortality 4

4. Inadequate monitoring: Close observation is required for potential development of hemorrhagic complications, disseminated intravascular coagulation (DIC), or eclampsia 3